Peds Boards 2

Should also be considered in patients from closed-space fires from carbon monoxide poisoning

cyanide poisoning

Recognize lupus rash

- The classic NSLE rash is characterized by elliptical erythematous, papulosquamous lesions with central clearing, annular erythema or dyspigmentation, and a fine scale, which worsens with exposure to ultraviolet light. - The rash becomes apparent during the first 3 months after birth and commonly spontaneously resolves after maternal antibodies disappear at 6 to 8 months of age.

ophthalmia neonatorum tx

- conjunctivitis in <4 weeks old - early = GC; day 5-14 = chlamydia - tx = oral azithromycin (per red book) - NEVER topical - NAAT = most helpful = conjunctiva itself

Recognize Transient Neonatal Pustular Melanosis

- no additional evauation

Recognize DIPG

- unresectable - survival rate <5%

Normal hearing has threshold of ...

0-20 dB - audiology referral even if mildly abnormal

object permeance is a skill at what age ...

10 mos

Anterior fontanelles close by ...

2 yo

Can use spoon by what age ...

24 mos

Staph food poisoning typical presentation ...

4-6 hours after ingestion of food with nausea, vomiting, and diarrhea - symptoms generally do not last long - severe dehydration more common

Normal neutrophil percent on CBC

40-60%

BF infants should be taking how much vitamin D?

400 units vitamin D daily

4 yo girl at 5th percentile for height and at 55th %ile for weight. Webbed neck, broad forehead, hypertelorism, pectus excavatum. Prominent pulmonic ejection click, heard best at left upper sternal border, is present immediately after 1st heart sound. 2nd heart sound is split and short 2/6 medium-pitched systolic ejection murmur is present. What karyotype is most likely to be identified upon further eval for this pt?

46 XX - Noonan syndrome - pulmonary valvular stenosis - hypertelorism

F kid here for WCC. Can broad jump 2 feet, hop on one foot 3 times, brush teeth without assistance. How old is she?

48 mos

Ability to roll from back to front appears at ...

5-6 mos

Skipping is usually seen at what age?

5-7 yo

ABCD for melanoma in kids

A - amelanotic (red bump as example) B - bleeding/bump C - colorless/uniform color D - de novo E - evolution

Acyanotic heart lesion + left axis deviation + murmur

AV canal defect

When can children begin using sunscreen?

After babies turn 6 months old, sunscreen application should be a regular part of preparing to go outside in the sun.

6 week old boy + jaundiced during WCC. Lethargic and feeds poorly. Nystagmus and microphallus. Glucose 38 and direct bili 5. Most likely is most likely to be found in additional eval of pt?

Agensis of septum pellucidum - Septooptic dysplasia (SOD) = hypoplasia of optic nerve, pituitary deficiencies, absence of septum pelliucidum, other midline abnormalities - HESX1 gene

Rare AR coagulopathy that manifests as bleeding with large platelets ...

Bernard-Soulier = inherited deficiency of glycoprotein 1b - ex. postsurgical bleeding

Nerve affected with "false localizing sign" seen with ICP ...

CN 6

Observational studies that can be used to determine the odds ratio of developing a particular outcome by analyzing groups of subjects with and without particular exposures...

Case-control studies

17 yo hospitalized girl suddenly develops a rash. Complains of mild itching and discomfort in the genital area. Widespread tense blisters overlying both inflamed and noninflamed skin noted in lower trunk and in genital region. Several "rings" of blisters also identified. Mucous membranes are clear. Following biopsy, linear deposition of IgA noted at dermal-epidermal junction of basement membrane on direct immunofluorescence. Standard drug of choice to treat this condition?

Dapsone - Linear IgA bullous dermatosis - Idiopathic or drug-induced subepidermal blistering disorder - 24 hours - 1 month after time of initiation of therapy - String of pearls sign

Lithium is a known teratogen that has been associated with what cardiac condition?

Ebstein anomaly - cyanosis 2/2 right ventricular failure with right to left shunting at atrial level (PFO or ASD) - 1st heart sound widely split with loud tricuspid component

Feature distinctive of Type 2 (proximal) RTA in contrast to other RTAs.

Fanconi syndrome

Plays a major role in predicting long-term alcohol use ...

Genetic predisposition - particularly in alcohol-dependent first- and second-degree relatives - children of alcohol-dependent parents are 4-6 times more prone to develop similar dependence than those without a positive family history - studies of twins suggest a genetic predisposition involving chromosomes 9, 15, and 16 - alcohol dependence from the time of first alcohol use develops more quickly in adolescents than adults

Which glycogen storage disease: presents in the first few months of life with failure to thrive, seizures, developmental delay, and severe hypoglycemic episodes. Clinical features in an untreated patient can include hepatomegaly, lactic acidosis, hyperuricemia, hyperlipidemia, hypertriglyceridemia, and sometimes seizures from hypoglycemia.

Glycogen storage disease I

Waardenburg is associated with what GI disease?

Hirschsprung

Teen with post-op ventricular tachycardia. Best tx after workup ...

IV amiodarone

Anaphylaxis d/t hymenoptera (bees, wasps), stings, foods, latex are d/t

IgE-mediated rxns against specific antigens

15 mo old F + recurrent otitis media + multiple infections between 4-7 mos + hospitalized with pna at 5-6 mos old + serum electrophoresis at the time = Ab deficiency. Today, electrophoresis is normal, as well as T cell function. Has not had infection for 3 mos. What immunoglobulin abnormality was most likely responsible for her earlier susceptibility to dz?

IgG - Hypogammaglobulinemia - Maternal IgG protective until around 4 mos old

Most specific test for diagnosis of acute EBV infection is detection of ...

IgM-viral capsid antigen (VCA)

A low U/L ratio indicates a relatively shorter trunk and longer legs, and it can be seen in

Klinefelter syndrome and delayed puberty

EKG findings of tricuspid atresia

LAD - also LAD = AV canal, endocardial cushion defect

Vaccines given as subQ injections

MMR varicella IPV

16 yo presents with difficulty releasing his grip on objects. He has also been observed to trip over his feet recently and has been seen falling several times. He lives with his mother. His dad died at 22 yo of a "heart attack." On exam, pt appears gaunt for his age. He has moderate temporal wasting and some difficulty annunciating words. Pt has fair strength of hands; however, he seems unable to easily release his grasp. His proximal muscles have normal strength. He has difficulty with plantar dorsiflexion. His Romberg exam is normal. What is most consistent with his presentation?

Myotonic dystrophy - inv't of facial muscles + weakness + atrophy - neck muscles + distal muscles affected - AD - cardiac inv't common: conduction defects, sudden death sometimes

abdominal migraines best treated with ...

NSAIDs triptans antinausea meds - rizatriptan = only triptan approved for children as young as 6 yo

Pregnant woman evaluated at 32 weeks gestation. Fetus noted to have frequent premature heart beat on monitor. Baby is having anywhere from 6-10 premature beats per minute. They are usually individual, but up to 3 have been noted in succession. Woman drinks 1 cup of coffee per day. Next step?

Obtain fetal echo - premature beats in fetus are not normal

8 yo with sickle cell presents for WCC. What indicates this patient has met the current recommendations for use of pneumococcal vaccine(s) in patients with sickle cell dz?

PCV 13 at 2, 4, 6, and 12-15 mos PPSV23 at 2 yo, followed by 2nd dose 5 years later

Equally effective for initial seizure control in neonates

Phenytoin and fosphenytoin - Once the neonate is stabilized, management is tailored to the underlying etiology of the seizures with the goal of seizure control.

Presents between birth and 6 mos of age. Child usually has marked hypotonia, absent tendon reflexes, weakness of intercostal musculature. Fasciculation of tongue is common feature. Mental function typically preserved.

Spinal muscular trophy type 1 / Werdnig-Hoffman dz

Parents of 2 mo old concerned that soon after every feed, their son "screams out in pain." During bathing, they have noticed "some of his bones seem very tender and even swollen." On PE, the pt is irritable and febrile. There is tender, firm swelling along the left mandible. A 2nd firm, tender swelling is evident over the the distal portion of the left femur. Skeletal survey to evaluate for nonaccidental trauma reveals cortical thickening and subperiosteal new bone formation along left mandible and distal portion of left femur. What is correct regarding these findings?

Symptoms will like resolve by 24-30 mos of age - Infantile cortical hyperostosis = Caffey disease - AD + incomplete penetrance - changes in bone occur prior to 6 mos old and resolve by 24 mos - affected infants = irritable and febrile - mandible affected in >95% of cases - labs = leukocytosis, elevated ESR, increased alk phos

Most commonly affected blood vessel in ischemic perinatal stroke

The left middle cerebral artery

Girl experiencing dysfunctional voiding of urine. Her symptoms (dysuria, lower abdominal pain, urinary frequency), signs (suprapubic tenderness), associated risk factors (dysfunctional voiding, constipation), and abnormal urinalysis results (presence of nitrites, white blood cells, and bacteria) favor diagnosis of...

acute cystitis - commonly caused by an acute bacterial infection - is inflammation localized to the bladder - risk factors for acute cystitis include female sex, lack of circumcision in boys, sexual activity, dysfunctional voiding, constipation, neurogenic bladder, bladder stones, indwelling catheters, anatomic abnormalities (eg, posterior urethral valves, vesicoureteral reflux), and underlying medical conditions (eg, diabetes mellitus, immunosuppression)

Hepatitis B surface antigen is present in individuals with

acute or chronic infection and vaccinated individuals for 3 weeks after receiving the hepatitis B vaccination

Manifestation of late lyme disease (stage 3)

arthritis - mos to years after untreated infection - involves large joints, especially the knee

Pts with Hurler syndrome at birth ...

asymptomatic - MPS type I - develop symptoms within first 2 years of life

Most patients with lead poisoning are ...

asymptomatic - venous blood level >/= 3.5 confirms diagnosis

In autoimmune polyglandular syndrome Type 2 (APS2), patients will have...

autoimmune thyroid disease T1DM primary adrenal insufficiency (Addison dz) - pts tend to be female - HLA-DR3, HLA-DR4 = common

Achondroplasia is inherited how?

autosomal dominant pattern with complete penetrance

In a hospitalized patient with an IV who is hypoglycemic and not completely conscious, best therapy is IV dextrose. PALS suggests...

dextrose 0.5 - 1 g/kg --> can be given as D10W (5-10 ml/kg) or D25 (2-4 ml/kg)

Characterized by infrequent brief focal motor seizures that occur predominantly in sleep. Clinical presentation of seizure is typically described as facial and arm twitching, speech arrest with accompanying oropharyngeal guttural sounds, and hypersalivation with preservation of consciousness. Generalized tonic-clonic seizures may occur in a subset of patients.

benign rolandic epilepsy (BRE) - also known as benign childhood epilepsy with centrotemporal spikes - common childhood focal motor epilepsy syndrome - typically presenting between the ages of 5 and 10 years - Electroencephalography in benign rolandic epilepsy demonstrates biphasic independently bilateral centrotemporal spikes on a normal background, often identified in light sleep.

Insulin should be initiated at diagnosis for youth with type 2 diabetes mellitus if

blood glucose level is greater than or equal to 250 mg/dL (13.9 mmol/L) or hemoglobin A1c is greater than or equal to 8.5%

Girl has mild neutropenia (1,000-1,500 neutrophils/µL), uncompensated anemia (reticulocytopenia), thrombocytopenia (100,000-150,000 platelets/µL), and macrocytosis. The differential diagnosis for anemia with reticulocytopenia can be classified by the presence or absence of other cytopenias. Anemia with reticulocytopenia associated with other cytopenias (complex anemia) raises concern about

bone marrow failure or leukemia

How to feed infants with cleft palate ...

bottle fed with adaptive techniques (ex squeezable bottles)

Adverse effects of propranolol

bradycardia conduction disturbances (eg, atrioventricular block) hypotension bronchospasm hypoglycemia

CT of child with tuberous sclerosis may reveal

calcified subependymal nodules = major diagnostic criteria

Baby with vitamin D deficiency. Low Ca, P. Elevated PTH, alk phos. Vitamin D levels pending. Tx?

calcium cholecalciferol calcitriol - giving P would lower absorption of Ca

observational studies that can be used to determine the odds ratio of developing a particular outcome by analyzing groups of subjects with and without particular exposures

case-control study

Children with obesity and chronically elevated liver enzyme levels should be screened for

causes of chronic liver disease

In a neonatal patient who presents with suspected meningitis, what would be the best choice for cephalosporin?

cefotaxime - 3rd gen

Chiari III refers to ...

cerebellar herniation into cervical or occipital encephalocele

The American College of Medical Genetics and Genomics recommends what as first-line testing for any patient with autism-spectrum disorder, developmental delay, or both?

chromosomal microarray and fragile X analysis - Pretest counseling is important before chromosomal microarray and fragile X analysis analysis are performed. - Pretest counseling should include discussion of need for genetic testing, possible outcomes, expected out-of-pocket expense, and referral to a genetics clinic, if indicated.

RVH in utero is suggestive of ...

coarctation obstructing right-to-left ductal flow

An abnormality of what cells is seen in Langerhans cell histiocytosis ...

dendritic cells

Kid with mild-mod persistent asthma. Tx?

combined inhaled steroid and formoterol inhaler used daily and as needed

9 yo M + fever, joint pain, rash x2 days. Started on amoxicillin for sore throat 8 days ago when he was in contact with a classmate with sore throat. 1 mo ago, he was successfully treated for acute otitis media with amoxicillin and has been well in the interim. PE = posterior pharyngeal edema without exudate and anterior and posterior cervical LAD. Has periarticular swelling of metacarpophalagneal joints, knees, wrists, ankles and dusky urticarial papules and plaques on lateral aspect of feet and hands and at junction of sole and side of foot. Cause of symptoms?

serum sickness - associated with amoxicillin use

Boy has significant short stature, a normal body mass index, a concordant bone age, and a high upper-to-lower body segment ratio (U/L ratio), indicating relatively longer trunk and shorter legs. These findings are most consistent with

skeletal dysplasia - The U/L ratio can be used to assess body proportions. - The lower segment is measured from the pubic symphysis to the floor while standing. The upper segment is calculated as height minus the lower segment. - Infants have a relatively high U/L ratio (about 1.7 at birth), which gradually decreases to 1 around age 10 years, reaches a nadir during early puberty, and returns to about 0.9-1 by adulthood. - A high U/L ratio indicates a relatively longer trunk and shorter legs, and it can be seen in skeletal dysplasias and precocious puberty.

Upon arrival and initial exam in newborn nursery, term female infant weighing 6 pounds 2 ounces is noted to have 4cm x 3cm blister on lower aspect of abdomen. There are also 2 intact blisters on her feet and several 5cm x2.5cm, denuded, equally erythematous lesions of posterior trunk. Most appropriate next step to best identify likely cause of this pt's findings?

skin biopsy - epidermolysis bullosa = mechanobullous disorder - tx = supported - type = inheritance pattern - infants must be protected against minor damage - tx cutaneous wounds with abx - avoid tape or adhesives

Girl with persistent microscopic hematuria. Next step?

spot urine calcium:creatinine ratio - look for hypercalciuria --> risk factor for future stones

A 4-year-old boy with symptoms of a viral respiratory infection for 3 days is being evaluated for sudden onset of fever to 39oC, respiratory distress with barking cough, and inspiratory/expiratory monophasic wheezing. His medical history is unremarkable. On physical examination, he appears ill and uncomfortable, with intercostal and suprasternal retractions. Of the following, the pathogen MOST likely associated with this boy's condition is

staph aureus bacterial tracheitis as a superinfection - Staphylococcus aureus is the most common organism implicated in bacterial tracheitis - Bacterial tracheitis can be rapidly progressing and life threatening. Up to 80% of patients with bacterial tracheitis require intubation, thus the first priority in patient management is careful evaluation and stabilization of the airway

In children 3 mos - 3 yo, what are the most common causes of meningitis?

strep pneumo neisseria meningitidis

Most common cause of peritonsillar abscess ...

streptococcus pyogenes

Kerion is an inflammatory reaction to a scalp tinea infection which presents as a large, hairless, red, and boggy area over the scalp. Trichophyton is the most common cause of tinea capitis, followed by Microsporum species. Tinea capitis is treated with

systemic oral antifungal agent such as griseofulvin, terbinafine, or fluconazole

Recurrence risk of trisomy 21/down syndrome is higher if

the mother is a carrier of a balanced translocation - lowest if the child has a freestanding chromosome 21

Patients eligible for medication abortion are

those who - are less than 11 weeks pregnant - have access to a treating clinician - have transportation to a medical center in case of an emergency

Where for calcifications occur in toxo?

throughout brain parenchyma, including caudate nucleus and basal ganglia - hydrocephalus + chorioretinitis + cerebral calcifications

Teen with hypokalemia. Has toxin-induced distal RTA. This is a hallmark of what drug?

toluene - high disappears quickly - inhalant

Celiac disease can cause abdominal pain, diarrhea, and slow weight gain, as well as host of extraintestinal symptoms, including ...

tooth enamel defects

Treatment of tinea versicolor

topical antifungal therapy - 2.5% selenium sulfide shampoo or ketoconazole 2% shampoo (used as a body wash) applied nightly for 1-2 weeks --> let it sit on skin for 10 minutes prior to rinsing - topical ketoconazole cream effective but typically not practical due to widespread distribution - recurrences are common, but selenium sulfide 2.5% shampoo can be used 1-2 times per month for preventative maintenance

For uncomplicated hemangiomas of skin, what is the treatment ...

topical beta-blockers

Useful way to distinguish between GI bicarb losses and urinary losses

urine anion gap - negative urine anion gap = diarrhea

Urine osmolality in SIADH ...

urine osmolality > serum osmolality

Infant with late-onset sepsis. 12 day old. Tx?

vanc and gent

Gastroschisis is believed to have been caused by ...

vascular accident involving right umbilical vein or right omphalomesenteric artery

VACTERL association, formerly known as VATER association, stands for

vertebral defects (V) anal atresia (A) cardiac defects (C) tracheoesophageal fistula (TE) renal defects (R) limb defects (L) - Diagnosis of VACTERL is established when at least three of these features are present. - VACTERL association is a diagnosis of exclusion.

When should varicoceles be repaired?

when affected testicular volume is less than that of unaffected side

Young girl exhibits a severe microcytic anemia with a complete absence of hemoglobin A on electrophoresis. This presentation is diagnostic of

β thalassemia major - Microcytic anemias result from the underproduction of hemoglobin, resulting in smaller erythrocytes during the hematopoietic process. A deficiency in any component of hemoglobin will result in a microcytic anemia, including iron deficiency and a defective production of the α or β globin subchains. - A single defective α globin gene results in a silent carrier, two defective genes in α thalassemia trait, three in hemoglobin H disease, and four in hydrops fetalis. As there are only two β globin genes, a single defective gene results in β thalassemia trait and two defective genes in β thalassemia major. - Children with β thalassemia major (transfusion dependent thalassemia) will inevitably develop iron overload

A 5-year-old girl is separated from her mother during a routine health supervision visit while the staff performs screening audiometry. She begins screaming and kicks the nursing assistant. Of the following, the BEST response to this situation is to

discuss the girl's response with her mother - The girl in the vignette is having an abnormal reaction to being separated from her mother. This response should be discussed with her mother as it may indicate a history of adverse childhood experiences (ACEs). - All children should be screened for ACEs at every health supervision visit. Any child who screens positive for ACEs should be referred to community resources. - Recognizing a potential history of traumatic events is key to providing trauma-informed care. These children are at increased risk for developmental delays and mental health concerns.

Prolonged and severe neglect can result in ...

disinhibited social engagement disorder - decreased avoidance or skepticism of strangers - lack of concerns when leaving and going with stranger - reduced need to touch base or remain close to caregiver when venturing out into new places - extremely friendly behavior - minimal social boundaries

retinopathy of prematurity is a disease of ...

disordered retinal vascularization in premature infants

17 yo F + 6 mo hx of amenorrhea admitted for severe anorexia. On day 2, she complains of severe constipation. Most appropriate management of constipation?

docusate - not a laxative = more appropriate for this setting

8 day old infant with bili of 32. Active hemolytic dz. Neuro toxicity on PE showing acute bilirubin toxicity. Admitted for PTX but found to have this high bili... Tx...

double volume exchange transfusion - can continue PTX during this time since need time to set things up; can also do IVIG during this time

Cartilage and bony defects that can be seen in MPS Type 1 / Hurler Syndrome ...

dysostosis multiplex = beaking of 1 or more vertebral bodies via hypoplasia of anterosuperior portion of vertebrae = proximal pointing of metacarpals = shortened, thickened clavicles = rounded iliac wings = inferiorly tapered ilia = hypoplastic epiphyses = expansion of sella turcica = thickened diploic space on radiographs of skull

The classical triad of symptoms in pheochromocytoma is

episodic headaches sweating tachycardia - Hypertension is also a common feature - In children, unlike adults, elevated blood pressure is more often sustained than paroxysmal - Other uncommon presenting features include pallor, constipation, panic attacks, blurry vision, weight loss, polyuria, and polydipsia

First-line therapy for strep throat in kids with penicillin allergy

erythromycin - absorbed better when given with food - othersources: keflex for mild allergy; clindamycin for higher risk

Although not a vaccine, pediatricians should be aware that palivizumab, the monoclonal antibody against respiratory syncytial virus (RSV), should be considered for administration how often?

every month for 5 months starting at the onset of RSV season - to infants who were born before 29 weeks' gestation and younger than 1 year when the RSV season starts - as well as to infants with certain types of heart defects or chronic lung diseases

Most common cause of macrocephaly ...

familial megalencephaly

Dysmenorrhea statistically is more likely among adolescents with

family history of dysmenorrhea - Its incidence increases with gynecologic age as many adolescents experience anovulatory cycles for the 1st few years of menarche

Following a few days of fever and myalgias, a 10-year-old boy presents with progressive shortness of breath, exercise intolerance, and a productive cough. Sputum analysis finds hemosiderin-laden macrophages. Chest X-ray shows infiltrates. Urinalysis detects mild proteinuria, and sediment contains erythrocytes and red cell casts. There is circulating IgG anti-GBM (glomerular basement membrane) antibody in the serum. What disorder is most likely present?

goodpasture syndrome - hallmark of Goodpasture syndrome is the existence of circulating IgG anti-GBM (glomerular basement membrane) antibody in the serum - Generalized symptoms usually precede the more specific symptoms related to the lung disease. - Renal disease represents only the 3rd step in the progression of Goodpasture syndrome. The typical pathologic finding is crescentic proliferative glomerulonephritis. - Corticosteroids and alkylating immunosuppressive agents have been suggested for treatment. - The recurrent massive hemorrhages, pulmonary hypertension, and respiratory failure indicate a worsening prognosis.

A 10-year-old boy presents with rapid growth; his height is >95th percentile. Growth velocity is >75th percentile, and his height exceeds the mid-parental height. His developmental milestones and body proportions are normal for his age. External genitalia are normal and do not show signs of onset of puberty. Facial features appear coarse; he has a prominent lower jaw, broad nose, bushy eyebrows, and enlarged tongue. Dorsal kyphosis is present. Bones and cartilage show overgrowth, and his hands and feet are large. What is the most likely cause of tall stature in this child?

growth hormone excess - pituitary gigantism - results in somatic overgrowth - facial features are coarse, with prominent jaw, broad nose, enlarged tongue, bushy eyebrows, thick skin, dorsal kyphosis, and enlargement of both hands and feet - muscle weakness, overgrowth of bony and cartilaginous tissues, skin pigmentation, and cardiomyopathy may occur - pituitary gigantism may occur due to pituitary hyperplasia or pituitary adenoma. Pituitary hyperplasia occurs when there are hormone deficiencies resulting in decreased feedback (e.g., hypothyroidism, hypogonadism, and hypoadrenalism).

Most common presentation of von willebrand disease

heavy menstrual bleeding in young girls

Muc more common cause of papular acrodermatitis in areas of the world with universal IZZ ...

hepatitis B

Bizarre behavior in teen with temporal lobe inv't on MRI =

herpes simplex meningoencephalitis

Lab findings in rhabdomyolysis

high P low calcium hyperuricemia hypoalbuminemia

Contraindication to nasal flu vaccine ...

hx of wheezing within last 12 mos

Potassium in adrenal insufficiency/addison's

hyperkalemia

Hypogonadism associated with persistent hypoglycemia i a neonate is consistent with

hypopituitarism

Caffey disease tx ...

indomethacin and prednisone

What term refers to wide range of statistical procedures that include chi-square testing and analysis of variance ...

inferential statistics

Droplet precautions are used to prevent transmission of organisms that spread by close respiratory or mucous membrane contact. Infections caused by common microorganisms that require droplet precautions are

influenza pertussis parvovirus B19 (before the onset of rash) Neisseria meningitidis (invasive) rhinovirus mumps adenovirus (pneumonia) AIM 4 NPR

Infections caused by common microorganisms that require droplet precautions are

influenza pertussis parvovirus B19 (before the onset of rash) Neisseria meningitidis (invasive) rhinovirus mumps adenovirus (pneumonia) RAIN PM

Small left colon syndrome most often observed in mothers who taken what medication?

insulin

propionic acid is mainly derived from metabolism of ...

isoleucine, valine, threonine, methionine - propionic acidemia via deficiency of enzyme propionyl-CoA carboxylase (normally converts propionic to methylmalonic acid)

Peripheral neuropathy is a common side effect of what TB tx ...

isoniazid - causes depletion of pyridoxine

Pts wearing isolated lap-belt restraints present with seatbelt syndrome, which classically consists of transverse abdominal wall contusion, lumbar Chance fracture, visceral trauma. Increased risk of what injury?

jejunal perforation/rupture - most common

During a health supervision visit for their child, the parents share that they are in the process of adopting a 15-month-old boy. They are excited to add to their family and would like guidance in preparing for this child's arrival. The parents ask when they should tell their child that he is adopted. Of the following, the MOST appropriate time is when the child

joins the family

Side effect of hydroxyurea

leukocyte suppression - decrease in platelets, leukocytes, absolute neutrophil count

What antiseizure medication can be used in conjunction with OCPs without interfering with action of contraceptives?

levetiracetem - women taking enzyme-inducing antiseizure drugs should us alternative method of contraception

Pts with HbH disease require ...

lifelong blood transfusions + deferoxamine - to manage chronic iron overload

Afebrile, well-appearing 4-week old male noted to have prominent mottling over left abdominal and lower thoracic regions, characterized by reddish-violaceous, reticulated patches with sharp demarcation at midline. With warming, lesions remain unchanged. These findings are often associated with...

limb hypoplasia on affected side cutis marmorata telangiectasia congenita - often associated with congenital anomalies, including but not limited to undergrowth or overgrowth of involved extremity - lesions do not disappear with rewarming - gradual disappearance over years is typical - can occur with other discrete syndromes, like sturge-weber and klippel-trenaunay syndrome

Following varicella vaccination, 1-3% of children have what adverse reaction?

localized varicella-like rash

Predominant genetic mutation in pts with Hirschsprung's disease ...

loss of function mutation in RET proto-oncogene

Testosterone levels in Klinefelter syndrome

low

Most girls with Turner syndrome develop primary ovarian failure (hypergonadotropic hypogonadism). Clinical guidelines recommend starting what after primary ovarian failure is confirmed?

low-dose transdermal estradiol - for pubertal induction at age 11 to 12 years in girls with Turner syndrome - obtain gonadotropin levels

4 day old female born at 34 weeks gestation, admitted to ICU due to fever, irritability, and poor feeding. Following uncomplicated LP, CSF reveals 276 WBC, 2 RBCs, protein of 440, glucose of 14. CBC include WBC 3,400 with 22% neutrophils (50% bands, 50% segs), 46% lymphocytes, 18% monocytes. What's most likely associated with these findings?

maternal hx of flu-like illness at 28 weeks gestation - increased # of peripheral monocytes = neonatal listeriosis = presents as flu-like illness at 2nd or 3rd trimester = may present prior to 7 days of gestation (early-onset) or from 8-30 days of age (late-onset) --> initial therapy = amp + gent

Infections caused by common microorganisms that require airborne precautions are

measles varicella Mycobacterium tuberculosis (TB) MaV

Little league elbow --> AKA

medial elbow apophysitis

Cholestasis in pts with CF is associated with reducing bile acid concentration in the small intestine, with subsequent impaired absorption of long-chain fatty acids. What's an important nutritional consideration for an infant ...

medium-chained triglyceride supplementation in formula

Most sensitive test to diagnose asthma ...

methacholine challenge - golden standard to rule out asthma

Most males with CHARGE have what GU abnormalities at birth ...

microphallus, cryptorchidism

Best measurement of infant's nutritional status if they have biliary atresia (because of organomegaly)...

mid-upper arm circumference

Disadvantage of retrospective cohort study ...

misclassification of exposure - outcome has already occurred

What triad of psychiatric disorders is most common in male adolescents who complete suicide?

mood disorder, conduct disorder, substance abuse

Premature baby needs how much sodium in relation to a term baby?

more sodium than a full term infant - sodium supplementation should be started immediately

A 5-year-old boy is seen in the emergency department after he fell from the monkey bars on the playground just prior to arrival. He reports severe pain in his left forearm. He rates his pain as a 7/10 on a pain rating scale that uses faces. Physical examination reveals an anxious child, crying in pain, with an obvious deformity of his left forearm. His left forearm and hand are neurovascularly intact. There are no other injuries. The boy's mother witnessed the fall and states that there was no loss of consciousness. Of the following, the BEST next step in the management of this child is to administer

morphine IV - acute, severe pain due to a forearm fracture. A short-acting opiate such as intravenous morphine would be the preferred medication to treat the pain - nonsteroidal anti-inflammatory drug such as ibuprofen may be appropriate in conjunction with a short-acting opioid but would not be appropriate monotherapy in a child experiencing acute, severe painq

mechanism of hyper-IgE syndrome ...

neutrophils fail to adhere to endothelium and enter tissues

Do protozoa cause eosinophilia?

no - able to replicate inside body though

A healthy rate of weight loss for adolescents is

no greater than 1.5% of body weight per week - Higher rates of weight loss are often the result of fluid loss and are associated with a reduction in lean muscle mass and strength

granuloma annulare tx

no intervention - "ringwork not getting better with antifungals" as a story - don't no cause - usually resolves by itself - no scaling to surface of skin

A 17-year-old adolescent girl was prescribed the wrong oral contraceptive pill. She is sexually active with 1 male partner. She has not experienced any side effects. Of the following, disclosure of this error is BEST made to the adolescent and

no one else - The decision to involve pediatric patients in the disclosure of medical errors depends on the situation and the patient's capacity and decision-making ability

In >80% of malaria infections among international U.S. travelers who were prescribed chemoprophylaxis, what was the downfall for prescribed CDC-recommended chemoprophylaxis regimens?

not followed correctly, such as starting too late, not taking consistently, stopping too soon after travel

The differential diagnosis for a macrocytic anemia in children is

nutritional deficiencies (vitamin B12 and folate) bone marrow failure/myelodysplasia certain drug exposures (eg, chemotherapeutics) hypothyroidism

Pt with multicystic kidneys has allograft renal transplant. 2 mos later, presents with fever, malaise, tenderness in graft region. Labs = rising creatinine. What other finding is chraceristic?

oliguria - acute graft rejection - fever, malaise, graft tenderness

Some drugs require acidic env't for absorption (azole antifungals, iron, thyroid hormone); so meds, like levothyroxine, should be taken when?

on empty stomach - ex. before breakfast

When can a kid with erythema infectiosum/fifth disease (due to parvovirus B19) return to school?

once the "slapped cheek" rash appears = no longer infectious

Most children with diarrhea caused by Shiga-toxin producing E coli O157:H7 (STEC) should be treated with ...

only with fluid replacement and not abx

Previously healthy boy in this vignette has a sudden onset of lethargy, pinpoint pupils, and hypopnea. Evaluation shows respiratory acidosis and hypoxia. These findings suggest

opioid toxidrome - most appropriate next step in management is intravenous naloxone

First-line tx for refeeding syndrome with hypophosphatemia ...

oral sodium phosphate

Potential complicaton of pregnant person with gonorrhea

preterm birth

Tick paralysis presents with

rapidly progressive ascending paralysis ataxic gait bulbar and cranial nerve dysfunction - The presentation is similar to Guillain-Barré syndrome - The presence of a tick on skin examination is supportive of the diagnosis of tick paralysis. - In cases of tick paralysis, there can be improvement in weakness as soon as 1 hour following tick removal, with full recovery within a few days.

Sexually active female with dysmenorrhea and migraine with aura. Uses condoms regularly. Next step in management?

recommend LARC - migraines = contraindication to OCP (estrogen) - teen = LARC doesn't require remembering

Classic ex of 2-hit hypothesis

retinoblastoma

The third leading cause of death in the US in ages 10-24 is suicide. It is helpful to assess what for any child or adolescent who has attempted suicide?

risk/rescue ratio - the risk of the action or attempt made relative to the likelihood of rescue

Children with biotinidase deficiency present with ...

seizures hypotonia ataxia dev'tal delay hearing and vision loss spastic paraparesis cutaneous abnormalities - labs = ketoacidosis, hyperammonemia, organic aciduria

hot tub folliculitis tx...

self-limited --> usually resolves in 1-2 weeks without tx

Infant with initial glucose >50, then ~35 at 1 HOL via POC. Asymptomatic. Next step in management...

send blood to lab for plasma glucose level - asymptomatic = doesn't need IV - can feed the baby

What test characteristic is desirable for screening tests for serious and treatable conditions to identify as many affected individuals as possible?

sensitivity

Baby with unilateral dysplastic kidney. Severe form of renal dysplasia. Bunch of cysts ~ "bunch of grapes" ~ no functioning tissue. Management?

serial renal US - no standard approach - many nephrologists/urologists just follow until it involutes - ensure other kidney grows appropriately as a result

Recognize lichen striatus

- asymptomatic or mildly pruritic - self-limited - inflammatory - resolves over several months - topic steroids help symptoms but not with resolution - grouped 2-5 mm pink to hypopigmented, flat topped papules - curvilinear, blashkoid distribution - most often located on extremity - darker skin types = hypopigmented

Recognize streptococcal fever

- atypical presentation of streptococcal infection - most common in toddlers - cause = group A strep - protracted nasal congestion

NICU wants to know if ppx fluconazole prevents yeast-induced central line infections in pts receiving TPN. They study a group of 750 pts with centra lines who are receiving TPN divide them evenly into two groups. At the end of the study, 8 pts from the control group were found to have yeast-induced central line infections compared to only 2 from the experimental group. How many pts do they need to treat with ppx fluconazole in order to prevent 1 central line infection?

63 = inverse of ARR - 8/375 - 2/375 = 0.016 --> 63

How soon after hepatitis A diagnosis can kids return to school ..

7 days after onset of symptoms

90% of children are able to sit without support by what age ...

7 months

Early on, breastfeeding can lead to painful nipples, which typically improve within

7 to 10 days

6 yo with 3/6 regurgitant, pansystolic murmur at left sternal border that radiates to right sternal border and a hx of supraventricular dysrythmias d/t WPW is also noted on EKG to have tall peaked P waves in leads II and VI, and RBBB. What are you most likely to see on echo?

Apical displacement of tricuspid valve, tricuspid regurgitation, dilated right atrium Tricuspid regurgitation / Ebstein anomaly

3 yo F + increasing vomiting after meals. Hx of ID an seizures. PE = spastic diplegia of LE's. Labs normal except for elevated serum glutamine and alanine. Deficiency in what enzyme = explanation for her symptoms?

Arginase - AR - urea cycle disorder

Blood gas + ventilation: increase in PO2 after change. Improved oxygenation. Main determinant of increasing someone's oxygenation on ventilation =

MAP - increase this via: increase pressure given (increase PEEP) - increase inspiratory time/time with inhalation = increasing inspiratory time

10 mo old + exposure to measles + mom reports family members staying in house developed symptoms 24 hours ago + next step in management of this infant?

MMR vaccine now, again at 1 yo, and 3rd dose at 4 yo - infants age 6-11 mos --> MMR vaccine within 72 hours of exposure or IGIM 0.5 ml/kg (max dose 15 ml) or IVIG 400 mg/kg within 6 days of exposure

UC is mostly associated with primary sclerosing cholangitis. PSC is progressive chronic inflammation and fibrosis of intra and/or extrahepatic bile ducts. There is obliteration of peripheral bile ducts and dev't of bile duct strictures. Pt will have elevated alk phos, GGTP, and liver enzymes. Best diagnostic test is...

MRCP - will show beading (stricture and dilatations) of bile ducts - less invasive than ERCP and usually performed first

4 week old male infant presents to ED with new-onset seizure and encephalopathy. He has been vomiting for past 24 hours with minimal oral intake. You suspect a stroke. What imaging modality is the best method of dx?

MRI + magnetic resonance venography - best method of diagnosis for central venous thrombosis

4 month old female presents with poor feeding and difficulty gaining weight. Her mom reports she has been a sluggish feeder since birth, but her stamina with feeding seems to be decreasing with time. The most striking features on physical exam include marked pallor and a hyperdynamic cardiac exam with mild tachycardia. Evaluation of hemoglobin reveals very low level of 2.6. Direct consultation with regional hematologist raises possibility of Diamond Blackfan anemia. Which of the initial findings would be most supportive of this diagnosis?

Macrocytosis DBA = congenital hypoplastic cause of profound macrocytic anemia - usually present between 2-6 mos - defective function of marrow

Pregnant mother is a chronic carrier of hepatitis b (positive for both HBsAg and IgG anti-HBc). Per CDC recommendations, infant born to mother who is HepBsAg positive should receive

Hep B immunoglobulin and hepatitis B vaccine *within 12 hours of birth*

2 hour old female + profuse amounts of mucus and saliva bubbling from nose and mouth, anal atresia, 3/6 harsh systolic murmur along lower left sternal border. What additional clinical findings is most likely to be identified in this pt?

Hypoplastic thumb and radial aplasia - VACTERL association - VSD and esophageal atresia

Immunocompromised pts are at increased risk for disseminated varicella. Tx?

IV acyclovir - continue until no new lesions

A 14-year-old male patient sustains a blunt injury of the spine in a motor vehicle collision. There is no history of unconsciousness. He is brought to the emergency department 2 hours after initial stabilization and immobilization of the spine at the collision site. Physical examination reveals his vital signs are stable and he is fully conscious. There is hypotonia and hyporeflexia in both legs. What should be included in the management of this patient?

Maintain mean arterial pressure at 85-90 mm Hg. - The patient has a blunt spinal cord injury. The correct management is to maintain mean arterial pressure between 85 and 90 mm Hg.

2 yo M + irregularly shaped patches on back + first noted 4 weeks ago when on vacation in Key West + dad was cutting limes the previous day while making pie + initially, patches were erythematous, painful, and nonpruritic; now hyperpigmented. None new developed. Only has lesions in sux exposed areas. Cause?

Phytophotodermatitis - via lime exposure - plants containing psoralens - phototoxic rxn

What channels are dysfunctional in Lange-Jervell-Nielsen syndrome?

Potassium channels - unexplained syncope + deafness in relative - AR - prolonged QT + vulnerable to torsades

Sample size calculations depend on a number of factors including:

Power of the study (set by the investigator) Level of significance (set by the investigator) Expected effect size (set by the investigator) Standard deviation in the population (random error in the sample population)

Newborn baby M + small hands and hypotonia. Mg sulfate given to mom. Child's hypotonia most likely d/t

Prader-Willi syndrome

Recognize pneumomediastinum

- auscultation of the heart shows distant heart sounds with a positive Hamman's sign, a crunching or clicking sound heard over the precordium and synchronizing with the heartbeat - chest radiograph also shows mediastinal air with more distinct cardiac borders due to pneumopericardium - results from the rupture of the alveolus, followed by air dissecting along the bronchovascular sheath and free air reaching the mediastinum, leading to pneumopericardium, pneumothorax, subcutaneous emphysema, and pneumoperitoneum - risk factors include bronchiolitis, staphylococcal pneumonia, bronchopulmonary dysplasia, foreign body aspiration, and penetrating chest injury - acute asthma is the most common cause of pneumomediastinum in older children and adolescents

Recognize erythema nodosum

- can develop in ppl taking: OCPs, sulfa abx - erythematous or purplish tender nodular subcutaneous lesions overlying pretibial surfaces - delayed hypersensitivity reaction to a drug; also associated with certain infections and underlying conditions

Hx of irritability, FTT associated with diarrhea, skin and mucus membrane lesions developing soon after infant is weaned from breast milk is c/w

acrodermatitis enteropathica - zinc deficiency = AR - gene mutation on chromosome 8 - symptomatic soon after discontinuation of breast milk

Summary of indications for tympanostomy tubes:

- Refractory infection with moderate to severe symptoms - Unresponsiveness to at least 2 antibiotics - Hearing loss of 20-30 dB or worse with effusion = 3 months - Impending or actual complications (mastoiditis, labyrinthitis, etc.) - Persistent infections (4-6 months) - Advanced middle ear disease (e.g., cholesteatoma) - Craniofacial anomalies that predispose to middle ear dysfunction

Recognize prolonged qtc interval

- The QTc interval is measured from the start of the QRS complex to the end of the T wave. - The QT interval must be corrected to the heart rate; this is done using Bazett's formula, according to which the measured QT interval is divided by the square root of the preceding R-R interval. - The normal QTc interval is less than 440 ms but is variable according to age and sex. - Long QT syndrome can be acquired (via electrolyte abnormalities, drugs, or underlying medical disorders) or inherited. - It is advisable to refer patients with long QT syndrome to a pediatric cardiologist, who can recommend appropriate treatment and monitoring. - These patients should seek genetic counseling, and their family members should undergo screening.

Risk factors for cerebral edema in kiddo with DKA include...

- age <5 yo - new-onset diabetes - low initial PCO2 - high initial serum urea nitrogen - lesser increase in serum sodium with therapy - tx with bicarb

Recognize Mycoplasma-induced rash and mucositis

- can be reeeally prominent

Recognize Haemophilus ducreyi

- causes chancroid disease = common in sub-saharan Africa - small gram-negative rods - in chains = "school of fish" and Latin America - initial lesion = erythematous papule --> rapidly involves into pustule --> erodes into painful ulcer with erythematous base = grayish-yellow purulent exudate - tx = 1g azithromycin orally vs 250mg IM ceftriaxone

Recognize pseudostrabismus

- symmetric corneal light reflex - reassurance

2 week old infant presents with 24 hour hx of poor feeding and irritability. Temp is 101.2 F. He is lethargic, difficult to arouse, and has bulging anterior fontanelle. LP with WBC 8600, protein 190, glucose 22, gram stain with gram negative rods. MRI reveals abscess formation in both frontal lobes along with meningeal enhancement c/w meningitis. Most likely cause of this pt's clinical, lab, and neuroimaging findings?

Citrobacter koseri - abscess via: lethargy, poor feeding, vomiting, bulging fontanelles - if brain abscess suspected, should do neuroimaging - brain abscess antimicrobial tx = 6-8 weeks

Malformed or absent thumbs can be a feature of several disorders, including

Fanconi anemia Diamond-Blackfan syndrome

3 mo old + poor wt gain + poor feeding / irritable / easily tires during BF'ing. BR 140 + RR 40. Hepatomegaly on exam. CXR = large, box shaped heart with decreased pulmonary blood flow. Echo = apical displacement of septal leaflet of tricuspid valve and sail-like appearance of anterior leaflet. What EKG findings likely to be found?

Right bundle branch block - ebstein anomaly = apical displacement of septal tricuspid leaflet + leaflet dysplasia - peaked P waves in leads II and V1 - short or prolonged PR interval

History and physical examination of unimmunized boy with a febrile illness and rash is consistent with the diagnosis of measles. In hospital or ambulatory settings, patients with suspected measles should be immediately isolated and placed on

airborne transmission precautions - Immunocompetent patients with measles must be isolated until 4 days after rash onset, whereas infected patients who are immunocompromised should be isolated for the duration of illness - Measles is a highly communicable acute viral illness. It begins with a prodromal stage of 2 to 4 days that is characterized by fever and at least 1 of the "3 Cs" (ie, cough, coryza, conjunctivitis) - This is followed by an erythematous maculopapular rash appearing 2 to 4 days after the onset of fever. The rash begins on the face and head and then spreads to the trunk and extremities

Recommended tx for severe listeria

ampicillin + gentamicin

4 yo with retropharyngeal abscess. Empiric therapy with what antibiotic?

ampicillin-sulbactam or clindamycin - coverage for group A strep, oropharyngeal anaerobic bacteria and/or staph - in pts who do not respond to clindamycin or in pts with severe disease, vanc or linezolid should be added - continue parenteral treatment until pt is afebrile and clinically improved - oral therapy (amoxicillin-clavulanate or clindamycin) should be continue to complete 14 day course

Teen playing baseball with dizziness, dyspnea. Known asthma but not responding to albuterol. Anxious. Tingling paresthesias. Also has tachycardia. No wheezing, stridor, nasal flaring, accessory muscles currently. 1:1 insp:esp ratio. Next step?

capillary blood gas analysis - to assess hyperventilation syndrome - possibly anxiety

Newborn admitted to NICU for further w/u of abnormal extremities. Bilateral absence of radius, bilateral genu varum, coxa valga. Labs = thrombocytopenia. What abnormality is most likely to be found in this pt?

cardiac abnormalities - TARS syndrome - thrombocytopenia with absent radius - part of 1q21.1 deletion syndrome

Major criteria of RF

carditis erythema marginatum subcutaneous nodules polyarthritis chorea

Congenital heart dz associated with Turner syndrome

coarct - bicuspid aortic valve

LH and FSH levels in Turner syndrome

high FSH and LH

Platelets in ITP

normal and/or large sized platelet - no other changes in peripheral smear

6 yo Caucasian M + hx of recent skin infection + frothy urine + UA = 4g/day of protein and fatty casts. Electron microscopy of kidney biopsy = effacement of foot processes. Light microscopy of biopsy of pt's kidney will most likely demonstrate ...

normal glomeruli - minimal change dz - most common form of nephrotic syndrome

Vitamin A levels in carotenemia ...

normal or mildly elevated

Rs pattern over right precordium of infant is reflection of ...

normal right ventricular predominance for that age - QRS with tall R wave and smaller S wave over right precordium normal in young infants

The initial treatment for Lyme arthritis is

oral antibiotics for 28 days, amoxicillin <8 years of age, doxycycline ≥8 years of age

16 mo old male presents with bowing of legs. Has been healthy. Growth velocity is poor. He has fallen to less than 5th percentile in length. His mom is short and also has bowed legs. He has normal calcium (9) but low phosphorous (2). You suspect hypophosphatemic rickets. What is necessary to treat this disorder?

phosphorous and calcitriol - hypophosphatemic rickets = decreased renal absorption of phos at renal tubule via increased FGF23 effect in kidney = phos wasting and decreased a-alpha hydroxylase activity --> tx = phos and calcitriol --> most common form = X-linked dominant

Common variable immunodeficiency = nodular lymphoid hyperplasia. Splenomegaly. Panhypogammaglobulinemia. Chronic pulmonary infections, unexplained bronchiectasis, recurrent giardia. Therapy =

replacement of IgG with IV immunoglobulin - IgG goal >800

A mother calls the pediatrician to report that her 5-year-old daughter revealed last night that her 12-year-old brother has been coming into her room at night and asking her to touch his "privates." When questioned by his mother, the boy denies that this has happened. The mother plans to sleep in the same room as her daughter until an evaluation is completed. Of the following, in addition to scheduling an appointment for this child, the MOST appropriate next step is to

report to local CPS

Pt has meningococcal meningitis, with septic shock and DIC. What type of cells would you see on smear?

schistocytes

Schizophrenia symptoms with manic episode or significant depressive component ...

schizoaffective disorder - psychotic feature must be present at least 2 weeks without mood symptoms

Size of platelets in Wiskott-Aldrich syndrome

small

Kiddo has anemia, retic count 5-20%, jaundice, splenomegaly, and cholelithiasis. What would you find on peripheral smear?

spherocytes = small, dark, dense hyperchromic red cells without central pallor --> hereditary spherocytosis

Pts with hyper-IgE syndrome have recurrent infections with what bacteria?

staph aureus h influenza strep pna

A neonate born to a primigravida who had limited prenatal care and no medical problems is being evaluated in the newborn nursery. In the past 24 hours, the neonate breastfed 8 times, had 2 wet diapers, and 1 stool. On physical examination, her birthweight is 2 kg, length is 42 cm, and head circumference is 34 cm. She is awake. Her examination findings are remarkable only for overriding sutures. Creases are noted over the entire plantar aspect of the feet. Of the following, based on an assessment of her gestational age, this neonate is MOST likely to have

stiff ear cartilage - For the growth-restricted neonate, stiff ear cartilage is the physical examination finding most consistent with her gestational age. - Most neonates with low birthweight (<2,500 g) are born prematurely. Although she has low birthweight, this neonate's general alertness, full plantar creases, and feeding pattern are consistent with that of a full-term neonate. Overriding sutures may be noted among neonates with growth restriction.

Pts with measles at risk of ...

subacute sclerosing panencephalitis encephalitis pneumonia

Teenagers should sleep how many hours per night?

teenagers aged 13-18 years should sleep 8-10 hours per 24 hours

Contraindicated in a pregnant mom with UTI ...

tetracyclines

Tx of kiddo with otitis externa / otalgia in swimmer

topical combined abx and steroid drops - pseudo/staph coverage - ensure membrane is intact

A 5-day-old female neonate is brought to the office in response to a call from the state laboratory reporting a thyroid-stimulating hormone (TSH) level higher than 200 mIU/L (normal <30 mIU/L) on her newborn screen. The neonate was born via uncomplicated spontaneous vaginal delivery at 40 weeks' gestation with a birthweight of 3.5 kg. Her mother took levothyroxine for Hashimoto thyroiditis and prenatal vitamins during pregnancy. The neonate has been breastfeeding well on demand. Her vital signs are normal. She has mild jaundice and a small, reducible umbilical hernia. Her thyroid does not appear enlarged. The physical examination findings are otherwise normal. Confirmatory thyroid function tests show a TSH of 520 mIU/L (normal 0.6-5.6 mIU/L) and a free thyroxine of 0.5 ng/dL (6.4 pmol/L; normal 1.2-3.4 ng/dL [15.4-43.7 pmol/L]). Of the following, the MOST likely cause of this neonate's laboratory findings is

thyroid dysgenisis - Neonate has congenital hypothyroidism with a markedly elevated thyroid-stimulating hormone (TSH) level. - The most likely cause is thyroid dysgenesis, which is the most common cause of congenital hypothyroidism in the United States. The term "thyroid dysgenesis" includes the lack of or incomplete development of the thyroid gland as well as ectopic thyroid tissue. Ectopic thyroid tissue, which does not function normally, may be found anywhere along the path of embryologic migration from the base of the tongue (lingual thyroid) to its normal position in the neck. Thyroid dysgenesis most often occurs sporadically. - Although it is important for affected women to maintain normal thyroid hormone levels during pregnancy, maternal Hashimoto thyroiditis does not cause congenital hypothyroidism. - A confirmatory venous sample should be sent for TSH and free thyroxine (FT4) measurement when the newborn screen is positive for hypothyroidism. If the filter paper TSH level is higher than 40 mIU/L, guidelines recommend that levothyroxine be started immediately without waiting for the confirmatory test results. Additional workup should not delay treatment. Neck ultrasonography to detect the presence or absence of a normally positioned thyroid gland or a nuclear medicine thyroid scan to detect functional thyroid tissue, including ectopic tissue, may be considered. Pediatric endocrinology consultation is also indicated. - Treatment with levothyroxine 10 to 15 μg/kg per day should be initiated by 2 weeks of age. Liquid formulations are not available in the United States and levothyroxine should not be compounded because of dosing inconsistencies. Rather, the tablet should be crushed and given orally on a moistened finger or in a small amount of breastmilk, formula, or water. Levothyroxine should not be delivered via a bottle because of the medication's affinity for plastic and the potential for dosing inaccuracies. - Thyroid function tests should be monitored every 1 to 2 months during the first 6 months after birth, with a goal FT4 level in the upper half of the normal range and TSH in the normal range.

Side effects of SABAs ...

tremors tachycardias palpitations HYPOKALEMIA HYPERGLYCEMIA

Toddler' fracture is caused by ...

twisting / spiral or torque force on tibia during fall or when attempting to free foot after it becomes caught - tx = long leg cast; healing is rapid = 4-5 weeks

supracondylar humerus fracture causes hyperflexion at elbow results in injury to ...

ulnar nerve --> flexor carpi ulnaris - flexes and adducts the wrist joint

1 day old M + delivered via c-section at 39 weeks + no complications. Exam grossly normal, except for mass in left upper abdomen. Dx?

ureteropelvic junction obstruction - most common cause of mass in newborn period

The best screening test for Lesch-Nyhan syndrome is

urinary urate-to-creatinine ratio - will be greater than 2.0 in a child who is younger than 10 years of age - hyperuricosuria and hyperuricemia (serum uric acid concentration >8 mg/dL [476 µmol/L]), while often present, are not sensitive or specific enough to confirm the diagnosis

Goat's milk is deficient in ...

vitamin D vitamin B12 iron folate goat with a BIFD uvula

characterized by a prominent white forelock, wide nasal bridge, sensorineural deafness, heterochromia iridis, and in some types, lateral displacement of the inner canthi.

waardenburg syndrome

Femoral neck stress fracture should be considered in female athletes with groin pain. What is the management?

- Should not bear weight on the hip - Magnetic resonance imaging = definitive diagnosis

Infant with severe allergic contact dermatitis with secondary bacterial infection causing pustules from adult laundry detergent/fragrance. What to do?

1. Rewash all his clothes with Dreft at least 3 times 2. OTC hydrocortisone 2x/day after cleaning with fragrance free babywipe. Avoid all fragrance topical products 3. STOP vaseline as can clog pores which can worsen pustules and stop breast milk to avoid potential irritation 4. Cool compresses to soothe areas

metabolic syndrome is defined as 3 of any of the following ....

1. abdominal obesity / waist circ >90th %ile 2. serum triglycerides >150 3. serum HDL <40 4. BP >90% for age and sex 5. fasting plasma glucose >100

Invasive meningococcal disease is a vaccine-preventable disease. Routine immunization with quadrivalent meningococcal conjugate vaccine (MCV4) is recommended for all adolescents at what ages?

11 through 12 years of age followed by a booster dose at 16 years of age - Adolescents who receive the first dose at age 13 through 15 years should receive a booster dose at age 16 through 18 years - A booster dose is not recommended for adolescents who receive their first dose of meningococcal conjugate vaccine at or after 16 years of age - Administration of serogroup B meningococcal vaccines is a consideration for immunocompetent individuals aged 16 through 23 years

Meningococcal conjugate vaccine (MCV4) typically given in 2 doses:

11-12 yo 16 yo - If first dose at 13-15 yo, need booster at 16-18 yo - If first dose at or after 16 yo, no need for booster

Peak height velocity occurs at what age in girls? ...

11.5 years in girls - 13.5 years in boys

The timing of endoscopy in symptomatic pts with alkali ingestion remains controversial, but most experts agree on initial eval within ...

12 and 24 hours

Hepatitis B vaccine should be administered within how many hours of birth to all neonates, including those weighing less than 2 kg born to mothers with a positive hepatitis B surface antigen?

12 hours of birth

Fine/mature pincer grasp is achieved by ...

12 mos

Posterior lateral fontanelle closes by ...

12 mos

Until what age should infants sleep supine?

12 mos

Hypertrophic pyloric stenosis usually begins at what age?

3-6 weeks old

15 month old can say how many words?

3-6 words

Female in NICU + covered in thick layer of vernix + slightly raised areola but no breast tissue palpated + incurving of ear only in superior portion + ear cartilage scant + pinna returns slowly from folding + 2 anterior sole creases present + lanugo covers most of body but is absent from face. Most likely gestational age of patient?

34 weeks

Incurving of 2/3 of pinna appears when gestaional age wise?

36-38 weeks

Tx of adrenal crisis requires prompt admin of IV fluids with dextrose and stress doses of steroids. Stress doses of hydrocortisone are provided at ...

50-100 mg/m^2

Novel rapid screening test for neonatal sepsis administered to 183 newborns, 80 with positive bacterial blood culture and 103 with negative bacterial blood culture. 41 of infants with positive blood culture has positive screening test, while 95 of infants with negative blood culture had negative screen. Blood cx is considered gold standard for determining disease state. Sensitivity of novel rapid screening test?

51.3%

What size ingested foreign body cannot pass through the stomach?

5cm

Child can sit momentarily without support propped on their hands at what age?

6 mos

Rolling from back to front is usually achieved by ...

6 mos

18 yo teen + pregnant after first sexual encounter + soon after birth, newborn is found to be jaundiced + child has bili of 13 + blood smear with spherocytes + mom's blood type is O, Rh-. Newborn's blood type is A, Rh+. Most likely cause of hyperbilirubinemia?

ABO incompatibility

Associated with Coombs-positive (direct Ab test positive) neonatal jaundice ....

ABO incompatibility

Tx of dilated cardiomyopathy

ACEI + diuretics

Recognize papular acrodermatitis

AKA gianotti crosti - koebner phenomenon

Craniosynostosis + bilateral syndactyly ..

Apert syndrome

12 yo Caucasian girl + 3 mo hx of joint swelling, fatigue, facial rash, worsened by sunlight exposure, is admitted because of febrile illness. She has had multiple hospitalizations in past for pyogenic infections. Vitals: BP 160/100; P 110; RR 20; T 104 F. She appears in moderate distress. PE reveals generalized pallor, cyanosis of hands with pruritic ulcerated lesions of finger tips, malar facial rash, bilateral warmth and swelling of both knees and ankles. Labs - WBC 3,000 - Hgb 6 - Platelets 100,000 - ESR 160 - CH50 0 - UA: 4+ protein, 3+ blood Cause of pyogenic infections?

C2 complement deficiency - has SLE + this deficiency (increased risk with SLE) - increased risk of strep pneumo, neisseria, h influenza

What vertebral levels exhibit pseudosubluxation in almost half of pediatric patients?

C2-C3 - common variant - seen in 50% of kids < 8 yo

The mother of a 16-year-old adolescent girl calls her pediatrician to discuss concerns. Her daughter has had epilepsy since she was 8 years old. The epilepsy has been well controlled on levetiracetam until last month when she had a breakthrough seizure while at a sleepover. Since then, she has become more withdrawn, refusing to go out with her friends or spend time with her family. She is more tearful and emotional and makes frequent statements that no one understands her. She reports no suicidal ideation. She has been reporting headaches on a daily basis, which are constantly present despite treatment with over-the-counter nonsteroidal anti-inflammatory drugs and acetaminophen. She has maintained excellent grades despite multiple headache-related absences over the past month. The mother reports that her daughter was about to get her driver's license when she had the breakthrough seizure, and now she must wait to be able to drive. The mother has become more careful, ensuring her daughter takes her medication as directed and checking on her more frequently. Tearfully, she shares that she is fearful that when her daughter goes to college she will not be able to take care of her epilepsy and will have more seizures. Of the following, the BEST next step for this patient is

Counseling to develop coping strategies = Impact of living with a chronic health condition. - Developmentally, adolescents are establishing their independence, often accomplished through pushing limits and having a sense of invincibility and an expanding social circle with increased impact of friendships and relationships outside the family. - Chronic illness, defined as a condition lasting 3 months or longer, can have significant impact on an adolescent's physical, emotional, intellectual, and social development, as well as shape and define relationships with parents and siblings. - Medication nonadherence, changes in lifestyle (ie, staying up late, tobacco or alcohol use), and physiologic changes can all impact control of a previously stable health condition. - During a seizure, there is the potential for physical harm, social embarrassment, and sudden unexpected death in epilepsy. These potential risks lead to anxiety related to when the next seizure may occur. In addition, laws and activity restrictions created to increase patient safety, including driving restrictions related to the level of seizure control, can have a significant impact on functional quality of life.

10 yo M + referred to genetic testing for long hx of recurrent infections, including pna, rhinitis, suppurative cervical LAD, dermatitis, enteritis. Over time his cultures yielded staph aureus, serratia, E coli, pseudomonas. Biopsy of lymph nodes with shows granulomatous lesions. Serum analysis reveals elevated immunoglobulin levels. What will be helpful to confirm suspected dx?

Deficient nitroblue tetrazolium dye reduction in neutrophils - CGD

5 yo girl presents with highly pruritic rash on her left foot, which appears to be spreading. It was first noticed after returning from a family vacation in the Florida Keys where she spent a lot of time playing in the sand and swimming in the ocean. PE reveals raised, excoriated, erythematous, and well-demarcated papules distributed in snake-like pattern over dorsum of foot. Most likely cause of pt's clinical signs and symptoms?

Dog hookworms - cutaneous larva migrans - tx = oral albendazole or ivermectin for children >/= 2 yo or >/= 15 kg body weight - topical therapy for those < 15 kg - itching tx = hydroxyzine vs benadryl

Recognize kerion, which is alopecia, redness, exudative discharge, and bogginess / an acute local inflammatory response to a tinea capitis infection --> the tx is:

Griseofulvin orally - a benzofuran - systemic antifungal treatment of choice for kerion and other noninflammatory tinea capitis infections of the scalp

What should be used to find tumor in opsoclonus-myoclonus syndrome ...

MRI of chest, abdomen, and pelvis - associated with neuroblastoma

Recommended to evaluate pts with rapid increase in HC >2cm per mo in infants <6 mos ...

MRI of head

Likelihood of disease after the result of a diagnostic test is known

Posttest probability

Oral rehydration is the first-line treatment for pts with mild to moderate rehydration. What is recommended?

Rapid oral rehydration over *3-4 hours* with a solution like Pedialyte

4 yo F + 24 hours of fever, fussiness, right otalgia. Hx unremarkable. PE = rhinorrhea + rt tympanic membrane dull yellow and bulging. Pneumatoscopy = movement on positive pressure only. Most likely cause of symptoms?

Right acute otitis media - bulging /fullness TM = most sensitive and specific finding - opacity or dullness of TM - redness or yellow discoloration of TM - middle ear effusion - decreased movement on otoscopy

Teen with misuse of androgenic steroids (taking in cycles of 6-12 weeks = stacking/pyramiding). Orthopedic complications include ....

acceleration of maturation with early epiphyseal closure and decrease in ultimate adult height

Barium enema of Hirschsprungs

aganglionic segment is distal narrowed segment, normal ganglionic segment is dilated proximally

Floppy baby. 12 mo old. Dev'tal delay hx. PE with: truncal hypotonia, head lag, slippage on vertical suspension, mild appendicular hypotonia. Strength normal and symmetric. Next step for dx?

brain MRI - brain vs genetic prob - not muscle or nerve prob (weak along with floppy usually)

Should be suspected in child with tetralogy of fallot who is cyanotic with new or different headache or new neuro symptoms ...

brain abscess

Girl is 4 years of age. At this age, expected fine motor skills include

copying a square tying single knots cutting a 5-inch circle using tongs to transfer writing part of the first name imitating a gate with cubes drawing a 4- to 6-part person

Chlorinated water exposure for young boy. Diarrhea follows. Dx?

cryptosporidium parvum

Newborn's NBS positive for high levels of phenylalanine. Impaired tetrahydrobiopterin regeneration. What enzyme is deficient?

dihydrobiopterin reductase

For children ages 12 years and older with mild persistent asthma, the 2020 focused updates include a new option for intermittent ICS use. Providers can choose to

either - prescribe daily low-dose ICS with as-needed SABA reliever or - intermittent use of ICS with concomitant as-needed SABA reliever only with wheezing An example of the intermittent approach would be 80-250 mcg of beclomethasone (or equivalent ICS) and two to four puffs albuterol (SABA) used every four hours as needed.

Polyhydramnios is associated with esophageal atresia, characterized by...

excessive drooling, coughing, choking, inability to feed - aspiration common

Hypoketotic hypoglycemia is a hallmark of

fatty acid oxidation disorders

Tularemia treatment

gentamicin or streptamycin

Teen has multiple café-au-lait spots, favoring a diagnosis of neurofibromatosis type 1 (NF-1). The associated headaches, flushing, sweating, tachycardia, and hypertension suggest

high level of catecholamine production --> pheochromocytoma

Acute sinusitis following a viral upper respiratory tract infection (URI) is the most common presentation for acute sinusitis in children. Acute or subacute sinusitis should be considered for children who develop new fever and worsening congestion after initial improvement, or whose viral URI symptoms do not resolve after 10 to 14 days. The American Academy of Pediatrics clinical practice guidelines for diagnosis and management of acute bacterial sinusitis recommend treatment with

high-dose amoxicillin or amoxicillin-clavulanate

neonatal thyrotoxicosis can cause

high-output cardiac failure

Often associated with spina bifida ...

hydrocephalus on brain MRI - as a consequence of Chiari II

Prader-Willi syndrome is caused by the lack of expression of paternally inherited genes in the imprinted region of chromosome 15 at 15q11.2-q13. The diagnosis is made when

methylation analysis of this Prader-Willi critical region identifies only the maternally inherited methylation pattern

Methemoglobinemia tx ...

methylene blue

What occurs in most subluxed (loose) teeth?

passively return to their anatomic position - should still urgently see a dentist

You are on standby in the delivery room to a newborn term infant that was just delivered by a lower segment Cesarean section. Indications for Cesarean section were fetal distress. At birth, there is meconium staining, but the infant is breathing and has a heart rate of 105/min. What is your first step in the care of the infant?

tactile stimulation - In an infant who is spontaneously breathing, current American Association of Pediatrics Guidelines on newborn resuscitation recommend drying and tactile stimulation via rubbing the back.

Most common complication of oligoarticular JIA ...

uveitis - can cause blindness

Most common trigger of asthma exacerbations in children?

viral URI's

Newborn + raised areola + superior incurving of ear + scant ear cartilage with slow return from folding, and 2-3 anterior sole creases. Most likely gestational age of patient ...

34-35 weeks - breast tissue absent and areola barely visible up to 34 weeks gestation - ear cartilage absent until 32 weeks - scant amounts of cartilage with slow return from folding = 32-35 weeks - superior incurving at 34-35 weeks - soles of feet: 2-3 creases at 34-35 weeks

The areola is first raised at what age infants?

34-35 weeks = 1-2 mm of breast tissue first palpable at 36-37 weeks

Recognize retinitis pigmentosa, which is associated with abetalipoproteinemia

= acanthocytes on peripheral smears - AR - can result in blindness - inability form very low-density lipoprotein and chylomicrons - steatorrhea - intellectual disability - ataxia

Recognize hot tub folliculitis

= pseudomonas folliculitis - develops within 24-48 hours of exposure - numerous, discrete, sometimes tender, pruritic papules, nodules, and/or papulopustular lesions - often concentrated over areas typically covered by bathing suits - linked to hot tubs, whirlpools, spa pools, swimming pools, water slides - most people require supportive therapy --> resolves over 1-2 weeks

TEN percentage

>30% detachment

If MMR is given, IVIG administration should be delayed by how many weeks?

???

Rocky mountain spotted fever - Na: - Platelets: - Liver enzyme:

- Na: decreased - Platelet: decreased - Liver enzymes: increased

3 yo admitted to PICU from ED d/t extensive burns to chest, face, and back via pulling pot of boiling water off stove. He receives fluid resuscitation. Purple lesions noted below level of burns. CBC - WBC 12000 - Hgb 10.2 - Platelets 32,000 Labs most consistent with this diagnosis? - PT, PTT: - Fibrinogen: - Other RBC shapes

- PT, PTT increased - Fibrinogen decreased - schistocytes DIC d/t burns - thrombocytopenia and purpura

Recognize bohn nodules

- whitish or clear mucous gland cysts - along the alveolar ridge in newborns

Ophtho referral if infant unable to fixate and follow objects by what age ...

3 mos

The boy in the vignette has otitis media with effusion (OME), supported by the findings of clear fluid behind a normal-appearing tympanic membrane and no fever. This condition likely began after an upper respiratory tract infection. Otitis media with effusion occurs often in toddlers and school-age children, especially those with allergic rhinitis or eustachian tube dysfunction. It is commonly self-limiting, with resolution within

3 mos - Otitis media with effusion is typically self-limiting and usually resolves within 3 months

Recognize chiari malformation type I

- descent of tonsils into foramen magnum - suboccipital headaches

Recognize Brachysyndactyly

- description of fingers and toes that are abnormally short and syndactyly of fingers that are webbed

For anaphylaxis, the epi solution concentration used for IM dosing is ...

1mg/ml

Male child + runs without falling + goes upstairs using rail, alternating one feet at a time. Age?

30 mos - build 8-cube tower - wash hands - name objects by use

Creases in soles do not appear until what gestation age?

32-33 weeks gestational age

Cephalohematoma

- 24 hours after birth usually - can increase in size over next days - resolves in appx 2 weeks - doesn't cross suture lines - large --> increases risk of jaundice

Complex febrile seizure dx

- >/= 15 minutes - >1 episode in 24 hour period - focal symptomology

Recognize Brugada syndrome

- AD - coving ST segment on EKG - unstable arrhythmias at low HR - consider implantable defibrillator

To declare brain death physician must document:

- Absent cortical function: No spontaneous movement or response to voice or painful stimuli - Absent brainstem function: Absent pupillary response with fixed and dilated pupils, absent corneal reflex, cough or gag, and absent oculocephalic (doll's eyes) and oculovestibular (cold calorics) reflexes - Apnea test: No spontaneous respiratory effort with a documented Pco2 above 60 mm Hg and greater than 20 mm Hg change above baseline Pco2

Recognize transposition of great arteries

- infant presents with central cyanosis since birth and has features of congestive cardiac failure (tachypnea, tachycardia, hepatomegaly, cardiomegaly) - diagnosis is supported by the radiograph of the chest showing cardiomegaly with a narrow base and plethoric lung fields - right upper lung field appears more plethoric than other areas - cardiac silhouette has an "egg on side" appearance, typical of TGA - infants with intact ventricular septum present with severe central cyanosis at birth - ECG shows right axis deviation and right ventricular hypertrophy

acute chest syndrome in sickle cell can happen via ...

- infection - infarction - atelectasis - fat embolism from bone marrow

mechanism of topiramate ...

- inhibits sodium channels - enhances GABA-a receptors - antagonizes kainate at AMPA receptor

Recognize epidural hematoma

- initial decreased level of consciousness, followed by period of normal activity last 4-6 hours (lucid interval), then rapid deterioration

Recognize herpangina

- limited to mouth - coxsackie A1-6, 8, 10, 22

Child has hyponatremic dehydration caused by excessive sodium loss from vomiting and diarrhea, exacerbated by attempted rehydration with hypotonic fluids (popsicles and water). This condition results in - serum sodium: - urine sodium: - urine osmolality:

- low serum osmolality - low urine sodium - high urine osmolality

Recognize Necator americanus

- microcytic anemia and peripheral eosinophilia - history of barefoot outdoor play in rural Indonesia are highly suggestive of a Necator americanus (hookworm) infection, which is transmitted through soil in endemic regions - Necator americanus is prevalent in North America, South America, Central Africa, parts of India, South Pacific Islands, and Indonesia - Treatment choices for hookworm include albendazole, mebendazole, and pyrantel pamoate

Heat exhaustion

- mild to moderate temp increase - no mental status changes - can have muscle cramps, tachycardia, decrease in BP

By definition NF1 must have 2 of the following...

1. >/= 6 cafe au lait macules measuring >5 mm in greatest diameter in prepubertal individuals and >15 mm in greatest diameter in postpubertal individuals 2. >/= 2 neurofibromas of any type or >/= 1 plexiform neurofibroma 3. axillary or inguinal freckling 4. >/= 2 iris hamartomas (Lisch nodules) 5. optic glioma 6. distinctive osseous lesion, such as sphenoid dysplasia or thinning (bowing) of long bone cortex (with or without pseudoarthrosis) 7. 1st degree relative with NF1

5 yo can name how many colors?

10

Infant has laryngomalacia, which will likely resolve without intervention by what age usually?

12 to 18 months - Evidence of good feeding and growth, normal oxygen saturation, and lack of respiratory compromise are encouraging findings for a benign course - Because the stridor is often dynamic, it may not be as obvious at an office visit as it may seem to the parents during the night at home - Inviting parents to bring audio/video recordings of the breathing pattern to an office visit is a way to make sure they feel heard and understood

AAP recommends universal screening for depression starting at age...

12 yo

Most trisomy 21 (except for cases of involving translocation or mosaicism) arise via nondisjunction in one parent during meiosis 1 or 2. RFLP yields info on DNA sequence differences between individuals. In what percentage of cases do extra chromosomes originate from the father?

<5%

5 yo M + 3 weeks of dry eyes, dry mouth, arthralgia, dysuria. Denies fever, wt loss, other recent infections. PMHx unremarkable. Appropriate diagnostic test?

Biopsy of salivary gland - Sjogren's - lymphocytic infiltration of exocrine glands

Sudden onset of symptoms in associated with low hemoglobin and increased reticulocyte count is consistent with hemolytic anemia, like in G6PD deficiency. What is most likely to be seen on unstained peripheral blood smear?

Bite and blister cells

50-90% of kids can pull to a stand by what age ...

9 mos

Ketamine is contraindicated in what age range?

<3 mos - relatively contraindicated in less than 1 yo

DRESS syndrome: viruses that play a role in reactivation of this?

HHV6 HHV7 CMV EBV

Adolescent men having sex with men should be screened at least annually for

HIV syphilis chlamydia gonorrhea

Pt is prescribed a bactericidal abx that binds to aminoacyl site of 16S ribosomal RNA with 30S ribosomal subunit. Blocks initiation of protein synthesis, leading to misreading of genetic code and inhibition of translocation. This abx is in ineffective against what group of bacteria ...

anaerobes - aminoglycosides --> transport into cell oxygen dependent

Failure of fusion of anterior neuropore leads to ...

anencephaly and encephalocele

Maternal medications associated with gastroschisis ...

cocaine vasoactive meds (nicotine, pseudoephedrine)

Clonidine can worsen ...

depression

Tx of eosinophilic esophagitis

dietary elimination/modification PPI topical steroids

Lower relapse rate for long-term therapy for kids with nocturnal enuresis ...

enuresis alarms - requires time commitment of at least 3 mos

16 yo F + dysphagia + normal esophagram + endoscopy = white exudate on esophageal mucosal with linear furrowing. Dx?

eosinophilic esophagitis

4 yo girl + 24 hr hx of mildly pruritic rash. Pink patches and edematous plaques with dusky centers that appear to look like "targets." Distributed symmetrically on distal extremities, including palms and soles. Pt has a cough and fever a week before rash appeared. What clinical scenario most likely preceded patient's rash?

Grouped vesicles and "punched out" erosions on erythematous base of orolabial mucosa - erythema multiforme - usually preceded by HSV - self-limiting - lesions clear in 1-3 weeks

1 yo + hypersegmented neutrophil + primarily drinks goat's milk. Most likely dx?

folate deficiency - goat's milk does not have enough folate for growing infant

In tylenol overdose, metabolic enzyme sites are saturated and the elimination of tylenol shifts in what direction?

from first-order to zero-order kinetics - in zero-order kinetics, rate of metabolism if independent of plasma concentrations = buildup of drug concentrations higher than expected - in overdose, kinetics switch from first order to zero order kinetics

Dx of tinea versicolor via ...

KOH prep

tinea versicolor confirmed by ...

KOH prep

17 yo M + undergoing renal transplant + within few minutes of implantation and connection of pt's vascular supply to new kidney, surgeon notices organ is poorly perfused. Donor's blood type is B-negative, while recipient is O-positive. What is contributing to ischemic event?

type II hypersensitivity rxn - hyperacute rejection - pre-existing recipient Abs

Serum sickness is what type of reaction?

type III

Recognize CXR of ebstein anomaly

typically presents with "wall to wall" heart

15 yo Black male presents to clinic with fatigue and increased thirst. Post-prandial glucose was 205 with other normal chemistry panel. No evidence of acidosis or ketones. Morning fasting sugar was 150 and A1c was 8.4%. You diagnose T2DM. Parents ask about management. Most appropriate initial step in management?

Metformin + diet and exercise - no metabolic decompensation - management dictated by A1C - A1C <8.5% --> 1st line medical therapy = metform and lifestyle modification - A1C >8.5% --> insulin in addition to metformin --> after establishing negative pancreatic autoAbs, titrate insulin and consider liraglutide

14 yo M + myopathy + mom had similar symptoms but less severe + maternal aunt and maternal grandmother also with symptoms. Father denies symptoms nor on his side of the family. Most likely inheritance?

Mitochondrial inheritance - Isolated myopathy - mom passes on to all her children

5 yo girl, new to your practice, presents for wellness exam with hx of chronic diarrhea. Her mom enforces strict vegan diet and "doesn't believe in vitamins or fortified products." She is <3 %ile for both height and weight. However, no prior growth charts are available. He exam is significant for alopecia and vesiculobullous rash around her mouth and anus. What nutrient deficiency is most likely responsible for this child's symptoms?

zinc - oral zinc supplementation leads to rapid improvement of symptoms

The evaluation of an adolescent with delayed puberty should include a comprehensive history and physical examination focusing on indicators of potential causes of the delayed puberty. First-line laboratory testing includes

gonadotropin (luteinizing and follicle-stimulating hormones) and estradiol (in girls) or testosterone (in boys) levels - Additional testing should be directed by the history and physical examination findings - A bone age radiograph may be helpful especially if short stature is present since it can predict catch-up growth. A delayed bone age, however, may be present with any cause of delayed puberty. Bone age correlates better with pubertal status than chronological age.

High P, Low Ca typical for

hypoparathyroidism

PTH concentration normal in what form of rickets?

hypophosphatemic rickets - increased renal loss of phosphate - most common rickets in North America - rachitic rosary type more common in males

Antibiotic prophylaxis decreases the risk of recurrent urinary tract infection (UTI) in children with vesicoureteral reflux (VUR); prophylaxis is indicated for children with

low-grade VUR with recurrent UTI grade III or higher VUR or non-toilet-trained children with any grade of VUR - Trimethoprim-sulfamethoxazole (TMP/SMX) is preferred for prophylaxis in children with vesicoureteral reflux because of its bactericidal action, despite a first urinary tract infection with an organism showing resistance to TMP/SMX. - Amoxicillin is recommended for infants younger than 2 months when prophylaxis is indicated to prevent urinary tract infection.

Type II Crigler-Najjar Syndrome is responsive to

phenobarbital

Encephalopathy is usually found in what stage of tylenol toxicity ...

stage 3 - 3-5 days after ingestion with recurrence of anorexia, nausea, vomiting, malaise - hypoglycemia = stage 3 too

Know the MenACWY-CRM (Menveo) schedule for infant, like a 2 month old, with a hx of splenectomy...

starting at 2 mos, is licensed for infants as a 4-dose series = 2, 4, 6, 12 mos

How does the measles rash distribute?

starts on face, behind the ears and hairline --> spreads down the neck, trunk, upper and then lower extremities - spares palms and soles - lasts 4-6 days

Exposure to thalidomide at 35-50 days of gestation is associated with

limb defects, including missing arms and/or legs and ear malformations with deafness

G6PD occurs more often in what gender ...

male

Pupils in opioid overdose ...

pinpoint pupils

mycobacterium avium-intracellulare usually results from coming in contact with organisms in environment (by playing outside in dirt). Rubbery node slowly increases in size and acquires violaceous discoloration of overlying skin. Cure is ...

total excision

What env'tal allergies are worse in the spring

trees - ragweed = worse in fall

You suspected inborn error of metabolism in infant. What lab has highest clinical yield to confirm suspected diagnosis?

urine ketones - no or few ketones = FA oxidation defect - excess ketones = glycogen storage disease

When should you screen for incipient diabetic nephropathy in new diabetes diagnosis?

urine microalbumin at initial diagnosis of T2DM 5 years after initial diagnosis of DM Type 1 - yearly screenings should then be performed

cellulitis-adenitis syndrome is a form of late-onset ...

S agalactiae infection (GBS) - 3-4 weeks old usually - bacteremia without a focus

3 yo + diffuse, erythematous, pruritic rash on chest, trunk, palms, soles. 7 days into course of amoxicillin for ear infection + initially developed URI symptoms before developing ear infection. Cause of rash?

Type IV hypersensitivity reaction - exanthematous drug eruption 2/2 delayed-type, T cell-mediated (type 4) reaction - involves palms and soles - itching - low-grade fever - eosinophilia

Initial step in management of suspected septic arthritis of the hip.

US

PDH deficiency is inherited ...

X-linked recessive or AR - ketogenic diet + thiamine supplementation - PDHA1 mutation - lactic acidosis, intellectual disability, ataxia, spasticity

Recognize EKG of anomalous origin of left coronary artery ...

anterior ischemia= ST changes in anterior leads, V1-V4, and Q waves in L, V5, V6 - present in first few mos - sx = only definitive tx

Most frequently injured ligament in ankle sprain ...

anterior talofibular ligament tear - anterior drawer test

Hereditary spherocytosis mechanism

abnormality of structural proteins ankyrin and spectrin - most common congenital hemolytic anemia in northern European ppl - cells less pliable - Coombs negative hemolytic anemia - small spherocytes - anemia - reticulocytosis - increased MCHC - positive osmotic fragility test - can have chronic hemolytic anemia + jaundice and splenomegaly - complications = cholelithiasis

In those with congenital growth hormone deficiency, linear growth becomes abnormal at what age?

about the age of 6 to 12 months - Patients with acquired growth hormone deficiency should undergo investigation for a brain neoplasm and other pituitary hormone deficiencies. - The weight-for-length or body mass index curve is important to consider in narrowing the differential diagnosis of abnormal growth.

A 12-year-old girl is being evaluated for tooth pain that started a few days ago, but worsened yesterday. She has left upper molar pain with localized gingival swelling; the pain is making it difficult to eat. Her vital signs include a temperature of 38.3°C, a heart rate of 105 beats/min, and a respiratory rate of 17 breaths/min. Her mouth has an area of redness, swelling, and fluctuance along the maxillary left molar, which is tender to the touch. Of the following, the MOST likely cause of this infection is

actinomyces israelii - Dental infections are associated with anaerobic bacteria. Actinomyces israelii is an anaerobic bacteria

Most frequent cause of death in children with sickle cell

acute chest syndrome - newborn screen = FS pattern - tx = macrolide + 3rd generation cephalosporin bc of overlap with pna

A penetrating eye injury with ring abscess formation in cornea is indicative of ...

bacillus cereus - pseudomonas and proteus also present with ring abscess formation - tx = intravitreal vancomycin

Labs in Menkes disease

decreased serum copper levels - X-linked recessive - impaired absorption and transport - intellectual disabilities - collagen abnormalities

Murmur of hypertrophic cardiomyopathy does what in squatting position?

decreases

Kiddo has hx of decreased urine output associated with HT and secondarily infected lesions that are descriptive of impetigo and hematuria. Dx of acute poststreptococcal glomerulonephritis (APSGN). Dx supported by ...

elevated levels of streptococcal Abs = elevated DNase B Ab (cutaneous strep infection)

Young females undergoing tx with minocycline appear to be at particular risk of developing autoimmune hepatitis, which has also been linked to tx with isoniazid, nitrofurantoin, and sulfonamides. Associated with what labs?

elevated serum transaminase levels normal alk phos direct hyperbili hypergammaglobulinemia

Teen has Wilson disease (hepatolenticular degeneration), an AR disorder of cellular copper export caused by defective mobilization of copper from lysosomes for excretion into bile. This defect leads to ...

elevated urine copper and copper accumulation in liver - chromosome 13 affected = elevated serum transaminases, decreased serum levels of ceruloplasm (carrier of copper in serum), decreased serum copper (due to decreased ceruloplasm), and increased urinary concentrations of copper

Appropriate test for child with 3 weeks of back pain and tingling in her hands...

emergency MRI of the spine

Indicated in cases of acyclovir resistance for HSV ...

foscarnet and cidofovir - occurs in immunocompromised pts

describe type 4 salter harris fracture

fracture through physis, adjacent metaphysis, epiphysis

Radiograph of Blount dz will show ...

fragmentation of medial epiphysis of tibia

Most specific finding of acute otitis media ...

fullness of bulging of tympanic membrane

Conversion disorder is now known as

functional neurological disorder

Adolescent is experiencing a hypersensitivity reaction and anaphylaxis after the administration of a medication aimed at addressing acute-on-chronic heart failure. Given his known allergies (morphine, vancomycin, and sulfamethoxazole-trimethoprim), what drug could elicit an allergic reaction because of his known allergy to sulfa medications?

furosemide - sulfonamide and can induce hypersensitivity reactions in individuals with known allergy to sulfa medications - loop diuretic commonly used to reduce intravascular volume and peripheral edema in children with congestive heart failure - inhibits sodium reabsorption by acting on the thick ascending limb of the loop of Henle, resulting in increased sodium excretion and free water loss

Most common complication of chronic suppurative otitis media ...

permanent hearing loss

Premie born at 33 weeks in a taxi. In the ER, a baby is noted to have temp of 35 degrees celsius. Child should be placed ...

under a radiant warmer at preferred skin temp

Know Amsel Criteria

- includes multiple clue cells on wet preparation - thin white discharge - pH > 4.5 - fishy odor = BV

Children typically learn to hold a crayon around what age?

12 months of age - scribble at 14 to 15 months of age - Children may begin stacking cubes around 12 months of age

Separation of physis describes

Type I Salter Harris fracture

AAP recommends children sit in rear seat of all vehicles until they are ...

13 yo

Although AAP recommends breastfed infants receive vitamin D supplementation, wha infants are the except to this?

Williams syndrome - 15% of pts with Williams syndrome will have hypercalcemia - recommended that multivitamin not be given to kids with Williams syndrome bc of vitamin D component

rooting reflex disappears when?

3-4 mos

Anterior lateral fontanelles close by

3 mos

Age: can draw triangle

5 yo

12 yo boy at pediatrician. Hx of recurrent fevers, conjunctivitis, joint pain, urticarial rashes. Attacks last for 1-2 days every few months. Between attacks, child appears to function normally. For past month, child has developed some hearing loss. Should courses of oral prednisone curtain fevers and rash, but hearing has not improved. Despite symptoms, child has been able to maintain average growth and dev't milestones throughout the years. Fam hx is s/f several cousins with similar complaints of fevers and rashes. Rinne and Weber tests reveals bilateral sensorineural hearing loss. Best additional tx for this pt?

Anakinra - Muckle-Wells syndrome = rare genetic disorder = cryopyrin-associated periodic syndromes (CAPS) - progressive renal failure d/t amyloidosis from longstanding inflammation - cryopyrin --> activation of IL-1beta --> IL-1 inhibitors = rilonacept, anakinra, canakinumab

What is recommended before removing live insect from ear ...

lidocaine or mineral oil --> will kill the insect

Observational studies that calculate relative risk from an exposure (incidence in exposed/incidence in unexposed)...

Cohort studies

What gene defect is responsible for achondroplasia? ...

GAIN of function mutation in FGFR3

Eye infection most likely in first 2-5 days of life...

N gonorrhea

Precautions for measles

airborne

When should pregnant women get Tdap?

between 27 and 36 weeks gestation

observational studies that can determine the prevalence of a particular outcome or disease

cross-sectional study

Meconium aspiration results from

intrauterine stress

Associated with green fecal mass obstructing ileocecal area ...

meconium ileus

Most common form of impetigo ...

non-bullous impetigo

Staph aureus from deviled eggs. What cause symptoms?

preformed enterotoxins

Most common manifestation of Turner syndrome and may be the only presenting feature.

short stature

First-line therapy for ADHD

stimulants

Avoidance of weight-bearing after a fall in a toddler is classic presentation of "toddler's fracture," resulting from twisting of the tibia. Tx?

long-leg cast

Poorly controlled maternal phenylketonuria is associated with

microcephaly growth restriction congenital heart disease developmental delays

Anti-seizure medication with side effect of acute-onset glaucoma

topiramate - other side effects = somnolence, fatigue, psychomotor slowing, cognitive disorders, nephrolithiasis

Recognize nevus sebaceous

- has small risk of growth developing within lesion after puberty - can develop thickening and secondary growth - malignancy is rare but can occur (usually basal cell carcinoma) - no hair / alopetic - will not resolve with time

Ibuprofen is an appropriate abortive treatment for acute migraine attacks. If there is no improvement, what can be added?

triptans

Showed improvement in phase II trials in acne scarring in patients with no active acne lesions

0.3% adapalene monotherapy

Spoon feeding (with spilling) is typically attained by what age?

15 months of age

At what age can 90% of kids walk unassisted?

15 mos

Recommended for health children 12-23 mos who have not previously received PCV13...

2 doses (at least 8 weeks apart) of PCV13

Varicella vaccination for those older than 13 yo ...

2 doses of varicella vaccine separated by at least 28 days in those unvaccinated

Use refrigerated breast milk within

4 days

A mother brings her infant daughter for a health maintenance exam with questions regarding the child's development. The daughter holds her head steady and unsupported, pushes down on her legs when her feet are on a hard surface, turns toward her mother's voice, smiles spontaneously, has begun to babble, and brings objects to her mouth. What age is most consistent with this patient's development?

4 months - Pushes down on legs when feet are on a hard surface - May be able to roll over from tummy to back

Colic can be expected to improve by...

4 months - first 6 weeks = cries 2-3 hours per day - 10 weeks = 1-2 hours per day - beyond 5 months --> look for other causes

rooting reflex, which aids infant in attaching to nipple, is present at birth and is typically suppressed by...

4 months old

Ability to lift head 90 degrees when prone appears at what age ...

4 mos

Age: can roll front to back + sit with trunk support + laugh out loud

4 mos

Age: can copy flag drawing

6 yo - also: tandem walk, ties shoelaces *think of upside down 6*

Aplasia cutis congenita and limb reduction defects are seen in what syndrome?

Adams-Oliver syndrome

Acute respiratory failure is caused by any clinical condition that impairs the pulmonary system and may result in hypoxia, hypercarbia, or both. The most common cause of cardiac arrest in children is an acute respiratory process; thus, reversal of acute respiratory failure is critical in preventing cardiac arrest in children. Wat may be warranted when there is hemodynamic stability and no clear contraindication?

A trial of noninvasive positive pressure ventilation - for acute respiratory failure

Mother of 4 yo girl states her daughter has complained of painful urination over last 2 days. On morning of presentation, she noticed blood on her daughter's underwear as she dressed her for preschool. No hx of fever or other systemic symptoms. After school, she attends a day care center and is cared for on the weekends by her grandparents. She has had no contact with her biological father since her parents' divorce 2 years ago. On PE, there is a friable, erythematous, donut-shaped mass extruding from the urethra. No vaginal discharge. Most appropriate next step in tx of this pt?

Application of topical estrogen cream BID for 2 weeks - following a sitz bath - resolution within 2-4 weeks - urethral prolapse = dysuria, frequency, difficulty urinating - prolapsed tissue easily bleeds

12 yo M + syncopal spells past 2 mos that occur with and without exercise. Recent episode happened while he was running during a soccer match. Had rapid return of consciousness after about 15 seconds. Vitals and PE are normal. No murmur during Valsalva. BP and HR during sitting, standing, and supine are normal. Baseline EKG with epsilon waves. What is causing his syncopal episodes?

Arrhythmogenic right ventricular dysplasia - defects in desmosomes - ventricular arrhythmias - fibrous and fatty infiltration of myocardium

W/u for cyclic vomiting syndrome ...

BMP upper GI series to r/o malrotation

A 36-day-old infant is seen in the emergency department after an episode of bloody emesis. The infant was born full term without any complications. She is breastfed and gaining weight appropriately. She was afebrile and in good health prior to this event. Since birth, she has had frequent spit-ups after feeding, but never any blood in her emesis previously. Today, her emesis was noted to be milk colored with streaks of red and what appeared to be small blood clots. On arrival to the emergency department, the infant has a temperature of 37.9°C, heart rate of 146 beats/min, respiratory rate of 38 breaths/min, and blood pressure of 90/68 mm Hg. Physical examination reveals a well-developed infant who is awake and alert. Her abdomen is soft, nontender, and nondistended without hepatomegaly or splenomegaly. The remainder of her examination findings are unremarkable. A bedside gastric occult blood test on aspirated stomach contents confirms the presence of blood. Of the following, the MOST appropriate next step in this infant's evaluation is

Apt-Downey test on aspirated stomach contents - The infant has evidence of upper gastrointestinal (GI) bleeding. She is clinically stable with normal vital signs. Many substances such as food, medications, or dyes can mimic hematemesis. Therefore, the first step in evaluation of red or pink emesis is to determine if blood is actually present with a test that uses the peroxidase activity of hemoglobin to catalyze a color change on a test card. - Once the presence of blood is confirmed positive, the most appropriate next step is to determine if the source of the blood is the infant or her mother. Maternal blood may be swallowed during the birthing process or when breastfeeding if the mother has bleeding nipples. These sources can be distinguished by the Apt-Downey test, which detects fetal hemoglobin. - A positive Apt-Downey test result indicates that the source of blood is the neonate/infant, a negative test indicates maternal origin. - The Apt-Downey test can be used on infants up to age 8 weeks; thereafter the reliability of the test decreases as the proportion of fetal hemoglobin decreases. - If the Apt-Downey test performed on this infant's stomach contents reveals a negative result, no further evaluation or treatment is indicated. If the Apt-Downey test result is positive, further evaluation is warranted.

30 month old presents with 2 day hx of increased temp, decreased activity, poor appetite. During past 6 mos, he has had sever episodes, one requiring hospitalization for tx of dehydration and bacterial pna. CBC reveals ANC of 180 and blood cx positive for Clostridium septicum. What clinical signs or symptoms is most likely to be identified during additional eval?

Aphthous ulcers and stomatitis - Cyclic neutropenia - Associated clinical findings = aphthous stomatitis, oral ulcers, pharyngitis, LAD - increased risk of C septicum

How should be temp be taken depending on age?

At age 3 years or younger, temperature should be taken rectally. At age 4-5 years and thereafter, an oral temperature may be taken.

3 yo F + mom concerns bc she falls frequently when running + wt at 90% + ht at 50%ile + BMI at 97%ile + PE = genu varum with intoeing while walking + x-ray = bilateral varus deformities of proximal tibia with medial beaking and downward slope of proximal tibial metaphysis. Next step?

Brace therapy - Blount dz - most common cause of genu varum in kids - present with excessive falling and in-toeing - brace therapy = initial tx until 4 yo

2 yo M + in ED with wheezing and resp distress. Symptoms improve with nebulized albuterol. Does not require oxygen. CXR reveals intrapulmonary soft-tissue density rounded structure in right lower lobe. Most likely cause of pt's symptoms?

Bronchogenic cyst - congenital malformation of bronchial tree - 5-10% of pediatric mediastinal masses

Causes majority of infectious mononucleosis cases not caused by EBV

CMV - fever, anorexia, malaise, fatigue, pharyngitis, cervical LAD, splenomegaly, hepatomegaly - 1/3 = rash

A 15-year-old, previously healthy adolescent is brought to the emergency department directly from football practice; he complained of left upper quadrant abdominal pain after being tackled. There was no loss of consciousness; he does not have a headache and has not vomited. He has left shoulder pain that is worse with inspiration. Vital signs include a temperature of 37.2°C, heart rate of 100 beats/min, respiratory rate of 20 breaths/min, blood pressure of 106/65 mm Hg, and room air oxygen saturation on pulse oximetry of 100%. He appears to be in pain but is alert and oriented. His examination findings are significant for left upper quadrant tenderness and slight abdominal distention. His skin is warm and well-perfused; he has good range of motion of all extremities. Two large-bore intravenous lines are placed, and a chest radiograph has normal findings. Of the following, the BEST abdominal imaging modality to confirm the diagnosis is

CT - adolescent in the vignette has sustained a splenic injury; despite this potentially severe injury, he is currently hemodynamically stable - computed tomography with intravenous contrast is the imaging modality of choice to confirm specific intra-abdominal injuries in children and adolescents with blunt abdominal trauma who are hemodynamically stable

Most common cause of chronic diarrhea in children 6 mos - 2 years

Cow's milk protein allergy - 2% of kids under 4 years old

Preferred imaging to r/o VP shunt malfunction

Cranial CT

Slow growing mid-line tumor that can cause hydrocephalus and impacts optic nerve tracts, causing bilateral hemianopsia. Headaches and vomiting are common presenting symptoms. Changes in personality or sleeping patterns are also common presenting signs.

Craniopharyngioma

Pubertal delay is more common in which IBD? ...

Crohn's

To estimate prevalence of autisim in large metro area, representative sample was selected and surveyed about their history. What best describes this study?

Cross-sectional study - analyze a population at a single point in time - assessed disease prevalence

A 7-year-old boy is on treatment for nephrotic syndrome for the last 4 years. He develops a relapse within a few days after stopping prednisolone. He has had several such relapses and is diagnosed with steroid-dependent nephrotic syndrome. He has now developed Cushingoid appearance, growth failure, hypertension, and cataract in the left eye. What is the most appropriate treatment for this patient?

Cyclophosphamide - Cyclophosphamide prolongs the duration of remission and reduces the number of relapses in children with steroid-dependent or frequently relapsing nephrotic syndrome. A dose of cyclophosphamide is 2-3 mg/kg/day as a single dose for 8-12 weeks. - Alternate day prednisolone is continued during the course of cyclophosphamide therapy. - Side effects should be carefully observed and discussed with the family before starting treatment. They include neutropenia, alopecia, hemorrhagic cystitis, sterility, increased risk of future malignancy, and disseminated varicella. White cell count should be monitored weekly, and the drug should be withheld if WBC count falls below 5000/mm3.

Typically, menarche can be expected to occur when during puberty?

within the subsequent 2 to 3 years of the development of thelarche - Most gains in linear growth and peak height velocity occur before menarche

Duchenne muscular dystrophy inheritance

x-linked recessive

Evidenced by thrombocytopenia, prolonged PT/INR, PTT ....

DIC - most likely cause = sepsis (ill-appearing + high WBC) --> broad spectrum abx until etiology determined

Cardioprotective free radical scavenger, is routinely administered with anthracycline treatment.

Dexrazoxane

Splitting of spinal cord into 2 portions by midline septum

Diastematomyelia

Arkansas + tick bite + pancytopenia

Ehrlichia chaffeensis

Young girl with bloody diarrhea. Went camping and exposed to tap water / contaminated. Cabin on lake in rural area = location. Dx?

E coli

6 day old girl born at home with assistance of midwife. Presents with persistent vomiting. Mom reports that she is a poor feeder. Takes on average 1 ounce of commercial cow's milk-based formula every 3-4 hours. She is more than 10% below her birth weight. Has jaundice to below the umbilicus and is lethargic. Bilateral cataracts noted. What organism would most likely cause sepsis in this pt?

E coli - galactosemia - AR - at risk for E coli sepsis - galactose levels increase when ingestion lactose - cataracts, growth failure, jaundice = common - contraindication to BF'ing - tx = soy-based/lactose-free formulas

Best medication to administer for organophosphate toxicity

atropine

X-linked lysosomal storage disorder characterized by periodic pain crises, angiokeratomas, corneal and lenticular opacities, stroke, left ventricular hypertrophy, sweating abnormalities, and renal disorder progressing to end-stage renal disease.

Fabry disease - Enzyme replacement therapy with recombinant or gene-activated human α-galactosidase

9 yo F + pain and numbness in hands and feet for last few mos + pain gettin worse and feels like "pins and needles" + chronic abdominal pain and diarrhea + intermittent blurry vision + labs = proteinuria. Most likely cause ...

Fabry dz - acroparesthesias = severe neuropathic limb pain - GI symptoms - corneal opacities - hypohidrosis - angiokeratomas - tinnitus/hearing loss

What would cause an abnormally prolonged aPTT that corrects with a 1:1 mixing study?

Factor 11 deficiency - part of intrinsic pathway --> deficiency would prolong PTT

What is activated protein C resistance associated with ...

Factor 5 Leiden mutation

More helpful than other maneuvers in detecting DDH in children >3 mos....

Galeazzi maneuver - initially presents at 8-10 weeks of age

Adolescent men having sex with men should be screened at least annually for

HIV, syphilis, chlamydia, and gonorrhea

Hepatitis D virus infection results in severe liver disease in presence of what virus?

Hepatitis B - Hepatitis D cannot replicate without coexisting infection with hepatitis B --> liver failure, cirrhosis - dx via anti-HDV Ab

What Abs are secreted in breast milk ...

IgA

12 yo boy presents with 4 week hx of hair loss. Parents deny associated systemic symptoms. Takes no daily meds and has no known allergies. There are 2 discrete smooth, circular alopecic patches on his scalp. Patient's nails are roughened and stippled in appearance. Most reasonable first-line therapy?

Intralesional steroids - alopecia areata

15 yo Asian girl complains of lower abdominal pain and bloating after eating wheat. Pain improves with defecation. She also has intermittent diarrhea and constipation. Her growth and appetite are normal. She denies any muscle wasting, delayed puberty, rashes, or ulcers. CBC and diff and basic chemistry are normal. Most likely cause of pt's symptoms?

Irritable bowel syndrome - functional abdominal disorder more common in adolescents - abdominal change and stool change - absence of extra-GI symptoma

Clenching/grinding of teeth is adverse reaction associated with acute intoxication of ...

MDMA

Delayed puberty in boys is defined as

the failure of testicular enlargement to achieve SMR G2 by age 14 years - A bone age, karyotype, and measurement of gonadotropin levels may be indicated for the evaluation of delayed or abnormal puberty

HT + bruit ove renal artery + axillary freckling + scoliosis =

NF1 - cafe au lait spots

Recognize lisch nodules

NF1 - cafe au lait spots - axillary freckling - multiple types of tumors

What group hate highest rates of SI in adolescents ...

Native American males

Paramedics attend to a 4 yo M who choked on a hot dog. Unconscious and cyanotic. Attempts to clear airway and establish air movement unsuccessful. Next step?

Needle cricothyroidectomy - preferred in children <10 yo

14 yo boy + left-sided scrotal mass that enlarges when he assumes upright position and during Valsalva maneuver. Dilated veins are readily visible on overlying scrotal skin. Testicle size appears normal. Best next step in treatment of patient?

Observation only - Varicocele - Can have dull, aching scrotal or testicular pain - Caused by valves in spermatic vein

A 12-year-old boy at a rural clinic has a history of recurrent hematomas of the thighs. He has remained active in athletics and has suffered four thigh hemorrhages severe enough to cause compartment syndrome in the past 3 years. He has no history of easy bruising or gingival bleeding. There is no history of bleeding into the joint spaces or gastrointestinal or urinary tracts. His older sister has a similar history. Growth and development are normal. Past medical history is otherwise unremarkable, and immunizations are current. On exam, there is palpable induration overlying both quadriceps muscles. No ecchymoses or petechiae are noted. What pattern of coagulation studies is expected with this condition?

Normal platelet count, prolonged PT, normal aPTT, normal thrombin time - Factor VII deficiency is an autosomal recessive coagulation disorder caused by mutation of the gene coding for factor VII on chromosome 13. - Platelet count, aPTT, and thrombin time are normal, while PT is prolonged since factor VII is part of the extrinsic pathway. - Acute bleeding episodes are treated with factor VII replacement or recombinant factor VII. - Prophylactic treatment is indicated for recurrent intracranial hemorrhages and hemarthroses.

Recognize Oculodermal Melanocytosis, in which a complication can be glaucoma

Ota - bluish, gray, irregular, patchy pigmentation on face in ophtho and/or maxillary distribution along trigeminal nerve - potential complictions = hemangioma of optic disc, glaucoma - does not resolve with time

For infants aged 6 months or younger who are exposed to measles, what is recommended?

PEP with intramuscular Ig - Intramuscular Ig must be administered as soon as possible after exposure but may be given within 6 days of exposure - For exposed infants aged 6 to 11 months, MMR vaccine can be given in place of intramuscular Ig and would offer protection if given within 72 hours of exposure - Infants receiving MMR vaccine before their first birthday should be revaccinated at 12 to 15 months of age and receive a third dose at least 28 days later, usually at 4 to 6 years of age

A 3-year-old boy with Landau-Kleffner syndrome has been closely followed for generalized tonic-clonic seizures as well as behavioral problems. Which of the following would be another expected finding in this disorder?

Progressive aphasia in a previously healthy child

Confirmatory test for a patient presenting with narcolepsy:

Polysomnography followed by multiple sleep latency testing (MSLT)

You want to develop a low-cost screening program for a rare but fatal genetic disorder called Condition X that can be diagnosed with a very expensive specific genetic mutation test. To cut costs but screen all the newborns in your state for this condition, you decide to use a blood test measuring protein Y as a screening test for Condition X. For newborns who test positive for protein Y, the genetic mutation test will confirm the diagnosis. You hope this approach will reduce the need for the expensive confirmatory genetic mutation test. What prerequisites are essential for this screening test for protein Y and the confirmatory genetic mutation test?

Screening test - highly sensitive, moderately specific; Confirmatory test - highly sensitive and specific - For a good screening program, the screening test should be highly sensitive so that no actual cases will be missed. It can have moderate specificity so that any false positives can be eliminated by a confirmatory test. - The confirmatory test should be highly sensitive and specific to ensure correct diagnosis no missed cases (high sensitivity) and no wrong diagnoses (high specificity).

initial diagnostic test of choice in a child suspected to have myopathy

Serum creatine kinase - Glucocorticoids have been shown to improve muscle strength and prolong ambulation in children with Duchenne muscular dystrophy

Can occur after initial exposure of foreign antigen without any prior sensitization.

Serum sickness/SSLR - via immunization of human host with heterologous / nonhuman serum proteins, such as anti-thymocyte globulin, monoclonal Abs, antiserum (ex. rabies and tetanus), and sting

Bacterial pathogen that can cause purulent vaginal discharge that is sometimes bloody. May or may not be associated with GI symptoms. Via contaminated food or water.

Shigella

Associated orthopedic abnormalities of Klippel Feil syndrome ...

Sprengel deformity - failure of scapula to descend to normal position Atlantoaxial instability Hemivertebrae Scoliosis

Type 1 myotonic dystrophy (DM1) is also referred to as

Steinert disease

port wine stain associated with what syndromes ...

Sturge-Weber syndrome Klippel-Trenaunay syndrome Cobb syndrome Proteus syndrome

Type of observational study used to assess associations between exposures (risk) and outcomes...

case-control study - most commonly retrospective and involves using 2 groups: those with the disease/outcome (cases) and those who do not have the disease/outcome (controls) - determine an odds ratio and can be used to evaluate rare outcomes or diseases - generally inexpensive and not very time consuming - measures associations but not causality

Recognize erythroderma, associated with toxic shock syndrome

TSS - fever, hypotension (resistance to fluids), AMS, erythroderma, mucous membrane inflammation

Pts with CGD increasingly susceptible to ...

catalase-positive bacteria and fungi (staph aureus, aspergillus, burkholderia, nocardia)

Meningoceles and meningomyeloceles occur in which portion of the spine?

They can occur at any portion of the spine - The most common area for these defects to exhibit is the caudal end of the neural tube and the vertebral arches. - Failure of the two halves of the vertebral arch is usually in the sacral, lumbar, and cervical regions.

7 day old male being treated for presumed sepsis in NICU. Has episode of gross hematuria. Was born at 32 weeks of gestation to a mother with insulin-dependent diabetes. Apgars 2, 5 following emergent c-section for fetal distress. On PE, palpable mass identified in left flank. On renal US, an enlarged and echogenic left kidney, associated with thrombus extending outside the kidney to the inferior vena cava, is noted. What lab finding is most likely to be identified during additional eval of this pt?

Thrombocytopenia - Renal vein thrombosis --> classic triad = gross hematuria, flank mass (unilateral or bilateral enlargement of kidneys), thrombocytopenia Labs - hematuria - proteinuria - polycythemia - hemolytic anemia - thrombocytopenia - possible acute kidney injury

generally sufficient in most cases of pathologic phimosis

Topical corticosteroids and gentle retraction

Recognize infantile eczema

Tx - Daily baths with fragrance free cleanser; bland moisturizer (like Vaseline ointment) at least bid - Can start with OTC hydrocortisone 1% ointment bid (ointment not cream) (med to rash first then Vaseline "all over") - If not significantly improved after 1-2 weeks, see your pedi for something stronger

In pts with autoimmune polyglandular syndrome Type 1 (APS1), pts first present with ...

chronic mucocutaneous candidiasis - then get hypoparathyroidism and primary adrenal insufficiency - male = female - no HLA association

Characterized by imperforate anus, thumb anomalies, renal anomalies, and cardiac anomalies, in addition to dysplastic ears and hearing loss ...

Townes-Brocks syndrome

16 yo M + playing baseball but experiences syncope after being hit in the chest with a baseball + quickly resuscitated with an AED + has rapid return of normal mental status + EKG = PACs + Echo and CXR both normal + labs with troponin of 0.15. Most likely cause of elevated troponin?

Transient ischemia related to ventricular fibrillation - Commotio cordis - resuscitated --> mild elevation in troponin via transient ischemia d/t fibrillation

17 yo girl complains of thinner of her hair. Diffuse hair loss and thinning are evident. No appreciable inflammation, scale, or scarring. No loss of other body hair. Microscopic exam of pulled hairs reveals majority lack gelatinous hair sheath, and they have uniform shaft diameter and small, white, club-shaped bulb. Pt might be experiencing side effect from which med?

Tx with propranolol for migraine prophylaxis - Telogen effluvium = sudden onset of noninflammatory diffuse hair loss without associated scarring - Med triggers = beta blockers, amphetamines, ACEI's, OCP's, retinoids, lithium

Fracture through physis and adjacent metaphysis describes

Type 2 Salter Harris Fracture

AR disorder. Accumulation of glycogen in muscle, liver, heart, kidney, smooth muscle, nerves. Only condition with marked hypotonia and cardiomegaly in infant who was normal at birth ...

Type 2 glycogen storage disorder - normal blood glucose - EKG = shortened PR intervals, high voltage QRS - enzyme-replacement therapy

12 yo girl + nasal discharge for 12 days with no improvement. Complains of facial tenderness. No fever or vomiting. No drug allergies. What is true concerning this patient?

Treatment = 10-21 days - acute bacterial sinusitis - tx = amoxicillin or augmentin

6 week old girl taken to ED custody by law enforcement personnel after pt's aunt noticed she would not move her left arm. Mom received no prenatal care and has 2 older children. Growth parameters are below 3rd %ile. She refuses to move her left arm and cries during ROM testing. X-ray of left arm reveals evidence of osteochondritis at metaphysis and periostitis of radius and ulna. Most likely cause of pt's findings?

Treponema pallidum - congenital syphilis - osteochondritis and periostitis (usually metaphysis and diaphysis of long bones) - wimberger sign = destruction of medial aspect of proximal tibial metaphysis - pseudoparalysis of Parrot

Suggestive of impending brain herniation:

Triad of bradycardia, hypertension, and altered respirations (Cushing triad)

First-line treatment for a pathologic phimosis

Triamcinolone

Fracture through physis and adjacent epiphysis describes

Type 3 Salter Harris Fracture

4 yo F + diffuse urticarial rash, tongue swelling, difficulty breathing. Stung by a bee at a family picnic 30 minutes ago. What type of HS reaction?

Type I - anaphylactic reaction

Broad-spectrum antibiotic coverage should be administered to treat infected dog bites. Antibiotics should cover the oral flora of the animal (e.g., Pasteurella, Staphylococcus aureus, streptococci, and oral anaerobes). In a patient with a severe penicillin allergy, what is recommended?

clindamycin plus ciprofloxacin

17 yo female + eval of abdominal pain. Pain is chronic, colicky pain in RLQ. No wt gain for a year and has delayed puberty. Thin, pale adolescent. No secondary sexual dev't. Abdomen is slightly distended. Good bowel sounds but mild tenderness in RLQ and palpable loop of bowel. Stool from rectal tests is heme-positive Stool has red and white blood cells. Stool cultures, ova and parasites, and c diff toxin are all negative. What studies is most likely to confirm your diagnosis?

Upper and lower endoscopy with mucosal biopsy - Crohn disease

4 yo + diffuse weakness, cerebellar ataxia, partial ophthalmoplegia. Most likely vitamin deficiency causing symptoms?

Vitamin E - spinocerebellar degeneration - antioxidant = prevents neurodegeneration and hemolytic anemia

Clinical observation and surveillance for common comorbidities is appropriate management for most children with tics. If there is functional impairment, what is the management?

comprehensive behavioral intervention treatment - α-agonists, antipsychotics, or topiramate can be used for symptomatic relief

A newborn term female infant born 24 hours ago was found to have hydronephrosis of the right kidney on fetal ultrasound during the 3rd trimester. This was recorded as an 8mm dilation of the right renal pelvis in the anteroposterior diameter. Though the baby was born without incidents and seems to be doing well and has a normal newborn exam. You note that the mother is 16 years old. She did not seek prenatal care until the 3rd trimester and was homeless early during her pregnancy. The father is not involved and the mother does not offer any information about him except that he's not around. The renal ultrasonogram (USG) comes back normal. Now the new mother and her mom are pressing for discharge. Her mother has been present during her stay and says she is a nurse and will help care for the baby. Which of the following evaluations should be performed on this patient?

Voiding cystourethrography (VCUG) prior to discharge - Hydronephrosis is found in about 4.5% of fetal ultrasound studies and is defined as greater than 4mm renal pelvic dilation in the anteroposterior diameter in the second trimester and as greater than 7mm in the third trimester - Infants with these criteria should undergo postnatal renal USG, however normal renal USG findings do not rule out vesicoureteral reflux - 10% to 30% of newborns with antenatal hydronephrosis have vesicoureteral reflux (VUR) and are at risk of renal scarring from urinary tract infection - Depending on the assessment of patient compliance with follow-up, the renal USG and VCUG may be performed during the newborn stay or later as an outpatient. In this particular case, the mother's young age and history of late prenatal care would warrant a study prior to discharge. If there were no concerns about patient compliance with follow-up, VCUG could be scheduled later as an outpatient.

1 week old M infant in NICu for poor feeding + aortic stenosis on echo + majority of feeds via NG + persistent hypercalcemia, despite adequate hydration and d/c'ing TPN. Dx?

Williams syndrome - hypercalcemia in neonates

Pts with what syndrome have increased prevalene of celiac disease?

Williams syndrome Turner syndrome Down syndrome

4 yo + enlarging abdominal mass and abdominal pain + birth was normal + vitals normal + PE = palpable mass in left abdomen + MR = left-sided cystic mass from kidney. Dx?

Wilms tumor - most common renal malignancy in kids <15 yo

Teen with elevated liver enzymes, fatigue, and family history of cirrhosis. Esophagram shows esophageal varices. All of these findings are consistent with

Wilson disease

Incontentia pigmenti iheritance

X-linked dominant

How is Lesch-Nehan inheriated?

X-linked recessive

The clinical presentation of the child in the vignette is consistent with Rett syndrome, a genetic neurodevelopmental disorder occurring almost exclusively in girls. Following a period of normal development, children with Rett syndrome experience a developmental regression with loss of speech, stereotyped hand motions with lack of purposeful movements, an autistic phenotype, and gait abnormalities. The developmental regression is preceded by

a plateau of head growth resulting in acquired microcephaly - The associated features may include seizures, respiratory and cardiac abnormalities, autonomic dysfunction, and sleep disturbances.

A 2-hour-old male neonate born at 38 weeks' gestation is being evaluated. His mother is a 22-year-old, gravida 1, para 0 woman with a history of mild phenylketonuria and herpes infection treated with valacyclovir. No active genital lesions were noted at the time of delivery. He was born vaginally with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. His weight is 2.2 kg (2nd percentile), length 46 cm (5th percentile), and head circumference 33 cm (10th percentile). Physical examination reveals a well-appearing neonate with 2/6 systolic murmur. Of the following, the findings seen in this neonate are MOST likely caused by

abnormal maternal phenylalanine level - Neonates born to mothers with PKU are exposed to elevated phenylalanine levels and may develop intrauterine growth restriction, microcephaly, structural cardiac anomalies, and developmental delays. The risk of developing these complications is proportional to maternal serum phenylalanine levels. - Phenylketonuria is caused by a defect in phenylalanine hydroxylase resulting in excess serum phenylalanine. It is the most common inherited disease of metabolism related to amino acid metabolism, with an incidence of 0.8 in 100,000 live births. - Elevated phenylalanine levels impair myelination and can result in profound neurologic impairment. - Management includes dietary restriction of natural proteins supplemented with phenylalanine-free amino acid mixtures. - More recent data suggest that restriction should continue through adulthood to optimize outcomes. - Strict dietary adherence during pregnancy improves neonatal outcomes

Antibodies against hepatitis B core antigen can be found in individuals with

acute, chronic, or resolved hepatitis B virus infection

A 3-year-old unvaccinated girl is brought to the emergency department 16 hours after she was bitten on the right lower arm by an appropriately vaccinated child at her daycare center. The family denies fever, current bleeding, or active discharge from the wound. On physical examination, her temperature is 37°C. There is a 2-cm bite wound on her right lower arm with mild penetration of the epidermal layer, minimal erythema and edema of the immediate surrounding tissue, no induration or fluctuance, no active bleeding or discharge, and mild tenderness to palpation. Of the following, the best NEXT management step for this girl is to

administer DTaP - Because she is unvaccinated, she should concurrently receive human tetanus immune globulin (TIG) within 21 days of the injury. If human TIG is not available, intravenous immune globulin may be used. Human TIG should be administered at a different site from the tetanus toxoid. - For individuals who have been vaccinated against tetanus previously, but are not up to date, there is little benefit in administering human TIG more than 1 week after the injury unless they have HIV infection or severe immunodeficiency with contaminated wounds.

2 day old infant boy born at home. No meds given. Blood in stool + petechiae + blood from umbilical stump. Tx?

administer vitamin K IV - a little early but possible - cofactor for II, VII, IX, X = hemorrhagic dx of newborn

Next step you can go to for asthma if albuterol + flovent 220 mcg 2 puffs BID is not working....

advair

Acrodermatitis enteropathica usually becomes apparent when?

after into of cow's milk-based formula after weaning infant from breast milk - AR disorder - ZIP4 gene on chr 8 - tx = oral zinc gluconate or sulfate 1-3 mg/kg/day --> rapid improvement of symptoms (within 10 days)

Children with congenital growth hormone deficiency are typically of normal size at birth. Linear growth deceleration occurs after what age?

after the age of 6 to 12 months - when growth hormone becomes important for linear growth - pts with acquired growth hormone deficiency should undergo investigation for a brain neoplasm and other pituitary hormone deficiencies

The rotavirus vaccine may not be administered after

age 8 months and 0 days

Feed amount for infants based on mos

age in mos + 2 oz = feed amount newborn ~ 2-3 oz q3-4 hours 1 mo ~ 3-4 oz q4h 2 mo ~ 4 oz q 6-7 hours/24 h 4 mo ~ 4-6 oz = 5 feedings/24h

Nasal fractures should be reduced prior to commencement of fracture healing, which occurs when?

approximately 5 to 6 days after the trauma

A 9-year-old boy presents with persistent bilateral middle ear effusion that began approximately 7 months ago following an episode of bilateral acute otitis media. He has already been treated with courses of amoxicillin and cephalexin. Past medical history is significant for rhinorrhea, accompanied by nasal congestion with itching, sneezing, and watery, itchy eyes; he experiences symptoms all year, with worsening symptoms during the month of April. Intranasal steroids have reduced these symptoms in the past. Family history is remarkable for his father's asthma. Growth and development have been normal and immunizations are current. Vital signs are normal. Height and weight are at the 50th and 25th percentiles, respectively. Examination reveals pale-blue, boggy turbinates with clear rhinorrhea, cobblestoning of the posterior pharynx with postnasal discharge, and puffy, dark lower eyelids. Both tympanic membranes are retracted and immobile. What is the most likely underlying disorder?

allergic rhinitis

Maple syrup urine disease is a disorder of...

amino acid metabolism - requires special formula

Best option for preventing migraines ...

amitriptyline - tricyclic antidepressant

Although outgrowing peanut allergy is unlikely, what is recommended?

annual skin testing until 6 yo

Baseline ophthalmological surveillance for children with Marfan syndrome includes

annual slitlamp examination - for assessment of refraction and monitoring for evidence of lens subluxation, glaucoma, or cataract - cardiovascular surveillance includes annual echocardiography with aortic root (Ao) measurements

sponylolisthesis

anterior displacement of vertebral body in face of interarticular fracture - most common cause of pain with back extension - common in adolescents - can usually see on plain radiography --> if can't see, can consider MRI - activities with repetitive extension or twisting + axial load = baseball, gymnastics, football

Characteristic x-ray findings of Scheuermann kyphosis ...

anterior wedging of >/= 5 degrees in 3 or more consecutive vertebral bodies apex for deformity in thoracic or thoracolumbar region

What lab test is like to determine neonatal lupus?

anti-Ro and/or anti-La in MOTHER

Girl has evidence of preceding group A streptococcal infection as well as carditis and polyarthritis. These findings meet the Jones criteria for acute rheumatic fever (ARF). The management of ARF includes

anti-inflammatory medication (aspirin), eradication of the streptococcal infection (penicillin), and treatment of carditis or heart failure

acute rheumatic fever results from untreated group A strep throat infection, causing ...

antigenic mimicry = generation of Abs that cross-react between bacterial M protein and human tissues

Boy with HIV + high viral load + low CD4 count + acute diarrhea illness + cryptosporidium after water exposure. Next tx?

antiretroviral therapy

Used for assessment of hearing loss after meningitis ...

auditory brainstem response - damaged usually to 8th CN

Microtia in infant. Isolated malformation. Best NEXT step...

auditory brainstem response test - middle ear can be affected as well - RUS will EVENTUALLY be needed

Chronic benign neutropenia is the most common cause of neutropenia in "healthy" children. It is caused by...

autoAbs to granulocytes - ANC usually 0-500 - median age = 8-11 mos - typically lasts ~ 2 years

What kind of condition is transverse myelitis

autoimmune inflammatory condition

Treatment may be recommended for GERD depending on the severity of symptoms, which can include:

avoiding overfeeding thickening feedings trial of elimination of cow milk protein trial of acid suppression therapy - Gastroesophageal reflux in infants is common, peaking at 3 to 4 months of age; 95% of infants experience resolution of symptoms by age 12 months. - Concerning "alarm" signs and/or symptoms in a child who appears to have gastroesophageal reflux include weight loss, late onset of symptoms (in children older than 6 months of age), or symptoms without improvement by 12 months of age.

Mechanism of pityriasis versicolor ...

azelaic acid-mediated melanocyte inhibition

Cat scratch/bartonella tx

azithromycin

Pts with diarrhea caused by Shigella initially present with nonspecific symptoms such as fever and malaise before developing abdominal pain, vomiting and diarrhea (bloody, mucoid). What is first-line tx?

azithromycin

21 yo military recruit + 2 wks of subjective fevers, fatigue, non-productive cough. Vitals nml. PE = bilateral wheezing. CXR = diffuse interstitial infiltrates. Sputum gram stain negative. Culture on Eaton agar grows "fried-eggs" shaped colonies. Most appropriate tx for this condition?

azithromycin - Atypical walking pna

5 yo boy presents for f/u bc 3 days of topical ophthalmic drops have not improved what was thought to be bacterial conjunctivitis. Mom reports her son has been less active over the past week + complaints of HA, fatigue, decreased appetite. Tempt 101.3. Right palpebral conjunctivae markedly injected without purulent discharge; a 3-mm, granulomatous-like lesion noted. Moderately tender 1x-2 cm preauricular lymph node noted on ipsilateral side. Pt would be best treated with ...

azithromycin - Parinaud oculoglandular syndrome = atypical presentation of catscratch = unilateral nonpurulent conjunctivitis - tx options = rifampin, cipro, TMP-SMX, azithromycin - there can also be resolution without tx

Latex-fruit syndrome = ppt allergic to latex have hypersensitivity to what foods?

banana kiwi avocado chesnut papaya white potato tomato

Rectal biopsy is gold standard for Hirschprungs, but what can demonstrate dilated proximal colon with narrowed distal bowel?

barium enema

At what age is puberty normal in girls?

beginning puberty for girls ages 8-13 is normal

A 4-month-old infant is seen for a health supervision visit. She does not track voices or respond to loud noises. She failed both her newborn hearing screen and a follow-up audiology evaluation in both ears. An otolaryngologist and audiologist confirmed bilateral sensorineural hearing loss. Her father asks about devices that could help improve her hearing. Of the following, the MOST appropriate device to consider for this infant is/are

behind the ear hearing aids - may be provided for infants as young as age 2 months and are the first-line intervention for infants with hearing loss - Cochlear implants should be considered in children aged 1 to 2 years with bilateral profound SNHL and older children with severe to profound SNHL if hearing aids or other amplification devices have not been effective.

Girl with osteomyelitis. Radiograph normal. Next step?

blood cx - can be + at 12-14 hours

Pts with NF1 present with ...

cafe-au-lait spots (flat hyperpigmented lesions) axillary freckling multiple types of tumors (peripheral nerve tumors and optic gliomas)

C. acnes release...

chemotactic factors that attract neutrophils, which release lysosomal enzymes; these enzymes, along with free fatty acids, produced during bacterial growth, cause inflammatory changes

90% of HSP cases occur when in life?

childhood

Adolescent women having sex with men should be screened at least annually for

chlamydia and gonorrhea

2-hr old F infant in nursery evaluated for enlarged clitoris and posterior fusion of labia majora. Most appropriate next step?

chromosomal analysis and 17-OHP level - virilized female - CAH via 21-OH defiency

Cutaneous diphtheria manifests as a

chronic, nonhealing ulcer

Associated with increased risk of functional ovarian cysts

cigarette smoking

Can be used as prophylaxis for meningococcal disease in children >/= 1 month old...

ciprofloxacin - single dose

Findings on penis common with reactive arthritis ...

circinate / gyrate balanitis (inflamed hyperkeratotic, whitish plaques on glans penis)

Ages of copying various shapes...

circle (3 yo) cross (3-4 yo) square (4 yo) triangle (5 yo) diamond (6 yo)

Consumption of what food is an indication a child (ex 12 month old) has pellagra

corn mash - in contrast to other grains, corn does not contain enough nicotinic acid *niacin* = dementia, diarrhea, dermatitis

Subtracts expected benefits of intervention or project from total cost of implementation, measured in monetary units ...

cost-benefit analysis

Neonatal Infant Pain Score (NIPS) looks at ...

facial expressions crying leg and arm motions agitation

Tx for refractory von willebrand dz ...

factor VIII with von Willebrand factor complex (Humate P)

Research study designed to measure performance on visuospatial, memory, and logic tests. Researcher plans to combine and analyze three variables as single metric of cognition. Setup best describes ...

factor analysis - allows researchers to investigate concepts not easily measured directly (cognition, SE status) - uses interpretable underlying factors as surrogates for unmeasurible variables

An 11-month-old infant presents with a rash covering her trunk, arms, and neck. She has had a febrile illness over the past 4 days, with a slight cough and coryza as her only other symptoms. The parents state that the fever has been up to 104° F but is improving. The rash developed as the fever defervesced. She had been well prior to this illness. Medications include acetaminophen; she has no known allergies. On examination, she is afebrile, acts happy and playful in her mother's arms, and manifests a blanching, maculopapular rash over her trunk, abdomen, arms, and a slight rash on her face and legs. What is the most likely complication from this condition?

febrile seizure - Roseola infantum (exanthem subitum) is a common illness in younger children, usually affecting those between the ages of 4 months to 3 years; 90% of cases occur in the 1st 2 years of life - characterized by a fever lasting 3 to 7 days, followed by rapid defervescence and the development of a blanching, maculopapular rash that lasts 1 - 2 days - children often appear well in relation to the high fevers that can occur - most common complication of roseola involves febrile seizures - many viruses can give a roseola-like illness, but the major cause of roseola is human herpesvirus 6 - other physical findings include a mild cough and coryza, suboccipital, posterior cervical, and postauricular lymphadenopathy, and eyelid edema

Recommended to diagnose protein-losing enteropathy

fecal α1-antitrypsin level - Elevated fecal α1-antitrypsin levels should prompt further testing for a cause of protein-losing enteropathy, based on the clinical presentation

About 1 hour ago, an 8-year-old boy was playing soccer and running when he stepped into a divot in the grass and rolled his right foot in. He fell and experienced severe pain in his right lateral ankle. He was carried to the sidelines, ice was applied, and he was brought to your emergency department. He has not put any weight on it. On examination, he appears very apprehensive and in moderate distress. His vital signs are stable. His right lateral ankle is mildly swollen without any bruising. He does not allow adequate palpation but indicates diffuse severe pain over the entire right lateral ankle area. What would be the most likely injury to expect in this patient?

fibular physis fracture - fracture of the fibular physis or growth plate is the most likely injury in the skeletally immature athlete who has inverted the ankle, has open growth plates, and experiences pain on palpation of the lateral ankle - incomplete rehabilitation is the most common reason for ankle injury - careful palpation of the lateral ankle will reveal pain over a specific area superior to the lateral malleolus, which corresponds to the level of the distal fibular physis - plain radiographs may demonstrate physeal widening - closure generally occurs around age 13 in girls and 15 in boys; this may vary depending on sexual development. Orthopedic referral is needed

During intraosseous cannulation, what confirms intraosseous needle is correctly?

firm standing of the needle on its own within the bone confirms - indicates that the needle is well-seated within—and is surrounded by—the bony cortex, and it is advanced far enough to be fully supported. After insertion of the needle, correct placement can be confirmed by: - Needle standing firmly on its own within the bone - Obtaining bone marrow upon aspiration from the needle - Flushing of the needle occurs without extravasation

Human bite wounds, puncture wounds, hand wounds, infected wounds, or wounds over 12 hours old should not be closed and should be allowed to

heal by secondary intention.

A 3-month-old girl is evaluated in the emergency department for vomiting several times over the past 24 hours. She has not had any diarrhea, fever, or congestion. She has appeared sleepier than usual and is not feeding well. Her 3 older siblings and mother are all healthy, as is her mother's boyfriend who lives in the same home. The girl was born at 33 weeks' gestation. She had an uneventful neonatal intensive care unit stay, initially requiring assistance with feedings and supplemental oxygen but was taking all feedings by bottle and no longer requiring supplemental oxygen by the time of discharge at 3 weeks of age. On physical examination, the infant is sleepy and difficult to arouse. Her pupils are equal, round, and reactive to light, oral mucosa is moist, heart rate is regular with no murmur, lung sounds are clear with no increased work of breathing, and her abdomen is soft and nontender with no palpable masses. Of the following, the MOST appropriate test to order is

head CT - Infant with vomiting and altered mental status, has signs and symptoms that cause concern for abusive head trauma (AHT). In this situation, head computed tomography (CT) is the best imaging modality. - This infant's risk factors for AHT include age, prematurity, and having a nonrelated adult living in the home. - Abusive head trauma should be considered in infants with inappropriate sleepiness or fussiness; emesis, especially without diarrhea; apnea; or seizures. Infants suspected of having AHT should have an urgent head CT performed. - If a CT scan demonstrates intracranial hemorrhage, urgent neurosurgery and ophthalmology consultation are required. - Physical examination findings concerning for physical abuse include bruises, especially behind the ears, or on the abdomen, neck, or on multiple planes of the body; any bruising in infants not yet mobile, such as those under 4 months of age; and oral injuries, such as a torn frenulum. - A skeletal survey should be obtained for children less than 2 years of age found to have oral injuries, concerning bruises, or a fracture that is not reasonably explained. If there is continued uncertainty, repeat imaging should be obtained after 2 weeks, as healing fractures may become apparent at that time. Laboratory evaluation should focus on causes of increased bruising or fractures. - A child with a fracture that is not consistent with the history provided should undergo laboratory evaluation including calcium, magnesium, phosphorus, alkaline phosphatase, complete blood count, complete metabolic panel, amylase, lipase, international normalized ratio (INR), protime and prothrombin time. If there is evidence of decreased bone density or an elevated alkaline phosphatase level, 25-hydroxyvitamin D and parathyroid hormone levels should be obtained. If there is an elevation in the aspartate aminotransferase (AST), alanine aminotransferase (ALT), amylase or lipase levels, abdominal CT should be obtained to evaluate for intra-abdominal injuries.

First-line treatment of anaphylaxis is

immediate administration of 0.01 mg/kg of 1:1,000 concentration of intramuscular epinephrine - Adjuvant therapies include corticosteroids, H1-receptor antagonists such as diphenhydramine, H2-receptor antagonists such as famotidine, inhaled β-agonists such as albuterol, and administration of intravenous isotonic crystalloid fluids - It is important for children with a history of anaphylaxis to be prescribed an epinephrine auto-injector and their families educated on its use. The correct dose of auto-injector epinephrine is 0.15 mg for children less than 30 kg and 0.3 mg for children greater than or equal to 30 kg.

Tx of guillain-barre syndrome

immune globulin / IVIG = first-line

A chronic wet cough associated with recurrent otitis and/or sinusitis suggests

immunodeficiency or ciliary dyskinesia

Clinical presentation, time of year, and age of the child strongly suggest bronchiolitis caused by respiratory syncytial virus. This is usually diagnosed clinically, but what can be used for definitive testing and can lead to rapid viral identification?

immunofluorescence of nasal secretion

Genetic cause of spinal muscular atrophy ...

impaired splicing of precursor mRNA

most cases of labial hypertrophy can improve with ...

improved personal hygiene

When do night terrors occur?

in non-REM sleep during first 1/3 of nocturnal sleep - 3-12 yo - more common with sleep deprivation

Clinical features of SVC syndrome relate to the lack of adequate venous return to the right side of the heart with upstream stasis of blood flow. Because the SVC receives blood from both the left and right brachiocephalic veins, patients will exhibit edema of the head and neck, distended neck veins, facial plethora, and cyanosis of the face. Patients may also develop cough, shortness of breath, and orthopnea, all of which suggest upper airway obstruction. Symptoms are generally worse when patients are placed in the supine position; what position could be life-saving?

in patients with significant vascular or airway compromise, prone positioning may help relieve some of the compression of the SVC and can be life saving - Supportive care, including elevation of the head of the bed, supplemental oxygen, and judicious use of diuretics, may be required

Hemodynamically unstable bradycardia in setting of prior viral illness may be d/t myocarditis. Initial approach toward restoring stability is to

increase HR via inotropic support or pacing

Majority of children and adolescents with isolated proteinuria have orthostatic proteinuria, characterized by...

increase in protein excretion when upright but normal protein excretion when supine

achalasia is characterized by what esophageal manometry findings ...

increased resting pressure of lower esophageal sphincter = gold standard for diagnosis

A 30-month-old girl is evaluated for loose stools. She started toilet training at 24 months of age but has not yet achieved daytime continence, which has frustrated her parents. For the past month, she has had 1 to 2 large, hard stools per week. While stooling, she hides in the closet and refuses to stool on her potty. Over the past week, she has had daily large-volume, nonbloody, loose stools. The girl eats a regular diet appropriate for her age and drinks 40 ounces of 2% cow milk per day. She has been growing and developing well. She appears well and in no distress. Her abdomen is distended but nontender. There is a large amount of stool palpable in the left lower quadrant. Rectal examination reveals a normal-appearing anus with brown soiling present, normal anal wink, and a dilated rectal vault with palpable, hard stool. Of the following, the MOST effective recommendation to manage the patient's condition is to

initiate a laxative

3 weeks after varicella infection, 4 yo AA boy presents with normal PE except for petechiae and bruising. His labs show: hgb 12.1, blood type A+, WBC 6300, ABC 1700, platelets 3000. What would be most appropriate to do next?

initiate close observation - ITP --> observation for kiddos with no bleeding or mild bleeding, regardless of platelet count

For kids with retropharyngeal abscess without respiratory compromise, it is reasonable to tx with ...

initiate empiric, broad-spectrum, intravenous antibiotic therapy, such as clindamycin, a second-generation cephalosporin, or a β-lactamase penicillin, for 24 to 48 hours before determining whether surgical intervention is necessary

gymnast's wrist refers to ...

injury to distal radial physis - gymnasts 12-14 yo - up to 35hrs/week

Complete disappearance of murmur when standing is almost always a sign of ...

innocent murmur

Diagnostic tool for biliary atresia...

intraoperative cholangiogram - new onset jaundice with elevated conjugated bili and elevated alk phos and GGT = extrahepatic ductal obstruction --> nonpigmented stool - biliary atresia = jaundice, pale stools, dark urine usually noticed 2-4 weeks after birth

A 30-month-old girl is evaluated for loose stools. She started toilet training at 24 months of age but has not yet achieved daytime continence, which has frustrated her parents. For the past month, she has had 1 to 2 large, hard stools per week. While stooling, she hides in the closet and refuses to stool on her potty. Over the past week, she has had daily large-volume, nonbloody, loose stools. The girl eats a regular diet appropriate for her age and drinks 40 ounces of 2% cow milk per day. She has been growing and developing well. She appears well and in no distress. Her abdomen is distended but nontender. There is a large amount of stool palpable in the left lower quadrant. Rectal examination reveals a normal-appearing anus with brown soiling present, normal anal wink, and a dilated rectal vault with palpable, hard stool. Of the following, the MOST effective recommendation to manage the patient's condition is to

initiative laxative Functional constipation/encopresis - due to toilet training refusal and stool withholding - The clinical picture is of a child who avoids passing large-volume stools due to dyschezia (excessive straining), leading to hard bowel movements, increased discomfort, and likely acquired megacolon - She has overflow incontinence associated with loose stools because she does not sense a full rectum - Polyethylene glycol, an osmotic laxative, at a dose of 1.0 to 1.5 g/kg/day for up to 3 days is advised - After cleanout, maintenance therapy includes daily laxative administration and behavior modification. Polyethylene glycol is recommended as first-line maintenance therapy starting at a dose of 0.4 g/kg/day, titrated to achieve daily soft stools for a minimum of 2 months - For children who are toilet training, medical therapy should not be discontinued until successfully toilet trained. Behavior modification may include scheduled toilet sitting for 5 to 10 minutes after meals, appropriate toilet positioning with feet securely planted on a surface, and maintaining a diary of episodes of incontinence and bowel movements to track progress

Early presentation of untreated celiac disease ...

iron deficiency anemia

All females with heavy menstrual bleeding (HMB) whose hemoglobin is not in normal range require...

iron supplementation - mildly low hemoglobin (ex 10) = 60 mg/day of elemental iron - for mod-to-severe bleeding = combined estrogen-progestin OCP in addition to iron AUB = occurs in cycles <20 days or >45 days apart = >7 days - results in large amount of blood loss per episode = >80 ml or >10 saturated pads or tampons per day and/or causes anemia - most common cause of AUB = immature hypothalamic-pituitary-ovarian axis

Hydrocephalus presents with signs and symptoms of increased intracranial pressure including

irritability lethargy vomiting headache papilledema sun-setting eyes cranial nerve 6 palsies firm and bulging fontanelle splayed sutures

Pts with infantile scurvy present with ...

irritability, bone tenderness, bone swelling, pseudoparalysis of legs

Most common side effect of isotretinoin ...

irritated, dry skin and dry lips (cheilitis)

Which seizure: abnormal EEG with 4-6 Hz generalized polyspike and slow wave discharge ...

juvenile myotonic epilepsy - tx = myoclonic epilepsy

Next best diagnostic test teenager with suspected androgen insensitivity ...

karyotype

2 day old male + bilateral undescended testes + normal-appearing phallus but no palpable testes. Most appropriate next step?

karyotype and 17-hydroxyprogesterone - all phenotypic male newborns with bilateral nonpalpable testes must be evaluated for sexual differentiation

In status epilepticus refractory to benzos, what is the appropriate med to administer next ...

levetiracetem

A 15-year-old adolescent is brought to the emergency department for a new-onset generalized tonic-clonic seizure that occurred while asleep. She was having a sleepover party and had stayed up until 3 am. Around 7 am, her friend found her unresponsive with generalized shaking, her eyes rolled up and open, and foaming at the mouth. The event lasted approximately 2 minutes before self-resolving. On arrival at the emergency department, the girl is drowsy but arousable. She has otherwise normal neurologic findings. Her mother reports that the adolescent has had several episodes of staring spells over the past few years which she attributed to her daughter ignoring her. Over the past year, she has periodically complained of "spasms" where her hand will suddenly jump or jerk. She denies substance use, recent or intercurrent illnesses, or trauma. There is no family history of seizures. Of the following, the MOST likely diagnosis is

juvenile myoclonic epilepsy - common genetic generalized epilepsy usually presenting in early adolescence - affected children may have a combination of myoclonic jerks, generalized tonic-clonic seizures, and/or absence seizures - seizures typically occur in the early morning on waking - often myoclonic seizures are mistaken for clumsiness; if there is a suspicion of myoclonic seizures, the history should include questions about early morning clumsiness, jitteriness, or dropping objects - most common triggers for seizures in JME are sleep deprivation, alcohol consumption, stress, and flashing lights - Interictal generalized polyspike and wave discharges at 4- to 6-Hz frequency are seen on EEG. Photic stimulation can provoke typical EEG findings or electrographic seizure in some children. - Valproic acid is effective in the treatment of JME; however, because of concerns for teratogenicity, valproic acid is not first-line treatment for adolescent girls. Other treatment options include zonisamide, topiramate, and levetiracetam. Lamotrigine can be used, but is known to exacerbate myoclonus in some children. - Juvenile myoclonic epilepsy is a lifelong form of epilepsy, requiring maintenance antiepileptic medication.

Girl with fusion of 2 cervical vertebrae (C2-C3). Short neck. Low posterior hairline. Dx?

klippel-feil syndrome - triad = short neck, fused vertebrae, low posterior hairline

Marked linear increases in density of metaphyses indicate ...

lead intoxication - prominent basophilic stippling on peripheral smear

Basophilic stippling of RBCs. Typical blue granules represent ribosomal precipitates and are associated with

lead or other heavy metal poisoning in children

Describe varicella in previously vaccinated children...

lesions are predominately maculopapular, rather than vesicular, and are located anywhere on the body - breakthrough varicella - >42 days postvaccination - usually < 50 lesions - less contagious than wild type infection

How long does simple febrile seizures last ...

less than 15 min

Late-onset listeria neonatal infection presents as ...

meningitis - after first week of life

Vocal cord dysfunction is more likely in people with what conditions?

mental health disorders - anxiety, OCD obesity females medical personnel

fomepizole is tx for what toxicity?

methanol and ethylene glycol toxicity - not ethanol (like mouthwash)

Mechanism of PCP ...

noncompetitively antagonizes NMDA receptors in the brain - peace pill - angel dust - crystal - purple rain

What side effect is more likely with progestin-only OCPs, rather than estrogen?

oily skin associated with acne - varying degrees of androgenic activity - hirsutism too

Wormian bones and osteopenia suggest ...

osteogenesis imperfecta

What primitive reflex would appear between 8 and 9 mos old ...

parachute reflex

EKG findings of accidental amitriptyline ingestion ...

prolonged QRS complex prolonged PR prolonged QT

Major factor in long-term mortality for Duchenne muscular dystrophy ...

respiratory insufficiency

Healthcare provider must protect confidential patient info unless ...

search warrant subpoena court order

Glycine elevations are seen in glycine encephalopathy (nonketotic hyperglycinemia) with the highest levels of glycine being in the CSF. What are used for tx?

sodium benzoate and dextromethorphan

Pts with symptomatic UPJ obstruction should have ...

surgical repair

Dietary restriction of what in chronic kidney disease needed?

phosphorus

Osgood-Schlatter dx made via ....

physical exam - tenderness over tibial tubercles - can be elicited by knee extension against resistance or passive knee flexion - rest, ice, proper stretching = tx

Traumatic myositis ossificans initial tx

physical therapy and immobilization - enables bone to heal and mass to resorb - excision may be indicated 6-12 mo after injury

Recognize cutaneous candidal infection

tx = topical clotrimazole or nystatin

Immunocompetent children with VZV treated with ...

tylenol symptomatically for fevers

Oseogenesis imperfecta is caused by defect in ...

type I collagen

Recent listeria outbreaks have been associated with consuming ...

uncooked hot dogs and goat cheese

Indications for indefinite anticoagulation ...

unprovoked proximal DVT or PE active cancer persistently elevated FVIII activity strong thrombophilia antiphospholipid Ab syndrome

Until what age should infants and children ride in a rear-facing car seat

until 2 yo or they reach height or wt limit set by car seat's manufacturer

Deficiency of what vitamins causes hemolysis in infants?

vitamin E deficiency - especially during 2nd month of life

Serious complication of unrepaired tetralogy of fallot with right-to-left shunting ...

brain abscess - along with cerebral thrombosis

Immigrated from Nicaragua. Cramp-like abdominal pain. Loose stools. Family with similar symptoms. Mild hyponatremia. Dx?

entamoeba histolytica

18 mo old has vocab of ...

10-25 words

rooting reflex disappears at what age ...

4 mos

Associated with symmetric IUGR

alcohol genetic abnormalities congenital anomalies early in utero infections chronic maternal anemia chronic maternal HT cigarette smoking maternal SLE

Laboratory data for neonate show hyperammonemia with anion gap acidosis; in the presence of urine ketones, what should be suspected.

an organic acid disorder

stepping reflex disappears when?

as early as 1-2 mos - extinguished by 3-4 mos

Has lowest progesterone and is OCP preferred for management of PCOS ...

drospirenone

Correct order of atopic march in children ...

eczema, food allergies, allergic rhinitis, asthma

Droplet and contact precautions are recommended for

hospitalized patients with - influenza - adenovirus - rhinovirus - coronavirus CAIR

silk glove sign associated with ...

inguinal hernia

Most sensitive test in children woth suspected UTI ...

leukocyte esterase

Mutation in what gene can lead to early onset DI ...

neurophysin

Gold standard for diagnosing food allergy ...

oral challenge

BM can remain in - refrigerator for: - freezer for:

refrigerator for 4 days freezer for 6-12 months

Eye symptoms noted in Williams syndrome

stellate pattern in iris

Can effectively reduce dental injury risk in young athletes

"Boil and bite" moldable mouthguards - Custom mouthguards are indicated for athletes with orthodontia, an underbite. or other dental malocclusion - Mouthguards are recommended for most young athletes participating in contact collision sports

Building a 3 cube tower is achieved by 50-90% of kids by what age?

15 months - can climb on furniture, bend down to pick up toy, drink from open cup when adult is holding it, point to one body part, use 3-5 words

Infants experience resolution of jaundice by ...

2-3 mos

Peak age of ALL

2-5 yo - most common childhood malignancy - poor prognostic factors = <1 yo and >10 yo

Recognize congenital long qt syndrome, in which the management is a med like nadolol

- EKG = bizarre T waves + prolonged QT wave - tx = beta blocker - it being a chronic prob differentiates it from hyperkaleia

caput succedaneum

- crosses suture lines!!! - resolves in 2-3 days - serial physical exams - can be associated with scalp bruising

Recommended STI prophylaxis after sexual assault includes the following:

- Empiric treatment for chlamydia, gonorrhea, and trichomoniasis - Hepatitis B vaccination for those unvaccinated, incompletely vaccinated, or vaccinated without adequate immune response - Human papillomavirus vaccination for those not previously or incompletely vaccinated - Assessment of HIV risk and provide, as indicated, HIV postexposure prophylaxis within 72 hours of the assault - Emergency contraception should be offered to all women

Indications for tympanostomy tube insertion include:

- Bilateral OME that lasts 3 months or longer with conductive hearing loss - Bilateral or unilateral OME that lasts at least 3 months together with risk factors for speech, language, or learning problems (eg, craniofacial anomalies, neurodevelopmental disabilities, etc) - Bilateral or unilateral OME that lasts 3 months or longer with tympanic membrane or middle ear damage - Recurrent acute otitis media (AOM) with OME at the time of evaluation

In prerenal acute renal failure caused by shock, the renal tubules respond to decreased perfusion by appropriately conserving sodium and water so that: - urine osmolality = - urine sodium = - FeNa =

- urine osmolality = >400-500 mosm/L - urine sodium = <10-20 mEq/L - FeNa = <1%

Hepatitis A vaccine or immune globulin can be used for hepatitis A postexposure prophylaxis and should be administered within

14 days of the exposure - Immune globulin should be used for hepatitis A postexposure prophylaxis in individuals younger than 12 months, older than 40 years, those who have vaccine contraindications, immunocompromised individuals, and individuals with chronic liver disease.

At what age does the American Academy of Pediatrics recommend that children can be unsupervised pedestrians?

10 yo

At what age should pediatricians first discuss drugs and sexual activity with pts with no increased risk for high-risk behaviors?

10 yo

Appx mortality rate for for anorexia following tx ...

10%

Healthy 3 yo M + mom pregnant with female child + father has hypophosphatemic rickets. Likelihood fetus will have hypophosphatemic rickets?

100% - X-linked dominant - completely penetrant --> 100% chance male would pass it to his daughter - Xp22.1

Small spontaneous pneumothoraces (<20%) can be treated with ...

100% O2 and re-evaluation n 4-6 hours

Researcher measured cholesterol of pts in community clinic. Average value is 200. Standard deviation 15 with normal curve. Likelihood that next pt will have cholesterol of 215?

16% - % of pop that lies outside 1 SD = 100% - 68% = 32% --> /2 = 16% In normally distributed pop - 1 standard deviation above or below mean contains 68% of pop being studied - 2 SDs above or below mean contains 95% of pop being studied - 2 SDs above or below = 99.7% of pop being studied

Learn to walk backwards and up steps with help at what age ...

16-18 mos

PCV13 should be administered to all children as a 4-dose series at

2, 4, 6, and 12 to 15 months of age - The minimum age at which PCV13 can be given is 6 weeks

Pt with overdose of drug X. Plasma concentration is 75 mg/mL. Inulin clearance rate is 100 mL/min. Tubular reabsorption of drug X is 5mg/minute. Expected excretion rate of this substance?

2.5mg/min = filtration rate - reabsorption rate = filtration rate - 5mg/minute - filtration rate can be estimated using inulin clearance rate ~ GFR = 7.5mg/100mL = 7.5 - 5 = 2.5mg/min

2 yo male + started on propranolol for SVT. Child does not receive a loading dose and receives therapeutic dose every 6 hours. Assuming propranolol half-life of 6 hours, how long will it take serum concentration to reach steady-state (>95%)?

30 hours - when no loading dose administered, therapeutic dose of first-order drug given every half-life will reach therapeutic steady state after 5 half-lives

At what age should child have all primary/deciduous teeth?

30 mos

Child can turn their head to the left and right in response to noise at what age ...

4 mos - sit with trunk support

What age knows 4 colors?

4 yo

Average kids gains how much per year from age 2 until puberty ...

5 pounds (2.3 kg) per year

Age: remembers telephone number

5 yo

Counting to 10 is a milestone of what age?

5 yo

Immune Thrombocytopenic Purpura (ITP) is a diagnosis of exclusion for a child with a recent viral illness and thrombocytopenia; what are a hallmark of the condition?

large platelets

Parents of 6 week old girl comment that their daughter consistently "winks" her left eye during feeding and ask if they should be concerned. On PE, ptosis of left eyelid is present; as the child sucks on the bottle, simultaneous eyelid blinking is observed. What best explains the etiology of these findings?

Abnormal innervation of oculomotor and trigeminal nerves Marcus-Gunn (jaw-winking) phenomenon - simultaneous eye-blinking during sucking-jaw movements as child contracts pterygoid muscle - surgical intervention is necessary when associated ptosis is severe or causes amblyopia

Following delivery of healthy male term infant, 18 yo, otherwise symptomatic mother (recently released from jail following her 3rd DUI conviction) is found to have a positive TB skin test and negative CXR. What best describes recommended management for mother and her newborn infant?

Begin rifampin in mother only; separation of mother and infant is not necessary - asymptomatic mom + positive TB skin test + normal CXR --> separation not recommended --> tx for latent TB infection = daily rifampin for 4 mos (replaced 9 mos of isoniazid)

Initial screening labs for cutaneous and GI angioedema ...

C4 levels of C1 inhibitor protein functional activity of C1 inhibitor

varicella-zoster virus fetopathy clinical manifestation

CNS - aplasia, microcephaly, hydrocephaly, calcifications Eyes - microphthalmia, optic atrophy, cataracts, chorioretinitis Extremities - limb atrophy, limb reduction, underdev't Skin - cicatricial scarring, hypopigmentation

5 yo M + diarrhea and chills + multiple loose bowel movements mixed with blood for 2 days. has not traveled or been exposed to abx. Ill-appearing. 3+ cap refill. Dry mucus membranes. Cause?

Campylobacter - diarrhea bloody in more than half of cases - fever, abdominal pain, vomiting

15 yo M + abdominal pain for last 3 mos + feels bloated, has loose stools, has not had much of an appetite. Feels more tired than normal and has increasing headaches. In past few days, has had ulcers in his mouth, and his dentist described a new but marked loss of enamel from his mouth. Cause of symptoms?

Celiac disease - aphthous ulcers

What meds are contraindicated during breastfeeding?

Chemotherapy drugs oral retinoids iodine amiodarone lithium

Can be used to diagnose CDH

Chest x-ray

98% of meylomeningocele patients have associated

Chiari malformation type II - hydrocephalus commonly seen due to obstruction

7 yo M + target lesion that developed after a tick bite. Parents refuse medical eval and treatment bc of their religious beliefs. Best course of action?

Consult CPS and/or obtain court order

Girl in college + confused, hyperthermic, red colored urine. Went to party previous night. Etiology?

Ecstasy related reaction - malignant hyperthermia = inherited = caused by anesthesia or stimulant drug

A 4-day-old male newborn is seen for a routine check; he has yellow skin from face to chest. He is full-term, had a normal vaginal delivery, weighed 3.180 kg at birth, and was 3.100 kg at discharge. He is breastfeeding well and stooling and voiding regularly. He is well hydrated and active. Laboratory results reveal an indirect bilirubin of 12.2 mg/dL and a direct bilirubin of 1.0 mg/dL. What is the physiologic mechanism behind this jaundice?

Elevation of indirect bilirubin due to increased bilirubin production - Unconjugated indirect bilirubin enters the liver and is excreted from the hepatic cells in a water-soluble form (conjugated and direct) into the biliary system. The direct bilirubin leaves the body in the form of stool. - The most common cause of indirect hyperbilirubinemia is benign neonatal hyperbilirubinemia (physiologic jaundice), which peaks at 2-4 days of life. Benign neonatal hyperbilirubinemia is due to an elevation of indirect bilirubin from increased bilirubin production after the breakdown of red blood cells, in conjunction with limited conjugation of bilirubin by the immature liver. - The increased breakdown of red blood cells occurs because fetal red blood cells have a shorter lifespan than adult red blood cells. Newborns have more red blood cells than adults with a hematocrit between 50-60%. Benign neonatal hyperbilirubinemia can be worse in breastfed newborns due to a higher level of dehydration as the mother waits for her milk to come in.

During exam of 14 yo boy with 6 hr hx of right testicular pain, bluish discoloration overlying superior lateral portion of right scrotum is noted. Localized tenderness of discolored area is identified along with normal cremasteric reflex. Most likely cause of this pt's clinical signs and symptoms?

Epididymitis - torsion of appendix common from 7-12 yo - "blue dot sign" - tx = analgesics, bed rest, scrotal support

LGA baby + born after difficult breech delivery + has tachypnea + X-ray = unilateral diaphragmatic elevation. Etiology

Erb's palsy

Which glycogen storage disease: is a glycogen and lysosomal storage disorder classified as either infantile-onset (IOPD) or late-onset (LOPD). Clinical features of IOPD include marked hypotonia, failure to thrive, and hypertrophic cardiomyopathy within the first year of life. Clinical features of LOPD include proximal muscle weakness with respiratory insufficiency; there is no cardiac involvement

Glycogen storage disease II, also known as Pompe disease

Which glycogen storage disease: includes several subtypes affecting liver, muscle, and other tissues to varying extents. Subtypes include: classic progressive hepatic, nonprogressive hepatic, the rare childhood neuromuscular form, as well as lethal forms (perinatal and congenital neuromuscular subtypes).

Glycogen storage disease IV

Cause of severe diarrhea in neonatal period. Caused by deficiency in intestinal sodium/glucose transporter. Diarrhea resolves when glucose and galactose eliminated from diet.

Glucose-galactose malabsorption

Woman in labor with full-term child. She has one other child and states he had a "strep prob" after being born and needed IV abx. She had a UTI 4 weeks ago and was given abx. GBS screen 3 weeks ago was negative. Next best step?

Give GBS prophylaxis immediately - possible invasive GBS dz in previous infant

13 yo admitted to hospital for surgical procedure. Child and parents are Jehovah's Witnesses. Request no blood transfusions during sx. Express understanding of potential consequences of not receiving blood transfusion. During sx, pt experiences life-threatening hemorrhage. Next step in management?

Give blood transfusion bc pt is a minor

You are evaluating newborn full-term infant with abnormal facies. Mom is 24 yo G1P1 with no prenatal care who presented to L&D after 6 hours of contractions with intact membranes and normal vitals. Male delivered without complications. He is small with "low-set jaw." SGA. Has micrognathia, posteriorly rotated and low-set ears. Epicanthal folds. Single transverse palmar crease. Syndactyly of 2nd and 3rd toes. Hazy appearance of right cornea with red reflexes. Muscular tone decreased. Infant sucks and swallows poorly with first feeding. What is true?

Has Smith-Lemli-Opitz syndrome + concominant hypospadias, CHD, death by 18 months of age - AR - defective cholesterol synthesis - defective CNS myelination, cataract formation

Key mediator in eosinophilic activation and plays important role in pathogenesis of atopic disease ...

IL-5

Immunocompromised + dehydration + shigellosis. Tx?

IV CTX + hydration

A pt on diuretics presenting with coma following a seizure. Lab results reveal sodium of 118. Appropriate emergency management?

Immediately raise serum sodium by 2-3 mEq/L with 3% saline - For severe symptoms such as seizure or coma, 3% saline is administered to immediately raise serum sodium by 2-3 mEq/L - After that, goal should be no more than 0.5 mEq/L/hour correction

HSP is also known as ...

Immunoglobulin A vasculitis - elevated serum IgA

What infants should undergo allergy testing before being introduced to peanut-containing foods?

Infants with severe eczema or egg allergy

A mother brings in her 6-month-old girl due to a 1-week history of feeding difficulty with occasional vomiting, increased fussiness, and some mild stiffening of her legs. She was born at term, and there were no problems during pregnancy or at delivery. She quickly began feeding well and achieved her major motor, language, and social milestones on time for the first 5 months. On exam, she appears alert but fussy, and she looks well nourished. She exhibits fist clenching and mild spasticity of her lower extremities. Lungs, heart, and abdomen are normal. She is unable to sit without support or bear any weight on her legs. She has head lag on pulling her to a sitting position. According to the mother, she was performing these actions a few weeks ago. Routine blood chemistry tests and urinalysis are normal. CSF shows elevated protein levels, and an MRI reveals areas of high intensity in the brainstem. What is the most likely diagnosis?

Krabbe disease - autosomal recessive sphingolipidosis caused by deficient activity lysosomal enzyme galactocerebrosidase (GALC), leading to destruction of oligodendroglia and the CNS and subsequent demyelination - typical age of onset is 3-6 months - an infantile onset or classic form (most cases) follows normal early development; it is characterized by spasticity, irritability, hypertonia, hyperesthesia, psychomotor arrest, then rapidly progressive neurologic degeneration, hyperreflexia, optic atrophy, persistent decerebrate posturing, and early death - feeding difficulties, such as vomiting and reflux, may cause failure to thrive. - CSF reveals elevated protein levels and an abnormal pattern of electrophoresis. Measurement of GALC activity helps confirm the diagnosis. - MRI detects high-intensity areas of demyelination in the brainstem and cerebellum.

15 yo girl with prolonged hx of persistent headaches, which are associated with several episodes of vomiting, presents with complaint of awakening with double vision. She is alert and oriented but frightened by her visual symptoms. On neuro exam, left eye is medially deviated, and she is unable to abduct eye beyond midline. Detailed fundoscopic exam reveals optic nerve edema. No evidence of mass lesion, ventricular enlargement, or vascular abnormality on MRI of brain. Best next step?

LP with opening pressure and CSF analysis - IIH

Imaging modality of choice in neonatal stroke. It can firmly establish diagnosis of either ischemic or hemorrhagic lesions, as well as identify arterial occlusion or stenosis, vascular malformations, and cerebral sinovenous thrombosis (CSVT) ...

MRI, supplemented by MR arteriography (MRA) and MR venography (MRV)

Dysmenorrhea in teen. Never sexually active. Lower abdominal pain worse with menstruation. Low back pain, nausea. No other causes. Tx?

NSAID ~ ibuprofen or naproxen - 1-2 days before onset of menses to 2-3 days after - stay hydrated since these meds are hard on the kidneys - primary dysmenorrhea

Children with sickle cell disease are at high risk of invasive pneumococcal infections and should receive what scheduling of pneumococcal vaccine?

PCV 13 at 2, 4, 6, 12-15 mos PPSV23 at 2 yo, followed by 2nd dose 5 years later

4 hour old infant + cyanosis and difficulty breathing. Awake with tachypnea. No significant retractions. Central cyanosis. 3/6 continuous murmur that is machinelike in quality. Lung fields clear. No abnormal lung sounds. Pulse ox in right hand is 68%. W/u rules out transposition of great arteries. Cause?

Pulmonary atresia - 2nd most common cause of cyanotic heart disease in first few hours of life - cyanosis, single second heart sound, PDA

AVSD in its severe forms causes early pulm HT and potentially Eisenmenger syndrome. What can protect lungs from overcirculation?

Pulmonary valve stenosis

Cohort study designed to examine dev't of MDD in children of divorced parents compared to children with married parents. What outcome measure is most appropriate for this study design?

Relative risk - probability of an event happening when comparing exposed and non-exposed group

Patient has premenstrual dysmorphic disorder (affects 5% of adolescents). What's approved for tx?

SSRIs: - fluoxetine - sertraline - controlled-release paroxetine - symptoms usually begin last week of luteal phase, followed by remission within 2-3 days of onset of follicular phase - SSRIs may be administered daily, during luteal phase only (starting on cycle day 14), or only at onset of symptoms

Most common arrhythmia associated with WPW ...

SVT

Most common form of scoliosis affecting 4-8% of patients especially boys. Usually round. Usually presents in late childhood with wedged vertebral bodies and cause is unknown but possibly genetic.

Scheuermann's disease

12 yo with hx of intellectual disabilities found to have loud systolic ejection murmur best heard at 1st right intercostal space and associated with thrill at both 1st right intercostal space and in suprasternal notch. Dysmorphic facial features also noted, including periorbital fullness, broad forehead, flattened nasal bridge, and wide mouth with full lips. What best describes the expected personality characteristics of this patient?

Strong expressive language skills, socially engaging, often perceived as charming - Williams syndrome - associated with supravalvular aortic stenosis - "elfin-like" facial features - unafraid of strangers - preference for interacting with adults as opposed to peers - deletion of of elastin gene located on chromosome 7q11.23

14 yo M + well aside from mild intermittent right leg pain for past 3 mos + denies traumatic events or difficulty with ambulation + large soft tissue mass tender to palpation + noted at distal metaphysis of femur + radiography = disruption of normal bone trabeculation with cortical destruction and associated soft tissue mass with variable ossification. Genetic mutation likely associated with pt's condition?

TP53 - osteosarcoma - and RB

14 yo girl + 4 mos after initial exam to discuss further management of her frequent headaches. She reports her discomfort improves with the rizatriptan that you have previously prescribed, but the episodes still occur ever 2-3 days. Her headaches are preceded by 20 minutes of visual symptoms, which resolve at onset of pain. She describes typical episode as bilateral pulsing headache accompanied by nausea, sensitivity to light and sound, occasional vomiting. Neuro exam is unremarkable and recent MRI of brain is normal. Most appropriate tx for this pt?

Topiramate - approved for ages 12-17 yo - chronic migraine headache with aura - has persisted despite abortive measures - HA prophylaxis indicated for migraines that occur 3-4 times per month or if prolonged and debilitating

History and physical examination findings for adolescent suggest interarticular hip pathology. What is the best initial step to assess the bony anatomy of the hip?

Weight-bearing radiography

16 yo M basketball player + 1 day of right arm pain and swelling + no trauma + developed gradually throughout practice and had to stop playing + visibly dilated veins and cyanosis of right arm. Cause?

UE DVT - Paget-Schroetter syndrome - axillary-subclavian vein thrombosis - associated with strenuous and repetitive activities of UE's - favors dominant arm

A 2-year-old male patient presents for a follow-up evaluation of a mid-abdominal bulge present since birth. The parents report that the bulge has gradually increased in size over the last year and increases in size with crying and straining. It does not appear to be painful. Examination reveals a dome-shaped bulge covered with taut, shiny, thin-appearing skin, 4.5 cm in diameter and apparently originating from the umbilicus. At the base of the mass, a 3-cm umbilical ring is palpable. What is the primary indication for surgery referral for this patient?

Umbilical ring size over 1.5 cm - Defects of 1.0 cm or less usually close spontaneously; those greater than 1.5 cm are less likely to close spontaenously. E - Expectant management of asymptomatic umbilical hernias until age 4-5 is considered safe regardless of the size of the defect. Surgical referral is indicated in this patient due to the umbilical ring size. - Spontaneous closure is less likely in patients with a fascial opening (umbilical ring) greater than 1.5 cm or a significant amount of protruding skin and in patients over age 5 or with an underlying predisposing condition (Down syndrome, Beckwith-Wiedemann syndrome).

Recognize aspergillosis and the tx for it ...

Voriconazole - neutropenia --> increased risk for pulmonary aspergillosis - triad = fever, pleuritic chest pain, hemoptysis

Wiskott-Aldrich syndrome inheritance ...

X-linked recessive

Egg allergy is a concern for what vaccine?

Yellow fever vaccine - refer to allergist for evaluation and possible skin testing

Compared with term infants, premature infants have decreased accretion of calcium and phosphorus and therefore require increased calcium and phosphorus intake. Unfortified human milk and standard term formulas do not meet the increased calcium and phosphorus requirements of the preterm infant. Calcium and phosphorus deficiency can lead to osteopenia of prematurity (also known as neonatal metabolic bone disease). Use of fortified human milk or a fortified premature formula improves bone mineralization and therefore decreases the risk of developing osteopenia of prematurity. Preterm formula should be used, in most cases, until

a corrected gestational age of 40 weeks - Preterm neonates have increased calcium and phosphorus requirements compared with those delivered at term. - Use of fortified breast milk or enriched preterm formula with increased calcium and phosphorus can decrease the likelihood of the development of osteopenia of prematurity.

The boy in the vignette has significant short stature, a normal body mass index, a concordant bone age, and a high upper-to-lower body segment ratio (U/L ratio), indicating relatively longer trunk and shorter legs. These findings are most consistent with

a skeletal dysplasia - The upper segment is calculated as height minus the lower segment. - Infants have a relatively high U/L ratio (about 1.7 at birth), which gradually decreases to 1 around age 10 years, reaches a nadir during early puberty, and returns to about 0.9-1 by adulthood. - A high U/L ratio indicates a relatively longer trunk and shorter legs, and it can be seen in skeletal dysplasias and precocious puberty. - A low U/L ratio indicates a relatively shorter trunk and longer legs, and it can be seen in Klinefelter syndrome and delayed puberty.

neurologic disorder presenting with acute paralysis of 1 or more limbs, preserved sensation, and normal mental status, typically within a week of onset of viral symptoms

acute flaccid myelitis - Clinical severity is variable. - Magnetic resonance imaging (MRI) of the spine will show extensive longitudinal centrally located inflammation affecting the gray matter of the cord. - Cerebrospinal fluid analysis typically reveals a lymphocytic pleocytosis. - Treatment is supportive; there is currently no recommendation for adjunctive therapies such as intravenous immunoglobulin, corticosteroids, or plasmapheresis. - Recovery is variable and often incomplete.

The mainstay of treatment for croup is

administration of oral, nebulized, or parenteral corticosteroids - A single dose of oral dexamethasone is beneficial for children with mild croup in the outpatient setting. - Therapeutic options for children with moderate to severe croup include dexamethasone (oral or intramuscular), nebulized epinephrine, and nebulized budesonide. - In most patients with croup, symptoms improve within 48 hours. *The diagnosis of croup is clinical, based on history and physical examination: abrupt onset of seal-like barking cough and inspiratory stridor associated with hoarseness of voice, low-grade fever, and rhinorrhea.*

The treatment of mild allergic contact dermatitis ...

aimed at reducing pruritus - topical antipruritic agent (a lotion such as calamine or a product containing pramoxine) - midpotency topical corticosteroid, an oral antihistamine - cool tap water compresses (these are soothing, promote drying of lesions through evaporation, and remove crust) For more widespread involvement or severe involvement of the face or genitalia, systemic corticosteroid therapy is useful. - Prednisone (beginning at 1 mg/kg per day or its equivalent) is tapered gradually over 2 to 3 weeks; earlier cessation may result in a rebound of the dermatitis.

Infant with OTC deficiency. What is likely to be increased?

ammonia - X-linked - most common urea cycle defect

Bacterial pneumonia in preschool and school-aged children is most often caused by Streptococcus pneumoniae for which what is the appropriate treatment?

amoxicillin

Women who develop lyme dz during pregnancy can be treated with ...

amoxicillin

Girl has persistent bacterial bronchitis, a relatively recently described cause of chronic cough in children. Before initiation of usually fruitless diagnostic tests, what is indicated in children who have no risk factors for underlying disease?

an extended course of antibiotics - A chronic cough with no abnormal physical examination findings and no history findings suggestive of a specific etiology is considered a nonspecific cough. - Appropriate diagnostic investigations in this context include chest radiography and a pulmonary function test. - No additional investigations are indicated unless results of these tests point to a specific diagnosis requiring further evaluation. - For a child with a chronic wet cough with no other abnormal findings suggests persistent bacterial bronchitis; the most appropriate management is a 2- to 4-week course of amoxicillin/clavulanate or equivalent narrow-spectrum antibiotic appropriate for respiratory flora.

Metabolic dz'es are most likely to have what inheritance pattern?

autosomal recessive

15 yo male + fever and sore throat associated with pruritic rash. You note pharyngeal exudates, cervical LAD, and maculopapular rash. Rash began on extensor surfaces of distal extremities and spread centripetally to chest and back. It spares his face, palm, and soles. Rapid strep is negative, as well as rapid mono test. Cultures are sent for strep pyogenes. Throat cultures return negative except for gram-positive rod that is growing on blood-enriched media. Most likely organism?

arcanobacterium haemolyticum (formerly corynebacterium) - increasingly common cause of pharyngitis in young adults and adolescents - can only be cultivated on blood-enriched media - tx = erythromycin but can also resolve without treatment

Management for kid <5 yo who has been exposed to TB

at high risk for disseminated diseases = CXR --> start therapy for LTBI (even if initial TB test is negative) - repeat TST 8-12 weeks later --> is positive, complete course

Bacterial sinusitis is present in 5% to 10% of cases of URI; it is distinguished from a simple viral URI by:

at least 10 days of symptoms without improvement new or worsening fever (> 39°C) worsening cough after initial improvement - A thorough history should be obtained to help distinguish between bacterial sinusitis and a new viral URI

15-30% of kids with T1D will develop ...

autoimmune thyroid dz - Hashimoto or Grave's

The World Anti-Doping Agency (WADA) has banned the use of this BP medication for a teen who needs antihypertensive medication but plays archery ...

beta blockers - banned in precision or control sports (e.g., shooting, archery, and golf) - rationale is that beta blockers can have an anxiolytic effect and can steady the hands; beta blockers, such as propranolol, have been used to treat 'stage fright' and essential tremor

20% of pts with bulimia have what personality disorder?

borderline personality disorder

Intellectual disability is present when

both intelligence and adaptive functioning are more than 2 standard deviations below normal (scores <70)

Pts with Klinefelter have increased risk of developing what cancer?

breast cancer

Priapism can be a consequence of SCD. Preferred intervention for acute priapism not responding to hydration and oxygen within 4-5 hours ....

exchange transfusion or hypertransfusion

observational studies that calculate relative risk from an exposure (incidence in exposed/incidence in unexposed)

cohort study

What foods need to be eaten after exercise to help replenish muscle glycogen and protein

carbs and protein - both = 4 kcal/g

Low levels of T4 stimulate an increase in TSH. An inappropriate TSH response to low circulating T4 is consistent with ...

central hypothyroidism

Recognize perianal bacterial dermatitis, in which the management is

cephalexin - Perianal bacterial dermatitis is a superficial cellulitis caused by Streptococcus pyogenes or Staphylococcus aureus. - The peak incidence is between 3 and 4 years of age, and boys are affected more often than girls. - Perianal bacterial dermatitis may be treated with a 14-day course of oral penicillin or amoxicillin. However, because S aureus appears to be an increasingly common cause, some clinicians prefer oral cephalexin or another antibiotic that possesses antistaphylococcal activity based on local resistance patterns or the results of bacterial culture and sensitivity.

Appx 10% of pts with coarct of aorta also develop Berry aneurysms. What screening is recommended?

cerebral angiographic study - ages 10 and 20

Tx of choice for children with DDH who are older than 6 mos at time of dx ...

closed or open reduction in OR under anesthesia with hip spica cast

Atypical Mycobacterium infection often presents with subacute to chronic, unilateral cervical lymphadenopathy without systemic illness in previously healthy young children. The superior anterior cervical lymph nodes or submandibular nodes are most frequently affected in atypical mycobacterial infection. The treatment of choice for atypical Mycobacterium cervical node infection is

complete surgical excision of the affected lymph node

Most appropriate diagnostic test for Paget-Schroetter syndrome ...

compression US of affected extremity - gold standard = contrasts venography

Tick repellants containing DEET (N,N-diethyl-3-methylbenzamide) at what concentration are needed for tick prevention?

concentrations >10% should not be used in children - Concentrations of 10-35%, are usually sufficient even in adults

Hemoglobin electrophoresis int pts with SCD will show ...

elevated HbS low HbA compensatory increased HbF

Reverse differential cyanosis, in which post-ductal oxygen saturation level is higher than pre-ductal level, is seen in

dextro-transposition of great arteries + either pulm HT or coarct of aorta

Prophylactic antibiotics that should be given after an avulsed tooth is replaced.

doxycycline or amoxicillin

Hemothorax is a known complication of central venous access, particularly of the subclavian vessels. Large pleural effusions may impair respiratory function and require

drainage with a thoracostomy tube or therapeutic pleurocentesis

Regarding medications, children have higher metabolic capacity, so ...

drugs must be given at shorter intervals

Vitamin A deficiency can be associated with what eye symptoms ...

dry eyes - night blindness, bitot's spots too

Mittelschmerz is often described as

dull ache at the time of ovulation - usually occurring 1 to 2 weeks before the onset of menses - can last for a few minutes to several hours

One of the most common causes of chronic diarrhea in kids 2-18 yo and associated with asthma and eczema ...

eosinophilic gastroenteropathy

Biopsy of erythema toxicum reveals ...

eosinophils

The abrupt onset and absence of a viral prodrome are typical of

epiglottitis - progressively severe sore throat - muffled voice - drooling - air hunger and fatigue if no intervention - sudden onset of high fever - usually no hoarseness or cough

Pertussis in young infants can have an atypical presentation characterized by

gagging, gasping, apnea, or bradycardia without the characteristic "whoop"

Infant + hypoglycemia + acidosis + normal ammonia (KEY) + ketones + positive reducing substance in urine. Dx?

galactosemia - reducing symptoms from galactose spilling over into urine

What functions in a negative feedback manner on gastric acid secretion ...

gastric D cells - somatostatin = major inhibitor of gastric acid secretion and is released by gastric D cells --> hormone acts through inhibition of both histamine and gastrin release

Management for scoliosis in milder cases (< 20 degrees) of curvature ...

general activity encouraged + no restrictions

Potential complication of hyphema

glaucoma

Has a transport max for reabsorption, above which the filtration rate exceeds reabsorption rate ...

glucose

Growth spurt height velocity when entering puberty earlier...

greater height velocity than peers

Volume resuscitation and sodium bicarb are mainstays of tx for salicylate toxicity but difficult to administer in pt with non-cardiac pulm edema. Presence of pulmonary edema = absolute indication for ...

hemodialysis - in setting of pulmonary edema + salicylate poisoning

Tay-Sachs dz caused by a deficiency of ..

hexosaminidase A

Is insulin high or low in acanthosis nigricans

high

Infant has urine that is normal initially but becomes dark brown to black when exposed to air. Hallmark of alkaptonuria or dark urine disease. AR defect in ...

homogentisic acid dioxygenase - involved in tyrosine metabolism

Murmur AS does what in squatting position?

increases

Child has hyponatremic dehydration caused by excessive sodium loss from vomiting and diarrhea, exacerbated by attempted rehydration with hypotonic fluids (popsicles and water). What are the following values: - serum osmolality - urine osmolality - urine sodium

low serum osmolality high urine osmolality low urine sodium - His loss of sodium is extrarenal; therefore, his urine sodium levels would be low and there would be water conservation by the kidneys in response to dehydration, resulting in a high urine osmolality

Neonatal acne is a common disorder, affecting up to 20% of healthy neonates. Involvement usually is limited to the face and most often presents within the first 30 days of life. This acneiform eruption is thought to be related to stimulation of sebaceous glands by maternal androgens and colonization with yeast species. Malassezia furfur and Malassezia sympodialis often are visible on pustule smears. Whether what most pediatricians know as neonatal acne is true acne is controversial. Many pediatric dermatologists prefer the term neonatal cephalic pustulosis to describe this common neonatal process. Most cases resolve spontaneously in several weeks and do not require treatment. In pronounced cases, what can be used?

ketoconazole cream, 2% or hydrocortisone cream, 1%, can be used - If true comedones or nodular lesions are noted, the child should be treated for infantile acne

BP med with significant risk of major congenital malformations among infants exposed to it. Congenital CV (septal defects, PDA) and CNS (neural tube defects) anomalies often reported following exposure during 1st trimester.

lisinopril

Children treated with cranial radiotherapy at least than 2 yo can have ...

major cognitive impairments

Most significant risk factor for suicide in female adolescents ...

major depression

abnormal tissue development leading to congenital anomalies, such as atrioventricular canal defect in trisomy 21

malformation sequence

Hemihyperplasia/hemihypertrophy are associated with what significant comorbidity?

malignancies, including intraabdominal tumors - especially during first 10 years of life

Risk factors for neonatal hypoglycemia

maternal diabetes LGA or SGA prematurity (Before 37 weeks gestation) neonatal sepsis hypothermia inborn errors of metabolism genetic overgrowth syndromes

Football player falls to ground after receiving valgus blow to outside of knee. What is injured?

medial collateral injury - tibial collateral ligement

defined as failure of a planned action to be completed as intended (error of execution) or use of wrong plan to achieve an aim (error of planning).

medical error

Boy who swallowed lamp oil. CXR c/f aspiration for volatile gases, causing ARDs. Next step?

oxygen - hydrocarbon aspiration

1st line tx for cluster headaches

oxygen + triptans

Bacterial vaginosis associated with what pH?

pH >4.5 (normal < 4.5) - this is part of Amsel criteria - elevated pH also associated with trichomoniasis

Iliotibial band friction syndrome description ...

pain during exercise, located over lateral femoral condyle

Osler nodes, associated with subacute bacterial endocarditis, are...

painful, violaceous nodules located in pulp of fingers and toes

severe, postprandial abdominal pain, history of hematemesis, weight loss, with epigastric tenderness =

peptic ulcer disease - acid-suppressing treatment with proton pump inhibitor (PPI) therapy should be initiated - chronic use of ibuprofen, a nonsteroidal anti-inflammatory drug (NSAID), increases the risk for development of peptic ulcer disease (PUD) in this adolescent - gastroenterology referral, and consideration of endoscopic evaluation, is also indicated - the presence of upper gastrointestinal bleeding (eg, hematemesis, melena, or brisk hematochezia) should raise immediate concern for PUD and result in urgent gastroenterology consultation - medical treatment of peptic ulcer disease can include use of proton pump inhibitors, histamine-2 receptor antagonists, and surface-coating agents (sucralfate)

Puts neonates at increased risk of renal vein thrombosis ...

perinatal asphyxia

A 10-year-old girl presents with a 2-month history of a lesion on the bottom of her foot. It has recently begun to hurt her and her parents bring her for an evaluation. No history of trauma is elicited. Past medical history is unremarkable. She is on no medications. On examination of her foot, you note a rough, keratotic, irregular lesion approximately 5 mm in diameter. Paring away the top of the lesion reveals multiple black dots in the midst of the lesion. The lesion is also tender upon direct pressure. What is the most likely diagnosis?

plantar wart - caused by the human papillomavirus (HPV) 1, and they are typically flush with the skin due to the pressure of ambulation - can be quite painful - usually be distinguished from other foot lesions by the presence of multiple black dots that represent thrombosed capillaries - many treatment modalities exist, including salicylic acid plasters or patches, trichloroacetic acid, liquid nitrogen cryotherapy, cantharidin application, and electrodesiccation.

Traumatic Tympanic Membrane Perforation management

primarily supportive --> heal on own

Underlying mechanisms of migraines ...

primary neuronal dysfunction

Management for peripheral pulmonary artery stenosis ...

reassurance

To decrease the risk that GERD will be misdiagnosed as EoE, children with suspected EoE should be treated with

proton pump inhibitor - for a minimum of 6 to 8 weeks before diagnostic endoscopy

Benign bone lesions often appear on radiographic imaging as solitary, discrete lesions with sclerotic margins, while malignant tumors often have ill-defined margins, periosteal reactions, or soft-tissue extension. Pain related to osteoid osteomas typically responds well to nonsteroidal anti-inflammatory drugs, worsens towards the end of the day, and is not related to activity. Management?

provide reassurance and arrange for outpatient follow-up with an orthopedist

Most common pathogen of otitis externa ...

pseudomonas aeruginosa

Centronuclear myopathy presents with ...

ptosis ocular palsies hypotonia - congenital myopathy

Hearing screen recommended n 6 yo ...

pure tone audiometry - school aged child = pure tone audiometry

On exam, a newborn seems well except for the appearance of the right upper extremity: held adducted and internally rotated at the shoulder with extension of the elbow. The forearm is pronated with flexion of the wrists and fingers. The patient appears well and in no apparent distress. What is the best next step in the management of this patient?

refer to PT - brachial plexopathy, an injury associated with traction placed on the C5-T1 nerve roots during birth. It may also occur in the context of shoulder dislocation and with primary tumors at the apex of the lung. Management of suspected brachial plexus injury requires: - Gentle handling of the affected limb - Radiographs of the clavicle and humerus to exclude bone injury - Observation for respiratory distress indicating diaphragmatic injury - Referral to physical therapy for passive range of motion exercises of the neck and proximal arm beginning 7-10 days after birth

A previously healthy, 1-year-old girl is screened for lead exposure with a venous sample. She lives in a community with a high density of homes built before 1950. She has had normal growth and development. The laboratory result is 6 µg/dL (0.29 μmol/L). Nutritional assessment and counseling about potential environmental exposures are provided. Of the following, the MOST appropriate next step is

repeat testing in 1-3 mos - There is evidence that measurable blood lead levels less than 5 µg/dL (<0.24 μmol/L) are associated with lower academic achievement and lower IQ scores, increased incidence of attention-deficit/hyperactivity disorder, and potentially also delayed puberty and impaired renal function. - Additionally, levels less than 10 µg/dL (<0.48 μmol/L) are associated with delayed puberty, reduced growth, and hearing impairment. - With a blood lead level of 6 µg/dL (0.29 μmol/L) --> should have a nutritional assessment, be counseled about potential environmental exposures, and have her lead level retested in 1 to 3 months.

Most common cause of mitral stenosis ...

rheumatic fever

Juvenile colonic polyps are relatively common and generally present in first decade of life. Typical age is 2-10 yo (with peak at 3-4 yo). What is the recommended screening if no suspicious characteristics on pathology?

routine screening not recommended until adulthood

What type of E coli... - bloody stools - no fever - exposure to petting zoos/cattle

shiga-toxin producing

15 yo gymnast + insidious lower back pain + no palpable paraspinal muscle tenderness or spasm. Full ROM of lumbar spine but pain made worse with extreme extension of spine. Deep tendon reflexes and neuro exam of LE's are 2+ (normal), symmetric, equal. Most likely diagnosis?

spondylolysis - defect in pars interarticularis

The umbilical stump usually falls off after 1 to 2 weeks, but may take up to 3 weeks. Before the umbilicus is completely healed and dried, caregivers should limit bathing to

sponge baths only

In a 3 mo old, illness started with cough, fever, and difficulty in breathing, suggesting the possibility of pneumonia. X-ray of the chest shows tension pneumothorax on the left side, with collapse of the left lung. Trachea and mediastinum are shifted towards the right side. There is blunting of the left costophrenic angle, suggesting the presence of fluid in the left pleural cavity. The most likely cause of this condition, in this case, is

staphylococcal pneumonia - hydropneumothorax is due to the rupture of a pneumatocele. - Staphylcoccal pneumonia commonly is diffuse and causes microabscesses which in turn form pneumatoceles. - A large pneumatocele can rupture, leading to pyopneumothorax, which requires intercostal drainage under water seal. It is also known for producing rapid symptom onset, high fever, and abdominal distension which can be confused with an acute abdomen.

Where to test for botulism toxin?

stool

Nontyphoidal Salmonella is the most common cause worldwide of bacterial gastroenteritis; the highest incidence is in children under the age of 5 years. Antimicrobial therapy for nontyphoidal Salmonella is reserved for infants younger than 3 months of age and those with a chronic gastrointestinal disease or immunocompromised condition. Children with nontyphoidal Salmonella can return to daycare when:

stools are contained in the diaper or when toilet-trained children no longer have accidents using the toilet and stool frequency is no more than 2 stools above that child's normal frequency, even if the stools remain loose

linear scleroderma typically treated with

systemic corticosteroids and/or methotrexate - to reduce the chances of permanent disfigurement, though sometimes small lesions that do not involve joints or the face can be treated with localized phototherapy, topical corticosteroids, or topical immunomodulators

In medicine, the ethical principle of beneficence means that

the physician should act for the patient's well-being - Respect for autonomy means that physicians should accept a patient's decision to agree to or decline treatment if the patient has the ability to understand the treatment and its risks, benefits, and alternatives

Confirms dx of Graves disease

thyroid-stimulating immunoglobulin

Initial symptoms of salicylate toxicity ...

tinnitus and vertigo

Nearly all clotting factors are synthesized by the liver, so levels will be affected with severe liver disease. 3 main exceptions are ...

tissue factor von Willebrand factor Factor 8

corneal abrasion management ...

topical abx ointment + oral analgesia

A majority of adolescents with acne can be treated with topical medications. What is a great therapeutic starting point?

topical retinoids and benzoyl peroxide - many retinoids are deactivated by benzoyl peroxide so must be used at different parts of the day - females must be on 2 forms of effective birth control for at least 1 mo prior to therapy, during therapy, and for 1 mo after therapy - in addition, 2 negative pregnancy tests are required to initiated therapy, and monthly pregnancy tests must be conducted through duration of therapy and 1 mo after completion of therapy

Nocardia species are aerobes that are weakly acid-fast and Gram-positive on Gram stain. While Nocardiosis is typically associated with immunosuppression, immunocompetent individuals are still susceptible. Pulmonary infection causes either pneumonia, lung abscess with cavity formation, lung nodules, or empyema. Primary lung infection can disseminate to other tissues, including the brain. The treatment of choice is

trimethoprim-sulfamethoxazole

Aplasia cutis congenita may be associated with what syndrome?

trisomy 13

Adult gait pattern is typically not established until what age?

6 to 8 years of age - most cases of in-toeing spontaneously resolve

At what age can kids write their first and last name ...

6 years

At 4 weeks old, infants show interest in objects that are how far away...

8 to 12 inches away - especially those that are black and white

Inheritance pattern of albinism ...

AR

Most common cause of SNHL is ...

AR non-syndromic disorder caused by connexin 26 mutations = 1/2 of all hereditary hearing loss

Amish parents bring their lethargic 3-week old infant to the emergency department. The parents mention difficulty since soon after birth. The baby opens his eyes and withdraws only to pain; there is no verbal response to pain. Pupils are reactive and there is tachypnea and hyperpnea. He is afebrile. His urine has a burned sugar odor. Laboratory studies indicate metabolic acidosis. What is the most likely cause of the coma?

Accumulation of branched-chain ketoacids - predisposition of Amish families and the characteristic odor of the urine suggest maple syrup disease. This is an aminoacidopathy due to the deficiency of α-ketoacid dehydrogenase; this leads to the accumulation of branched-chain ketoacids.

Most commonly used decontamination method.

Activated charcoal - but is declining in use - may have benefit if administered within 1 hour of ingestion - contraindications = risk of aspiration, late presentations, pts who require endoscopy, heavy metal ingestion, alcohol ingestion, inorganic ion ingestion

16 yo girl evaluated for fever, headache, and myalgias. Has sore throat. Sexually active with multiple partners and has used IV drugs in the past month. Temp is 101 F. Pulse is 110. Has posterior cervical and axillary LAD. EBV and GAS negative. What test would best screen for suspected diagnosis in this patient?

HIV plasma RNA = early HIV infection = mono-like illness --> viral RNA is very high --> RNA testing best way to screen

meconium ileus are d/t to what gene mutation usually ...

Delta 508 / CF

6 mo old + RSV. Child drinks 6 ounces of formula every 3 to 4 hours + baby food. Mom took vitamin D and phosphorous supplements during her childhood and required surgical correction of leg abnormality. PE = frontal bossing + widening of wrists. X-rays = cupping of ribs and fraying of radius, ulna, femur, tibia, fibula. What abnormalities do you expect to see?

Elevated urinary phosphorous - familial hypophosphatemic rickets - most common non-nutritional form of rickets - X-linked dominant - PHEX mutation - proximal tubular reabsorption defect of phosphate conversion to vitamin D - urinary phosphate loss always increased

Syndrome with severe respiratory distress resulting from pulmonary hypoplasia, narrow dysplastic thorax, extremely short ribs.

Ellis-van Creveld syndrome - natal teeth - too few or too many teeth

5 mo old in ED after LOC + shaking of arms and legs for several seconds. Several other ED visits with similar episodes (occurs 2-3 hours after feedings). Has not met dev'tal milestones. PE = large, protruding abdomen. Glucose 35, serum pH 7.29, lactate 4.2 (elevated). Enzyme affected by kid's condition is found in what cellular compartment?

Endoplasmic reticulum - type 1 glycogen storage dz / von Gierke - hepatomegaly, renomegaly, hypertriglyceridemia, hyperuricemia, growth failure - glucose-6-phosphatase is on ER --> defect in this enzyme

A female neonate is delivered full-term and dies shortly after birth. She is diagnosed with a lethal form of osteogenesis imperfecta, an autosomal dominant condition. The unaffected father tells the physician he has a healthy daughter and a son from another mother who was affected with the same condition. What is the best explanation for this occurrence?

Gonadal mosaicism - Lethal forms of autosomal dominant conditions are typically due to spontaneous new mutations that occur in a particular gene at the time of conception. - Gonadal mosaicism is the most likely explanation for the family history described above; the father of this patient has gonadal mosaicism and carries the disease-causing mutation in some, but not all, of his germ cells.

A 16-year-old boy has been noted to have intermittent dark urine. He started playing high school football 3 months ago. He first noticed the urine color change after football practice last month. It cleared, but then he noticed it again a few weeks later. He denies any dysuria, urgency, frequency, discharge, and sexual activity. He was sick with a bad "cold" a few weeks ago. There is no known family medical history of kidney problems, but he was adopted and is not sure about his biological parents. On exam, he appears alert and quiet. His blood pressure is 132/86 mm Hg. Abdominal and back exams are unremarkable. The rest of the exam is normal. Urinalysis reveals clear colored urine with 2+ blood and trace protein. Microscopy of urine shows a few RBC casts. What is the most likely cause of this patient's gross hematuria?

Immunoglobulin A nephropathy - Acute illnesses or strenuous exercise occurring with hematuria in the adolescent population is most commonly due to immunoglobulin A nephropathy (IgAN), occurring mostly in older children and young adults. - Granular deposits of IgA and C3 in the glomerular mesangium and IgA immune complexes from circulation are seen. - IgAN is characterized by recurrent episodes of gross hematuria accompanied by upper respiratory tract infections or persistent asymptomatic microscopic hematuria with or without proteinuria. - Blood pressure may be normal or slightly elevated. - The most appropriate treatment is not known, but some agents used have included omega-3 polyunsaturated fatty acids, corticosteroids, and ACE inhibitors.

Infant with bronchopulmonary dysplasia. Chest wall compliance: Lung compliance: Airway resistance:

Increased chest wall compliance, decreased lung compliance, increased airway resistance - The total respiratory system compliance is the sum of chest wall compliance and lung compliance. - The total respiratory system compliance tends to be low in infants with BPD, returning towards normal by 1 to 3 years of age. - Their lungs also have increased air trapping and Functional Residual Capacity (FRC), which tends to normalize by 1 year of age. This reduces their lung compliance. - Premature infants tend to have increased chest wall compliance due to the soft and pliant rib cage, which easily moves in with the inspiratory pull of the diaphragm on the lower ribs. - Studies of Infant Pulmonary Function Tests (IPFTs) have shown increased airway resistance in general in these infants; although in this case, the presence of acute RSV infection may also further increase the airway resistance, leading to increased ventilation-perfusion mismatch and worsening respiratory failure.

A 4-year-old female patient presents with a history of bilateral otitis media with effusion (OME) that began 4 months ago after an episode of bilateral acute otitis media. The first occurrence of OME was treated with amoxicillin/clavulanate. Since then, she has had no fever, otalgia, or irritability, but she continues to be mildly inattentive to her mother's voice. Vital signs are unremarkable. On exam, the tympanic membranes are dull, blue, and retracted bilaterally, with decreased movement on pneumatic otoscopy on negative pressure and on positive pressure. Audiometry reveals a 30 dB hearing loss on the right and 35 dB on the left. What treatment should be recommended?

Insert bilateral tympanostomy tubes - Otitis media with effusion (OME, serous otitis media) often follows acute otitis media (AOM). OME lacks the acute features of AOM: fever, otalgia, irritability, vomiting, diarrhea. The tympanic membrane appears dull with a yellow or blue coloration; it is typically retracted or neutral in position, with decreased mobility on pneumatoscopy with positive and negative pressure. - Approximately two-thirds of cases of AOM are followed by OME, lasting an average of 3 weeks. - The only treatment for OME of demonstrated benefit is antibiotics. If OME persists beyond 12 weeks, it is considered chronic. - Audiometry should be performed at this point because most cases of OME are accompanied by an average hearing loss of 25 dB. If OME persists after a trial of antibiotics, insertion of a tympanostomy tube should be considered after 6 months if unilateral (not an absolute guideline) and after 3 months if bilateral.

7 yo girl with upward displacement of lens of her left eye (ectopia lentis) by pediatric ophthalmologist and with having mutation of FBN1 gene by geneticist. You have examined this girl numerous times in the past, but for the first time today you notice a systolic click, followed by a systolic murmur while examining the heart. Murmur becomes more prominent and noticeably longer in standing position. Most likely cause of "click murmur" in this pt?

Mitral valve prolapse - Marfan syndrome - associated with aortic root dilation and mitral valve prolapse

Recommended tx following close contact with a child with invasive meningococcal disease in a day care setting?

Oral rifampin 4 doses over 2 days - 10 mg/kg/dose for those >/= 1 mo - 5 mg/kg/dose for those <1 mo

Murmur of most common cardiac finding in rubella ...

PDA = continuous murmur, heard best at left infraclavicular area that radiates to left back

An 18-month-old girl presents for a routine checkup. While in the waiting room, she finds a brightly colored thumbtack and puts it in her mouth. Her mother quickly grabs her, startling the child and causing her to gasp, cough, and scream. Summoned immediately, the clinician encounters the mother attempting to extract the tack from her daughter's throat. The girl's cough is weak and ineffective, and she then stops crying. The clinician begins the Heimlich maneuver, which is unsuccessful. The girl becomes unresponsive. After securing her airway, a chest radiograph reveals that the foreign body is in the upper 1/3 of her esophagus. What is the most appropriate next step in the management of the patient?

Rigid esophagoscopy - Once the airway has been managed, early endoscopic removal of a sharp foreign body should be considered regardless of whether it is located in the esophagus or stomach. - Rigid esophagoscopy with a fiberoptic telescopic system and forceps extraction provides the advantage of removal under direct visualization. There is low risk of esophageal perforation or abnormalities, and injuries are easily identified. Intubation is required, which protects the airway.

Children who require cochlear implant are at increased risk for ...

S pneumoniae - those >/= 2 yo should also receive PCV23 at least 8 weeks after they have received all recommended doses of PCV13

Near term male born via vaginal delivery and experiences respiratory distress shortly after birth. Preductal oxygen sat is 90%; postductal sat is 80%. Undergoes endotracheal intubation and is given surfactant, showing some improvement in his respiratory status. He is started on dopamine infusion bc of hypotension and is subsequently started on inhaled nitric oxide. Echo reveals structurally normal heart with right-to-left shunting at both foramen ovale and PDA. What meds was his mom most likely taking throughout her pregnancy?

SSRI - PPHN of newborn - risk mostly in first trimester

12 month old F child brought to ED by parents with several-day hx of fevers, rash, diarrhea. For pas several months, she has had these recurrent symptoms that wax and wane for period of 3-7 days, especially after vaccinations. Previous meds have not been helpful. Has tender cervical lymphadenopathy, erythematous macular rash of neck and trunk, mild splenomegaly, and warm, tender knees and ankles. Labs: 14,000 WBC + normal differential, Hgb 12.8, platelets 175,000, ESR 60. CMP normal with BUN/Cr 10:1 and normal liver function panel. ANA titer <1:40 (negative). Anti-CCP Ab negative. UA and blood cx negative. Chest radiographs shows nothing. Plain radiographs of both ankles and knees negative. Most appropriate test to establish diagnosis?

Serum immunoglobulin levels - to differentiate hyperimmunoglobulin D from other periodic fever syndromes = AR = mutation in MVK gene (encodes mevalonate kinase) = excessive interleukin-1

A 13-year-old boy presents with right breast development over the last 5-6 months. He is on the swim team, so this has caused him much consternation. The breast swelling is slightly tender and without any drainage. He has been healthy, denies taking any medications, denies any substance use or trauma, and is doing well at school. On exam, height is 63" (160 cm) and weight is 115 lb (53 kg), which are both 75th percentile, BP is 94/68, pulse 68 beats/min. There is slightly tender 7-8 cm of right breast elevation and swelling extending from the areola that is non-erythematous. The left side is normal. There is no axillary lymphadenopathy. The testes are descended bilaterally and measure 3 cm in size. Pubic hair shows sparse growth of long downy hair at the base of the penis. There is no axillary or facial hair. He has scant acne. His mother is very anxious and would like further testing. What is the most appropriate initial evaluation to rule out any underlying pathology?

Serum testosterone, estradiol, FSH, and LH levels - Approximately 70% of pubertal boys develop at least breast buds during adolescence. Some will have more extensive growth. - The initial evaluation must include ordering lab work including serum testosterone (free and total), estradiol, FSH, and LH levels. This lab work will rule out primary hypogonadism such as Klinefelter's syndrome as a cause for gynecomastia, which is often the presenting symptom. - Physiologic gynecomastia is probably a result of low testosterone relative to estradiol levels during puberty and should resolve on its own in 6 months to 2 years. The incidence of breast enlargement peaks at age 13-15 and may be unilateral or bilateral. - Reassurance concerning the benign nature of the condition is usually all that is needed. - Gynecomastia that occurs at other ages or stages of sexual development or that evolves rapidly would warrant further investigation. Physiologic gynecomastia usually resolves by age 18 when adult androgen/estrogen ratios are achieved.

16 yo F + pallor, petachial rash of lower limbs, alteration of sensorium and sz's following brief flu-like illness (fever, fatigue, arthralgia). PE = BP 149/90 and hemiparesis of right side. Spleen palpable 2.5cm below costal margin. Blood = hgb 6gms%, TLC 12,000, plt 30,000. Peripheral smear = schistosis (fragmented RBC). Retic count elevated. Serum LDH increased. PT, aPTT, fibrinogen = normal. BUN and Serum Cr increased. UA = proteinuria and microscopic hematuria. Dx?

TTP - MAHA (schistocytes), thrombocytopenia, CNS inv't - renal inv't too - increased LDH = supportive

Clitoris is prominent with small and separate labia major what premature age infants?

between 30 and 35 weeks

Majority of aspirated foreign bodies in children are located where?

bronchi

The risk that the patient will experience another febrile seizure in the future is dependent on the age at the time of the initial simple febrile seizure. The recurrence rate in children under 1 year of age: In children over 1 year of age:

The recurrence rate is 50% in children under 1 year of age 30 - 35% in children over 1 year of age - The risk of developing epilepsy is approximately twice that of the general population (2% vs. 1%) if the child has no underlying neurological abnormalities and did not have a complex febrile seizure.

15 yo evaluated for polyuria and polydipsia for past 2 weeks. Denies fever, headache, abdominal pain, recent weight loss. She is sexually active with multiple partners. Has hx of bipolar disorder well controlled with lithium. Lab eval reveals Na 148. UA negative for glucose, nitrites, leuk esterase. Urine spec gravity 1.003. Most appropriate next step in eval?

Urine and serum osmolality - Nephrogenic DI via lithium via resistance to ADH within collecting ducts - Inability to concentrate urine --> hypernatremia, elevated serum osmolality (>300), low urine osmolality (<300)

A 3-month-old male infant is seen by his pediatrician for a well-baby check-up. His physician notes that he has had excessive growth since his birth. He is hypertonic and shows some motor delay. His cry is hoarse. He has a distinct facial appearance, including micrognathia, hypertelorism, and large, low-set ears. Karyotype analysis is normal. What is the most likely diagnosis?

Weaver syndrome - accelerated growth disorder of unknown etiology and is the most likely diagnosis - characterized by a distinct facial appearance, a low-pitched and hoarse cry, and hypertonia - also present are psychomotor delay, loose skin and umbilical, and inguinal hernias - excessive growth is seen in early infancy in all cases, and prenatally in most - characteristic facial features include micrognathia with a distinctive dimpled chin, hypertelorism, large and low-set ears, and telecanthus

A 5-day-old girl is seen for follow-up of hepatomegaly, which was noted at day 4 of life. She is the 3 kg product of an uncomplicated pregnancy, labor, and vaginal delivery; she was discharged from the newborn nursery at 36 hours. She has been bottle fed and was well until day 4, at which point she was evaluated for lethargy. At that time, hepatomegaly and jaundice were noted. She is monitored in the hospital; occasional hypoglycemia is present. On examination today, vital signs are normal. There are bilateral lens opacifications (determined by ophthalmology to be cataracts), mild hepatomegaly, icterus, and occasional lethargy, from which she was easily aroused. What specific diagnostic test will most likely reveal the cause of the infant's constellation of symptoms?

Urine-reducing substances measurement - Lethargy, cataracts, jaundice, hepatomegaly, hypoglycemia, and positive urine-reducing substances most strongly suggest galactosemia, the classic form of which is due to a deficiency of the enzyme galactose-1-phosphate - Ingestion of formula containing lactose, a disaccharide composed of glucose and galactose, will produce some or all of the findings described above in individuals with the enzyme deficit. - Newborn screening will usually detect the condition, but early discharge before a lactose load may render the screen negative. - The cornerstone of treatment is the elimination of lactose/galactose from the diet. - Untreated, symptoms will progress, leading to intellectual disabilities, sepsis, hepatic failure, and bleeding. - Cataracts may be seen as early as a few days after birth. Even early treatment does not eliminate the risk of developmental delay. Definitive diagnosis may be made by several methods, including red blood cell GALT levels.

What should be monitored in pts on methimazole?

WBC - can inhibit granulopoesis --> agranulocytosis - stop drug if leukopenia occurs

Sensitive indicator of active inflammation in Crohn disease and UC ...

calprotectin

Infants with congenital rubella are most severely affected if mother was infected when?

first 8-12 weeks of pregnancy

Thyroid function tests can be abnormal in

obesity non-thyroidal illness

Cyanotic heart lesion + left axis deviation ...

tricuspid atresia

TEE (transesophageal echo) usually more for

valvular pathology or endocarditis

Ibuprofen use within 24 hours can cause a false-positive results for what illicit drug?

PCP cannabinoids barbiturates benzos

18 hour old male born at 35 weeks gestation is noted to have abdominal distention and bilious vomiting. Plain abdominal radiograph reveals varying-sized loops of distended intestine, a relative absence of air-fluid levels, and a "soap bubble" appearance of portions of intestinal content. What is most likely associated with these findings?

Pancreatic insufficiency - via meconium ileus (80-90% have CF) = failure to pass meconium, abdominal distention, intestinal obstruction via impaction of thick, tenacious meconium in distal small bowel

recognize retinal hemorrhage

- if none = unlikely shaken baby has occurred

Hepatitis A vaccine is typically given at what age?

1 year of age, with a second dose 6 months after

Pediatric patients should be screened for substance abuse at routine health supervision visits starting at age

12 yo

Language of a 3 year old ...

200+ words speaks in 3 word sentences uses pronouns and plurals correctly names body parts 75% of words understood by others

At 11 mos old, children will have how many deciduous teeth?

4 - 7 + 4 = 11 - timing of primary eruption = 7+4 rule - at 7 mos, children should have their first teeth

Neonatal varicella occurs when mothers develop infection around the time of delivery (perinatal transmission). Maternal varicella infection during what timeframe confers the greatest risk of neonatal varicella?

5 days before through 2 days after delivery - Varicella-zoster immune globulin (VZIG) administration as postexposure prophylaxis to high-risk susceptible neonates prevents symptomatic infection in approximately 50% of cases; if VZIG is not available, administration of immune globulin intravenously is an alternative prophylactic treatment strategy to prevent neonatal disease.

PDA is an abnormality derived from which aortic arch?

6th

Teenage gymnast has signs and symptoms of spondylolysis. Initial studies for patients suspected to have this?

AP, lateral, and oblique radiographs of lumbar region - oblique --> posterior elements = "Scottie dog" (fracture of pars interarticularis) - at risk = gymnasts, ballet dancers, wrestlers experiencing chronic, progressive lower back pain - repetitive trauma from repetitive microtrauma from lumbar extension - L5 vertebra usually - injury sometimes complicated by L5 slipping onto S1 = spondylolisthesis

Although neonates and infants frequently are colonized by toxigenic c diff, they rarely develop symptomatic dz. Why?

Absence of intestinal receptors for c diff toxin A = low rate of infections

Drug of choice for treatment of dog, cat, or other mammal bites

Amoxicillin-clavulanate - For children with penicillin allergy, oral alternatives are extended-spectrum cephalosporin plus clindamycin or trimethoprim-sulfamethoxazole plus clindamycin

Which neonates require a brainstem auditory evoked response?

Any neonate who remains in the neonatal intensive care unit more than 5 days

bleeding disorder due to poorly functioning platelets

Bernard-Soulier syndrome

4 yo girl steps on dirty nail with cow feces on it. Her dog licks her foot after the injury. Her parents are rural migrant workers and, as far as they know, she has not received any immunizations. What should she receive today to prevent tetanus?

DTaP and tetanus immunoglobulin - dirty wound + <3 IZZ or IZZ hx unknown --> give tetanus IG + vaccine

A 35-year-old G3 P2 Caucasian woman has just undergone chorionic villi sampling (CVS) secondary to a prior pregnancy affected with trisomy 21. Her CVS results are normal, 46,XX. The pregnancy history is significant for hypothyroidism; it is well-controlled with 88 mcg of levothyroxine daily. She had hyperemesis, which resulted in a brief hospitalization for intravenous fluids in the 1st trimester. Why should this patient be offered maternal serum screening in the second trimester of this pregnancy?

Determine the risk for open fetal defects - CVS involves removal of placental tissue (villi) either transvaginally or transcervically (depending on anterior or posterior location of the placenta) at approximately 11 to 13 weeks gestation. - This test is considered diagnostic in the assessment of fetal aneuploidy via karyotype analysis. - Amniocentesis, also considered a diagnostic procedure, is performed at 15 to 22 weeks gestation and involves the removal of amniotic fluid containing fetal fibroblasts, as well as amniotic fluid alpha fetoprotein (AFAFP). Analysis of the levels of AFAFP can assess the risk for open fetal defects. - Because CVS specimens do not include amniotic fluid at this early gestational age, dorsal or ventral wall defect risk assessment cannot be performed. Therefore, it is recommended that women who have undergone a CVS procedure have maternal serum screening in the second trimester to assess for open fetal defects exclusively by measurement of maternal serum alpha-fetoprotein (MSAFP).

DRESS stands for

Drug Reaction with Eosinophilia and Systemic Symptoms - most often associated with antiseizure medications metabolized by CYP 450 and sulfonamides - 2-8 weeks after exposure to offending agent - facial edema and dusky, erythematous papules first

Longstanding unrestrictive left-to-right cardiac shunt can result in remodeling of pulmonary vasculature and pulmonary hypertension. When a longstanding left-to-right cardiac shunt causes pulmonary hypertension and results in right-to-left shunting with cyanosis, it is called

Eisenmenger syndrome. - Chronic cyanosis has adverse effects on most systems in the body

What confirms the diagnosis of cystic fibrosis

Elevated chloride levels on 2 occasions with adequate amounts of sweat

What is often seen with ASD and is the most specific of the above findings, since it is not part of the normal developmental spectrum?

Extreme aloofness

Adolescent male has primary ciliary dyskinesia (PCD), an autosomal recessive condition characterized by recurrent respiratory infections, male infertility, and situs inversus. What results in the male infertility associated with PCD?

Flagellar dyskinesis causes hypomotile sperm The best diagnostic approach for PCD uses a panel of tests including: - Genetic analysis - Nasal NO measurement - Structural examination of respiratory cilia via electron microscopy - Immunofluorescence for abnormal dynein arm proteins - Functional cilia waveform analysis where available

Typical timing of maximal growth velocity in average pubertal female?

Follows thelarche and precedes menarche, typically between ages 11-12

Strength training programs can reduce the risk of sports-related acute and overuse injury by 66%, and are effective at helping young athletes build strength and enhance athletic performance. What can be used by athletes of all sizes and allow for individualization of movement patterns, progression of motor control, and strength?

Free weights - eg, dumbbells, kettlebells, and medicine balls - preferred over weight machines for resistance training in young athletes - weight machines are generally sized for adults, cannot accommodate the smaller frames of many middle school athletes, and do not confer the same injury prevention and performance benefits as free weights

3 yo boy with hx of mild asthma is brought to your clinic for assessment of nighttime cough. For past 2 evenings he has awaken in middle of night with harsh-sounding barky cough that is unresponsive to cool mist or rescue inhaler. During day he is asymptomatic, and his appetite and activity remain unchanged. Mom denies fever, daytime cough, rhinorrhea, congestion but notes that occasionally his voice sounds harsh. Vitals stable. Lungs clear with good air entry bilaterally. Remainder of exam unremarkable. Most likely cause of child's cough?

Gastroesophageal reflux - spasmodic croup - noninfectious croup in which child wakes up in middle of night with barking cough and mild-to-moderate stridor --> following day, child is asymptomatic --> usually 2-3 night cycle

Pts with this present with primary amenorrhea and absence of breast dev't by 13 yo.

GnRH deficiency

17 yo girl presents with leg weakness and muscle cramping associated with tingling and difficulty walking. Symptoms started 2 days ago and are worsening progressively. She is afebrile, her HR is 58, and her BP fluctuates between 105/65 - 90/55. Fundoscopic and CN exams are normal. Describes tingling sensation in LEs that is more pronounced distally; weakness and absent reflexes also noted in LE's. After LP, CSF testing reveals protein level of 105, glucose 78, and WBC 2. Most likely diagnosis?

Guillain-Barre syndrome - affects peripheral nerves = demyelination and flaccid paralysis - autonomic dysfunction - CSF = elevated protein, normal glucose and WBC

Typically develops early in the course of ADPKD

HT

Clinical manifestations of 11-hydroxylase deficiency result from high adrenal production of mineralocorticoid 11-deoxycorticosterone and the androgen precursor DHEAS. Pts present with ...

HT hypokalemia androgen excess - decreased cortisol - high ACTH

Children who born at IUGR are at increased risk for developing ...

HT and metabolic syndrome as adults

change in behavior by study participants d/t awareness of being observed ...

Hawthorne effect

4 mo old + FTT + ht and weight below 1st %ile. Hepatomegaly + UA = elevated urinary pH + elevated urinary AAs and urinary phosphate. INR 2, serum glucose 47. Cause?

Hepatorenal tyrosinemia

Infant presents with resp distress, nausea, vomiting. CXR = posterolateral diaphragmatic hernia with left thoracic air-fluid level and mediastinal displacement. Cause of symptoms?

Hernia of Bochdalek = 95% of CDHs

16 yo presents with firm, painless mass in lateral portion of left thigh, which has continued to enlarge during previous 2 weeks. He is a member of his high school soccer team and is "always getting kicked" during practice and games. On x-ray, a round soft tissue mass with mature peripheral ossification and a more radiolucent center of immature osteoid is noted to be slightly separated from the femur. Most likely diagnosis?

Heterotopic bone / traumatic myositis ossificans - follows blunt soft tissue trauma - tx = rest, immobilization, NSAIDs

Neonate's clinical presentation of delayed passage of meconium and dilated bowel on abdominal radiography, in combination with a white forelock suggests the diagnosis of

Hirschsprung disease - Waardenburg type 4, or Waardenburg-Shah syndrome, is known to be associated with Hirschsprung disease - inherited in an autosomal dominant pattern - neonates may have a white forelock, wide nasal bridge, sensorineural deafness, heterochromia iridis, and lateral displacement of the inner canthi of the eyes - germline mutations in the PAX3 gene are responsible for WS

4 yo boy with hx of rash on hands presents for evaluation. Pruritic and sometimes complains of burning and stinging. Had similar rash 2x in past year. On PE, palms and lateral aspects of fingers have multiple, deep seated, inflamed vesicles, some of which have ruptured to form superficial crusts. There are also several tense bullae noted on both palms. This disorder is most often associated with ...

Hyperhidrosis - Dyshydrotic eczema = recurrent outbreaks of highly pruritic, sometimes painful, inflammatory vesicles and/or bullae - tx = topical steroids following application of wet compresses = symptomatic relief

10 day old infant with emesis + lethargic and appears dehydrated and weak. Has lost weight since birth. CV and abdominal exams are normal. No testes identified on GU exam. Sodium is 124. What other lab abnormalities is likely to be found on BMP?

Hyperkalemia - salt-wasting CAH - wt loss, dehydration, vomiting - hyponatremia, hyperkalemia, hypoglycemia, acidosis

Diabetes insipidus - Na: - Serum osmolality: - Urine osmolality:

Hypernatremia High serum osmolality Low urine osmolality

You are covering PICU when a 15 yo boy with T1DM is admitted with severe DKA after insulin omission for past 3 days. Following vigorous fluid resuscitation, he appears euvolemic with normal blood pressure but develops altered mental status. Best next step in management?

Hypertonic saline - cerebral edema --> hypertonic saline or manitol - typically within first 12 hours of treatment

Kiddo with CF + asymptomatic. Growth of pseudomonas on culture. Next step?

Inhaled abx tx for eradication of organism - decreases CF exacerbation if get rid of colonization even; even if asymptomatic

You are asked to assess the growth of school children between the ages of 6 - 11 years. What tissue or system shows maximum growth during this period?

Lymphoid - The growth of lymphoid tissue is very rapid between 6 to 11 years of age (middle childhood period), and the maximum growth is between 8 to 9 years of age. - During this period, there is hypertrophy of the lymphoid tissue. As a result, the tonsils and adenoids are enlarged, and lymph nodes may be normally palpable. - The lymphoid tissue of the Waldeyer ring (lymphoid tissue surrounding the oral and nasal cavities) is most active immunologically between the ages of 4 - 10 years. - The size of the lymphoid tissue starts decreasing after puberty, and it reaches the normal adult size at 18 - 20 years of age.

causes of anion gap metabolic acidosis

Methanol Uremia DKA Paraldehyde Isoniazide or Iron Lactic acidosis Ethylene glycol Salicylic acid

3 day old term infant + bilious emesis and abdominal distention. Uncomfortable, lethargic, tachypneic. Distended upper abdomen. Duskiness of skin over abdomen. Absent bowel sounds. Poor perfusion. Blood in diaper. Labs = metabolic acidosis. X-ray = distension of stomach and proximal small bowel with paucity of air in colon. Surgery = necrotic intestine. Dx?

Midgut volvulus = most common obstruction in neonatal period - failure of cecum to move to RLQ + failure to adhere to posterior abdominal wall --> twisting of mesentery around it

16 yo F + dx with T1D + started on regular insulin and NPH + returns for f/u in 1 week + 3am glucose in the 50s + correct dx and management of pt?

NPH should be replaced with long-acting insulin analog - has nocturnal hypoglycemia 2/2 taking too much NPH insulin in the evening

Pt has classic triad of migratory arthritis, tenosynovitis, dermatitis, 1 of 2 forms of disseminated gonococcal infection. This form presents within 2-3 weeks of primary GU infection, often during menstruation. Migratory polyarthralgia can simultaneously involve small or large joints. The definitive diagnosis of disseminated gonococcal infection is made by identification of ...

Neisseria gonorrhea (molecular testing and/or culture) on specimen of blood, endocervix, synovial fluid, or skin lesion - Among these sites, endocervical swab for gonococcal NAAT has highest sensitivity for identifying this organism - specialized media (Thayer-Martin) required

3 yo + 2 weeks of limping, constipation, decreased appetite, fever. Vitals nml. PE = pallor, decreased active and passive movement of right hip, and mass in left flank. Labs = anemia of 7.4 hgb, ESR 114. Skeletal survey = punched out lesions in bones of pelvis and femur. Cause of symptoms?

Neuroblastoma - abdominal pass, constipation, proptosis, periorbital ecchymosis, horner syndrome, weakness (spinal cord compression), palpable non-tender subQ nodules, opsoclonus-myoclonus atxia syndrome, systemic symptoms

17 yo M + severe right testicular pain and nausea for last 5 hours. PE = markedly swollen and tender right testis that is positioned horizontally. Right cremasteric reflex is absent. Most appropriate management?

Operative orchidopexy - testicular torsion

16 yo boy who plays on high school baseball and soccer teams. Presents with 3 mo hx of gradually worsening right knee pain. Plain radiography shows zone of radiolucency in lateral portion of medial femoral condyle that surrounds well-demarcated, thin, linear fragment of partially attached subchondral bone. Most likely diagnosis of this pt?

Osteochondritis dissecans - ROM and strengthening exercises --> beneficial to cartilage healing

2 yo boy brought to ED bc grandparents heard him coughing and discovered him attempting to drink from small container containing kerosene. Upon arrival, he is scared but behaving normally. He has odor of petroleum distillate. His RR is 28, and his vitals are normal. In additional to supportive care with oxygen and bronchodilators, what best describes the most appropriate care for this pt?

Perform CXR 6 hours from ingestion - low-viscosity, high-volatility petroleum distillate hydrocarbon product --> pulmonary aspiration = inflammation of bronchopulmonary tree - CXR may be initially normal but show changes later - asymptomatic pts can be watched for 6 hours following ingestion

Probability of disease before a diagnostic test is ordered

Pretest probability

An anxious-looking young mother brings a 6-week-old female infant to your office wrapped in many blankets. When your nurse unwraps her, she appears lethargic; she has dusky and mottled skin and very weak pulses. As your staff quickly provides ventilation and compressions, you prepare to establish vascular access. What is the best site for immediate vascular access for this infant?

Proximal anteromedial tibia - Insertion of an intraosseous needle directly into the anterior tibia is relatively easy and will ensure rapid vascular access.

Findings on hand with bulimia ...

Russel sign

Indicates the onset of puberty in males

Testicular enlargement of 4 mL in volume - For boys, peak height velocity occurs during sexual maturity rating genital 3 to 4

Workup for solid testicular mass ...

beta-hCG alpha-fetoprotein LDH testicular US or MRI CT scan of chest and abdomen

Lab eval of homocystinuria reveals ...

elevated methionine in plasma and CSF

Pt experiencing symptoms of celiac disease. Has positive tTGA. Next step?

endoscopic small bowel biopsies - IgA-tTGA = first-line screen for suspected celiac disease --> small biopsy confirms diagnosis

K, Ca, P in tumor lysis syndrome...

high K high P low Ca

Most widely used initial tx of jellyfish sting

hot water

Hepatitis B core antibodies develop only in the presence of

infection

Female premutation carriers of fragile X have increased risk for

premature ovarian failure mood and anxiety difficulties abnormalities on MRI

Patients on insulin pumps can lower basal rates by how much during exercise?

∼10-50% or more or suspend for 1-2 h during exercise - Decreasing basal rates or long-acting insulin doses by ∼20% after exercise may reduce delayed exercise-induced hypoglycemia

Recogniz Hirschsprung

- Barium enema shows a segment of narrowed bowel - May occur in isolation but may also be associated with syndromes such as trisomy 21, Joubert syndrome, Goldberg Shprintzen syndrome, and Smith-Lemli-Opitz syndrome - Occurs because of an arrest in migration of ganglionic cells to the rectosigmoid region. Without adequate innervation, the colonic muscles do not relax properly. - Affected neonates typically exhibit failure to pass meconium with or without abdominal distention. - Abdominal radiography may show mild bowel dilation. - Typically, digital rectal examination will result in a large foul-smelling bowel movement. - Toxic megacolon, a rare complication of Hirschsprung disease, is caused by increased luminal pressure, which compromises perfusion of the bowel wall causing bacterial translocation. On physical examination, affected neonates have abdominal tenderness, distention, and erythema.

How long to apply steroids for mild eczema

- Low potency topical corticosteroid (Class VII) twice daily for up to 3 days beyondclearance -For mod-to-severe disease:Medium potency topical steroids (Class III-IV) twice daily for up to 3 days beyondclearance - Also, consider possible secondary infection that may need oral abx - If flare not cleared up in 7 days, consider nonadherence, infection, misdiagnosis, referral - For relapsing course, consider maintenance topical calcineurin inhibitors (pimecroliumsor tacrolimus, 2-3x weekly) and/or maintenance topical corticosteroid (1-2x weekly if med strength, 1-2x daily if low strength)

Management of children with GSD V (McArdle's disease) includes:

- Obtaining a baseline creatine kinase level - Physical examination with assessment of muscle strength/weakness - Simple healthy lifestyle interventions: A diet rich in complex carbohydrates; Maintenance of good hydration - Regarding exercise: Avoid handgrip, weightlifting, dynamic (eg, ball games), lengthening muscle contractions (eg, jumps), intense dynamic aerobic (eg, running, strenuous swimming); Encourage: daily moderate-intensity aerobic training (eg, walking, bicycling) to increase cardiorespiratory fitness; glucose-containing fluids prior to exercise to avoid rhabdomyolysis. - Avoidance of general anesthetics—muscle damage may occur with the use of muscle relaxants or inhaled anesthetics

Clearance for return to participation in contact sports after concussion requires

- full symptom resolution - no symptom recurrence with high-intensity physical activity - return to baseline neurocognitive function and academic performance

Boys on average stop growing in height by what age?

17 years

Growth hormone deficiency usually presents when?

18 - 24 mos

Dev'tal milestones age: hold doll in hand + makes tower of 4 blocks + turns pages of storybook + scribbles with crayon + feeds herself using spoon + removes socks + puts hat on her head.

18 mos

Makes tower of three cubes by what age?

18 mos

Correct management with infant experiencing symptomatic hypoglycemia ...

2 mg/kg of 10% dextrose fluids

Posterior fontanelle and metopic suture are closed in most infants by ...

2 mos

Cyclic vomiting syndrome, a migraine variant, is a functional gastrointestinal disorder defined as

2 or more episodes of intense nausea and vomiting that lasts hours to days over a 6-month period, separated by a return to baseline health between episodes Treatment for CVS includes management of the acute episode (abortive strategies) and prophylactic therapy. - Abortive strategies include supportive care (eg, quiet, dark environment, maintaining hydration), and, if necessary, antiemetic therapies or triptans. - Prophylactic therapies include stress management, avoidance of fasting, and elimination of trigger foods (if relevant). - Medical management is indicated if episodes result in multiple hospitalizations or school absences, or are occurring more than every 1 to 2 months. - First-line therapy for children older than 5 years of age is amitriptyline; cyproheptadine is recommended for children 5 years of age and younger. - Prophylaxis with propranolol is recommended as second-line therapy for all ages. - Complementary therapies, including acupuncture or cognitive behavioral therapy, may be considered.

4 yo Caribbean child + enlarged clitoris and female external genitalia. Hyperpigmentation, diaphoresis, dilated pupils. Blood glucose 40, serum sodium 132, K 6. Blood gas with pH of 7.2, HCO3 16, PaCO2 32. Additional testing likely to reveal deficiency of ...

21-hydroxylase - most common cause of CAH

A boy runs well, walks up and down stairs one step at a time, builds a tower of seven cubes, puts three words together, and handles a spoon well. He cannot go up stairs using alternating feet, build a tower of ten cubes, or hop on one foot. What is the age of the child?

24 months - average 24-month-old can run well, walk up and down stairs one step at a time, build a tower of seven cubes, put three words together, and use a spoon.

Jump in place at what age ...

24 mos

How long to correct for age for in premature infant ...

24-30 mos after birth

Pinna is soft and remains fold at what age premature infants?

24-31 weeks - thin cartilage, pinna springs back from folding between 36 and 39 weeks - pinna firm and remains erect from head at 40 weeks

Exclusively breastfed infants often stool only once every

3 to 4 days

How many words should 13 mo old have ...

3 words

How long does it take a child to double their birth length?

3 years

It is important to measure head circumference at each health supervision visit through age

3 years

15 yo F in ED + at rave part before + took pill to feel "closer" to friends + pill made her sweaty and caused blurry vision, nausea, teeth grinding. Drug?

3, 4 - MDMA - Molly/ecstasy - entactogens

Suggested intervals between administration of immunoglobulin preparations and vaccinations containing live virus range in what time intervals?

3-11 mos

Menarche usually occurs at what Tanner staging for females?

3-5

moro reflex disappears by what age?

3-6 mos - so does the palmar grasp reflex

Visual acuity reaches adult level of 20/20 by what age?

5-7 yo

Approximate potential energy from dietary fat

9 kcal/g = highest density source of potential energy - carbs and protein = 4

Age? - can get into sitting position - sit without support - crawl - pull to stand - pick up objects with 3-finger pincer grasp - bang blocks together - make 2-syllable sounds - imitate speech sounds - say "mama and dada"

9 mos

What percentage of adolescents who commit suicide suffered from known psychiatric disorder at the time of their death ...

90%

SJS percentage

<10% epidermal attachent

Patients with what CD4 percentage can get live vaccines regarding HIV?

>/= 15% for at least 6 mos

No vernix in what gestational age infants?

>/= 42 weeks

Positive TB skin test for any child over 4 years old is ...

>15mm

Chronic pancreatitis (CP) is diagnosed with imaging consistent with CP (eg, pancreatic calcifications, pancreatic duct strictures and/or dilations) AND at least 1 of following criteria:

Abdominal pain consistent with pancreatitis Evidence of exocrine pancreatic insufficiency Evidence of endocrine pancreatic insufficiency - Evidence of exocrine pancreatic insufficiency includes low fecal pancreatic elastase level and fat-soluble vitamin (vitamin D) deficiency

Male born at 35 weeks of gestation + admitted to NICU for emergency c-section for placental abruption. Noted to have marked shortening of both upper extremities. Plain radiographs show bilateral absence of radii with presence of complete thumbs and hypoplasia of distal humeri and shoulder girdles. What is most likely to be identified during further eval of the pt?

Absence of megakaryocytes in bone marrow - thromboctyopenia-absent radius syndrome = absence of hypoplasia of megakaryocytes in bone marrow and associated thrombocytopenia - AR - severe thrombocytopenia + bilateral absent radii - do NOT have abnormalities of thumb - can have ASD or tet of fallot

12 day old male born via home birth at full-term gestation with no previous medical care is brought to ED bc of decreased feeding, lethargy, vomiting. On PE, he is tachycardic with hypotension. Lab eval shows: Na 125, K 6.2, bicarb 14, glucose 42. Adrenal crisis d/t previously undiagnosed adrenal insufficiency suspected, and pt is given bolus of IV crystalloid with dextrose. Best next step in management of this pt?

Administer IV hydrocortisone = hydrocortisone IV bolus at 100 mg/m^2 = adrenal crisis - initial management = IV fluids, dextrose (if hypoglycemic), stress dosing of hydrocortisone (50-100 mg/m^2)

A 30-year-old mother with full-term pregnancy developed chickenpox 3 days before delivery. She delivered a term female infant weighing 3.2 kg with a good cry. Systemic examination is normal, and neonatal reflexes are intact. What is the appropriate step for prevention of varicella infection in this infant?

Administer varicella zoster immune globulin.

6 yo M + behavioral probz/aggression began 1 year ago. Progressive cognitive deterioration, spasticity, vision loss. Elevated very long chain FAs. MR = white matter T2 hyperintensities (symmetric, involve posterior regions sparing U fibers). Dx?

Adrenoleukodystrophy - X-linked recessive - deficient peroxisomal enzyme acyl-coenzyme A synthetase --> accumulation of VLCFAs - 4-8 yo

RSV is the most common cause of bronchiolitis in young children. What is a serious complication associated with RSV dz?

Apnea

Used to distinguish fetal hemoglobin from adult hemoglobin in neonates with rectal bleeding ...

Apt test

Form of bacterial strep throat resistance to penicillins ...

Arcanobacterium haemolyticum

Infant born at term with ambiguous genitalia (labial fusion and clitoromegaly). During pregnancy, mom developed hirsutism, severe acne, deepening voice. US of infant reveals normal uterus. Karyotype = 46, XX. Cause of condition?

Aromatase deficiency - rare - AR - lack of conversion of androgens to estrogens - excess androgens from placenta - require early estrogen placement

8 yo previously health girl presents to ED for lethargy. She has had mild cold symptoms for a week without fevers and was difficult to wake this morning. On PE, temp is 102 F, RR 30, HR 138, BP 80/40, and O2 sat 95% on RA. She is minimally responsive to pain, pupils are 4 mm and equally reactive to light. Breath sounds are clear. She has has soft systolic murmur across her precordium, 1+ pulses distally and cap refill 4 seconds. Abdomen soft. You initiate tx with fluids and she develops tachypnea. On repeat exam, she has bibasilar rales and her liver is now palpable below costal margin. Her mental status, HR, and BP remain the same. Best next step in management?

Initiate epi drip - cardiogenic shock - early heart failure

An 11-year-old boy presents for a checkup and immunizations. He has been healthy and does not take any medications. Grandparents on both sides have high blood pressure and diabetes. His exam is normal, and he is at the 75th percentile for height and the 95th percentile for weight. BP is 122/76, putting his systolic reading just above the 90th percentile for his height. His BP is remeasured at this visit three times by manual auscultation, and the average reading still places his systolic reading above the 90th percentile. What is the most appropriate next step in management?

Manage weight, diet, and activity and return in 6 months. - Pediatric hypertension is the maintained elevation of systolic or diastolic BP at or above the 95th percentile for age, gender, and height. Repeated measurements by manual auscultation should be done to confirm the presence of a sustained elevated reading. - Prehypertension occurs when the systolic or diastolic BP is 90-95th percentile (or between 120/80 and the 95th percentile). - Counseling on weight management with dietary recommendations and activity is indicated; he should return in 6 months for a recheck.

must be considered in cases of chronic lymphadenopathy that are unresponsive to appropriate antibiotic therapy especially when there are risk factors for tuberculosis

Mycobacterium tuberculosis - Nontuberculous mycobacteria are ubiquitous in a wide range of environmental sources (such as water, food, and soil), and the disease is acquired via exposure to environmental sources. - Young children ranging from 1 to 5 years of age are most frequently affected. - Most infections caused by NTM occur in previously healthy children. - Infection with NTM can occur opportunistically in individuals with compromised cell-mediated immunity (eg, receipt of immunosuppressive therapy, acquired immunodeficiency syndrome, genetic defects in interferon-γ receptors or interleukin12). - Pulmonary disease caused by NTM can complicate bronchiectasis in patients with cystic fibrosis.

A 3-month-old male infant is brought to the emergency department because of fever, lethargy, and a rapidly progressive rash over a period of several hours. The previous day, he was in his usual state of health and activity level. He was drinking 6 oz of formula every 3 hours and making several wet and dirty diapers per day. He did not have fevers until the day of admission. He attends day care. There were no reported complications during pregnancy, and he received his 2-month immunizations. On arrival to the emergency department, he had a temperature of 39.5°C, heart rate of 190 beats/min, respiratory rate of 70 breaths/min, blood pressure of 70/20 mm Hg, and oxygen saturation of 100% on room air by pulse oximetry. He appeared toxic and lethargic. His extremities were warm and flushed with flash capillary refill. His abdomen was soft, nontender, and with no organomegaly. Over the next 30 minutes, intravenous access was obtained and 80 mL/kg of normal saline was administered. Blood cultures were obtained and antibiotics were given. He now has a temperature of 39.0°C, heart rate of 180 beats/min, respiratory rate of 80 breaths/min, and blood pressure of 80/25 mm Hg. His oxygen saturation by pulse oximetry is 93% on room air. He remains lethargic, and his extremities are still warm with flash capillary refill. He has bilateral rales on lung auscultation, and his liver is palpable 2 cm below the costal margin. Central intravenous access is obtained. Of the following, the MOST appropriate intervention to support this patient's hemodynamics is to start an infusion of

NE - The previously healthy infant rapidly developed fever, lethargy, and hypotension consistent with a diagnosis of septic shock. - Despite receiving 80 mL/kg of fluid resuscitation, his shock has not improved. - The features of warm extremities and flash capillary refill further characterize the condition as "warm shock". - Of the response choices, the best next step in management is to start an infusion of norepinephrine. - Boluses of isotonic fluid up to and over 60 mL/kg are given rapidly until signs of shock are reversed or until rales or hepatomegaly develop. In forms of shock refractory to fluid or in which more fluid would worsen the condition, vasopressors or inotropes should be started and titrated to improvements in perfusion and/or blood pressure. - "Warm shock," as described for the infant in the vignette, often responds to medications causing vasoconstriction, such as norepinephrine, vasopressin, or high-dose epinephrine or dopamine. - If shock is refractory to inotropes or if the patient is at risk for adrenal insufficiency, hydrocortisone should be started. This approach can be effective for cardiogenic shock as well. - If perfusion worsens or if rales or hepatomegaly occur during the early phase of fluid resuscitation, as can occur during cardiogenic shock, fluid boluses should be stopped, and an inotropic infusion should be started.

19 month old, previously well boy + refuses to bear weight on his right leg. Deny trauma, associated symptoms, or hx of fractures. Radiograph of right leg reveals anterior tibial bowing and fracture in middle third of tibia. What condition is known to be associated with likely cause of these radiographic findings?

NF1 - congenital pseudoarthrosis = false joint ~ unilateral anterolateral angulation/bowing of tibia progressing to thinning of cortex and pathological fracture

A previously healthy, 6-month-old, male infant is admitted to the pediatric intensive care unit for respiratory failure secondary to respiratory syncytial virus. He is intubated and supported with a ventilator. Of the following, the BEST modality to support this infant's nutrition is

NJ feeding - Providing nutrition through the stomach or jejunum is preferred over intravenous nutrition because of a lower risk of adverse reactions and better cost-effectiveness - Short-term nutritional support (1 to 3 months) should be provided with nasogastric or nasojejunal feedings; chronic nutritional support can be accomplished with a gastrostomy or jejunostomy tube. - Children at high risk of aspiration benefit from jejunal feedings.

13 yo was jus diagnosed with JIA. She has non-disabling systemic symptoms and has not had fevers. Global assessment score is 4. Has 2 active joints. No evidence of macrophage activation syndrome. Most appropriate initial therapy?

NSAIDs - mild to moderate acute dz

What meds decrease the clearance of lithium ...

NSAIDs, like ibuprofen

A 7-day-old female neonate was born to a 16-year-old single mother. The neonate presents with a 2-day history of bilateral conjunctival edema, hyperemia, and watery-to-mucopurulent discharge. Otherwise, she has been eating fairly well, and there has been no fever, vomiting, diarrhea, or respiratory symptoms. The mother had sporadic prenatal care, and the neonate was born by normal spontaneous vaginal delivery. The neonate received routine topical prophylaxis with erythromycin. Vital signs and exam appear unremarkable, with the exception of both eyes. What is the most likely diagnosis?

Neonatal chlamydial conjunctivitis - most frequently identified cause of infectious neonatal conjunctivitis is Chlamydia trachomatis, transmitted perinatally from infected mothers (especially adolescents) - Conjunctival edema, hyperemia, and watery-to-mucopurulent discharge develop 5-14 days after birth and can last over 2 weeks. - With prolonged infection, a pseudomembrane and bloody discharge may develop. - Treatment must be with oral erythromycin 50 mg/kg/day for 14 days; topical therapy is ineffective.

Child returns to your office when he is 5 yo. When he was 2 yo, mom was worried bc child's left foot appeared to be rotated internally when child walked. At that time, you diagnosed internal tibial torsion and reassured mom that this is a normal pattern of bone growth and that it will resolve over time. Mom now complains that same problem has recurred, but both legs seem affected - both feet turn in while walking. When child is standing, you notice that each patella points inward along with feet turning in. With child in prone position, you are able to achieve 90 degree internal rotation of each femur but less tan 45 degree external rotation of each femur. You notice the patient prefers to "W" sit. What do you recommend to the mother?

No intervention - problem will resolve itself - most common cause of in-toeing in child older than 2 yo = internal femoral torsion = femoral anteversion --> prone position: increased internal rotation of hip, greater than 45 degrees; external rotation generally <45 degrees - generalized ligamentous laxity may be present

What disorders may be worsened during acute phase of infections cause by group strep?

OCD and/or tic disorders

Oubreak of salmonella. Contaminated chicken = source. Two children who didn't eat chicken developed disease. 12 children who ate chicken developed disease. Of 50 children in school, 25 ate chicken, and 25 brought lunch from home. What is the odds ratio for developing salmonella d/t exposure to contaminated chicken?

OR = (12 x 23) / (2 x 13) Odds ratio = odds that individual with specific condition has been exposed to risk factor by odds that control has been exposed - odds of dz in exposed group = 12/13 - disease in exposed group / health in exposed group - odds of dz in non-exposed group = 2/23 - odds ratio = (12/13) / (2/23) = (12x23)/(2x13)

A 16-year-old girl presents with a 2-week history of numbness and tingling around the mouth and at the fingertips; she has never had similar symptoms in the past. She is taking no medications and reports no change in her diet or activity pattern. There is no history of tobacco, alcohol, or illicit drug use. Past medical history and family history are unremarkable. Growth and development have been normal and immunizations are current. Vital signs are normal. Tapping on the inferior zygomatic arch anterior to the ear produces repetitive twitching of the corner of the mouth (Chvostek sign), and inflating a blood pressure cuff to 20 mm Hg above systolic pressure produces a sustained spasm of the wrist (Trousseau sign). Laboratory evaluation reveals a total serum calcium of 8.1 mg/dL, with a normal serum albumin. What is the next step in evaluating this patient's hypocalcemia?

Obtain a PTH level - Assaying the parathyroid hormone (PTH) level is central to evaluating hypocalcemia. - In hypocalcemia, if the PTH level is elevated, a cause of hypocalcemia other than hypoparathyroidism exists (e.g., decreased intake, decreased absorption, increased excretion including renal insufficiency, decreased 1,25-dihydroxy vitamin D caused by metabolism defects, renal disease, dietary deficiency, and lack of sunshine). - If the PTH level is not elevated in hypocalcemia, hypoparathyroidism is likely. - Note that hypomagnesemia can suppress PTH secretion even in the presence of hypocalcemia.

Location of headache that warrants careful consideration of the need for neuroimaging because although pain in this location may occur with migraine with brainstem aura, formerly called basilar-type migraine and may also may be seen with posterior fossa neoplasms and Chiari malformations.

Occipital pain

Persistent umbilical drainage is the most common presentation for urachal anomalies such as umbilical polyps, urachal cysts, and patent urachal sinuses. What is the preferred imaging modality for these anomalies?

US - Because of the risk of urachal adenocarcinoma, children who have a urachal anomaly as well as other symptoms such as infection, pain, or drainage of fecal matter should undergo surgical excision. - Children who are otherwise asymptomatic can be monitored closely.

Septic arthritis in young child. Will not bear weight and holds rigidly with pain. Most likely due to bacterial infection. Imaging?

US - need to make dx as soon as possible

After returning from spending the weekend with her dad, an 11 yo female is brought by her mom to the ED after she noticed grayish-white stain on her underpants. Pt denies any abuse, but the mother remains concerned bc her former husband has a hx of drug use and "seems to have people in and out of his house and all hours of the night." During PE, wet prep of vaginal secretions is obtained, which reveals numerous lactobacilli and few polymorphonuclear leukocytes. What findings is most likely to be present during additional evaluation of pt?

Vaginal pH <4 - 4.5 - physiologic leukorrhea - normal increase in vaginal secretions ~ 6-12 mos prior to menses

Otherwise healthy, 17 yo girl, present with 10 day hx of vaginal discharge, which is confirmed on speculum exam. Findings on microscopic exam of saline wet prep include majority epithelial cells that appear stippled with adherent bacteria. Adding potassium hydroxide to vaginal fluid produces fishy odor. What is most likely associated with clinical and microscopic findings?

Vaginal pH > 4.5 - bacterial vaginosis - tx = metronidazole - 500mg 2x daily for 7 days - alternative = 0.75% vaginal metronidazole (5g administered intravaginally once daily for 5 days

A full-term male was admitted to the neonatal intensive care nursery for unilateral cleft lip identified at delivery. A brain MRI was requested and found to be normal. On physical exam, the newborn has a unilateral left-sided cleft lip with no other unusual dysmorphology. The mother reports that she was born with a unilateral cleft lip, which was surgically repaired in infancy, but no cleft palate. She is otherwise non-dysmorphic and is cognitively normal. Her mother and her maternal grandmother were also born with isolated unilateral cleft lip. The patient's mother adds that she has always had these funny bumps on her lower lip that her mother and maternal grandmother also have. What condition is the most likely explanation for this phenotypic combination of features?

Van der Woude syndrome - causes facial clefting and lower lip pits or mounds - typically cognitively normal - can show congenital lower-lip pits or fistulas or small mounds with a sinus tract leading from a mucous gland of the lip - also associated with cleft lip, cleft palate, or cleft lip with or without cleft palate and follows autosomal dominant inheritance, as seen in this family

During initial eval of 4-lb, 1-oz female born at 36 weeks of gestation, she is noted to have HC of <3%ile. Additional abnormalities include bilateral cataracts, multiple areas of scarring (some of which appear to be following along a dermatome), and shortened hypoplastic left leg. Most likely cause of these clinical findings?

Variella virus - congenital varicella vaccine - zig-zag like scarring = cicatrix --> may follow a dermatome - hypoplastic and malformed extremities - microcephaly, microphthalmia, cataracts chorioretinitis

7 mo old + FTT, persistent diarrhea, thrush. Hx of recurrent viral, bacterial, fungal, protozoal infections. Maternal uncle who had similar symptoms to pt and died at young age. Inheritance pattern?

X-linked recessive defective IL-2R gamma chain or AR adenosine deaminase deg - SCID

Clinical presentation of the boy with acute onset of gastroenteritis after recent consumption of pork intestines (called "chitterlings" or "chitlins") is suggestive of

Yersinia enterocolitica gastroenteritis - Transmission of Y enterocolitica occurs mainly through the fecal-oral route, often via ingestion of contaminated meat products (especially pork), inadequately pasteurized milk products, contaminated water, and contaminated vegetables - Person-to-person spread of Yersinia enteritis has occurred from handling raw pork intestines (ie, chitterlings) without good hand hygiene during traditional winter holiday celebrations - Other less frequent modes of transmission include direct or indirect contact with animals (eg, pigs, dogs, cats, rats) - Yersinia enteritis may be complicated by bacteremia, especially in infants, older children with excessive iron storage (eg, deferoxamine therapy, sickle cell disease, β thalassemia), and immunocompromised hosts - Antimicrobial therapy is recommended for all immunocompromised patients, neonates and infants, and all patients with invasive Yersinia infection

Boy has evidence of chronic kidney disease (CKD) secondary to renal dysplasia. His history and findings of polydipsia, nocturnal enuresis, an inability to concentrate urine, growth failure, uremia, anemia, and acidosis are consistent with a diagnosis of CKD. He is also at risk for metabolic bone disease (MBD) characterized by

a high parathyroid hormone level - A high serum phosphorus level, low serum calcium level, and secondary increase in parathyroid hormone level are seen in metabolic bone disease caused by chronic kidney disease Metabolic bone disease - previously known as renal osteodystrophy - common complication of CKD and can result in growth failure and skeletal deformities - inability to excrete phosphorus and synthesize active 1,25-dihydroxyvitamin D because of decreased kidney function leads to CKD-MBD - low 1,25-hydroxyvitamin D level causes impaired absorption of calcium from the intestine, resulting in a relatively low serum calcium level - parathyroid glands are then stimulated by the relative hypocalcemia, causing secondary hyperparathyroidism - in the early stages of CKD, calcium and phosphorus levels may remain normal, but the parathyroid hormone level increases as a compensatory mechanism to maintain a normal serum calcium level - as CKD advances, there is a high serum phosphorus level, low serum calcium level, low 1,25-dihydroxyvitamin D level, and a secondary increase in parathyroid hormone level - serum alkaline phosphatase level, a marker of bone turnover, is elevated

In a child with ADHD on a stimulant, if weight declines by more than 25%, what should be considered?

a stimulant holiday

17 yo F admitted to PICU with HAs, vision loss, HA. Developed fever and HA over 12 hours ago prior to admission. 2 wks ago had febrile illness with sore throat, and myalgias that resolved in 7 days. PE = confuse female with mild erythema of posterior pharynx, weak UE and LEs. MR brain = 16 hyperintense lesions throughout cortex an subcortex of brain (asymmetric). No midline shirt or mass effect. Most likely cause?

acute disseminated encephalomyelitis (ADEM) - often follows viral or bacterial infection

Boy has acute kidney injury (AKI) as supported by a decrease in urine output after an episode of diarrhea and vomiting, and elevated blood urea nitrogen and creatinine levels. Presence of minimal blood and protein on urinalysis and detection of granular casts. He most likely has

acute tubular necrosis (ATN) - secondary to hypovolemic shock (tachycardia, hypotension, and prolonged capillary refill) - most common cause of renal AKI in hospitalized children - results from damage to renal tubular cells by ischemia (eg, shock, trauma, sepsis, burns), exogenous toxins (eg, nephrotoxic medications, contrast agents), or endogenous toxins (eg, myoglobinuria, hemoglobinuria) - urinalysis shows epithelial or muddy-brown granular casts, mild pyuria, and low-grade proteinuria - urine may be dilute, with specific gravity below 1.010, urine sodium level greater than 40 mEq/L, and fractional excretion of sodium (FENa) greater than 2%

Kiddo has fatty acid oxidation disorder. MCAD deficiency is the most common FAOD with hypoglycemia after period of fasting or stress. Absence of both urinary-reducing substances and ketones in a child with hepatosplenomegaly suggests defect in fatty acid metabolism. What aids in diagnosis of FAOD?

acylcarnitine profile - associated with elevations of C6, C8, C10, C10:1 carnitine esters

What agents used for routine decontamination of hands in health care settings is most bactericidal and least irritating to the skin ...

alcohol-based hand rub

To establish dx of rheumatic fever, obtain what to link symptoms together?

antistreptolysin O Ab titer

Pyloric stenosis more common among those who:

are Caucasian are preterm offspring of parent with pyloric stenosis

A 16-year-old adolescent boy is seen for a health supervision visit accompanied by his mother. During the visit, he shares that he has developed significant anxiety, contributing to a decline in school performance and self-esteem. His mother has encouraged him to attend cognitive behavioral therapy sessions, but he has been reluctant to go because he does not feel comfortable "talking with a stranger." When interviewed without his mother present, he shares that his anxiety has affected his friendships, and he cannot sleep because he spends hours at night worrying about different things. He reports no substance abuse, or suicidal or homicidal ideation. Of the following, the BEST next step in this child's care is to

ask him about his concerns regarding cognitive-behavioral therapy - Starting at age 7 years, most children are able to participate in a developmentally appropriate manner in shared decision-making. This will evolve as their cognitive development advances, allowing increasing participation in discussions and decision-making.

Most pts with IgA deficiency are ...

asymptomatic - found incidentally - no further monitoring or interventions

Most common presentation for Hodgkin's lymphoma ...

asymptomatic lymphadenopathy - usually cervical or supraclavicular

Sturge-weber syndrome defined as

at least 2 of the following - facial port-wine stain - leptomeningeal angiomatosis - elevated intraocular pressure

What ADHD med carries a warning about SI?

atomoxetine - NE reuptake inhibitor - rare potential cardiac risk - improves ADHD behaviors without exacerbating tics

What kind of bacteria is Moraxella catarrhalis ...

beta-lactamase producing gram-negative diplococcus - colonizes upper resp tract - tx = augmentin or bactrim - otitis media, sinusitis, COPD

west nile virus is most commonly transmitted to humans by mosquitos. However, person-to-person transmission can occurs via ...

blood transfusion and solid organ transplantation

Recognize late decelerations, in which resuscitation of term infant with severe HT In mom may immediately require...

blood transfusions

In non-emergency situations, when red flags are identified by clinical evaluation of headache, what is the preferred neuroimaging modality?

brain magnetic resonance imaging - due to the high level of detail and lack of radiation exposure

Infant has detectable L-alloisoleucine. Dietary restriction?

branched-chain AAs - maple syrup urine disease - AR

Ehler-danlos is d/t defect in synthesis and processing of ...

collagen

6 mo old baby + referred for ophtho eval + required prolonged mechanical vent, tracheostomy, percutaneous gastrostomy insertion in NICU. His mom is healthy, with no evidence of neuromuscular disorder. PE = bilateral ptosis and restricted eye movements. Following admin of edrophonium, his ptosis improves, his sucking reflex becomes stronger, and his eye movements achieve full range. Most likely cause of pt's symptoms?

congenital myasthenia d/t congenital acetylcholine receptor deficiency - AR

What happens to ESR in kids with sickle cell?

decreased ESR - cells' inability to aggregate

Newborns with significant hypospadias and undescended testicles or ambiguous genitalia should be evaluated for ...

defects in sexual differentiation = karyotype, FSH, LH, testosterone, urinary 17-ketosteroids, 17-hydroxycorticosteroids

Echo findings of myocarditis

dilated left venitricle + decreased contractility

Warfarin is commonly associated with adverse drug reactions d/t drug reactions. Diclofenac may ...

displace protein-bound warfarin, increasing free biologically active warfarin and INR --> increases risk of bleeding

Manifestations of inhalant abuse include

double vision nystagmus excessive salivation flushed skin dysphoria delusions slurred speech hallucinations - Acute cardiotoxicity and dysrhythmias are the most common cause of death resulting from inhalant abuse - Chronic abuse of inhalants can lead to cardiomyopathy, leukoencephalopathy, neuropathy, and severe neurotoxic sequelae - The initial evaluation of a child suspected of intoxication due to inhalant abuse includes electrocardiography to assess for arrhythmias, arterial or venous blood gas analysis, and a basic metabolic panel. A urine drug screen may be considered to evaluate for polysubstance abuse

Esophageal obstruction presents clinically with chest pain, dysphagia, and the inability to swallow fluids or oral secretions. Emergent consultation with a pediatric gastroenterologist and a therapeutic endoscopy are indicated for esophageal obstruction. Older children with esophageal food impaction are likely to have

eosinophilic esophagitis

Used to quantify GERD

esophageal pH monitoring intraluminal esophageal impedance

Cortisol timing to assess for Cushings

evening - around 11pm

A low T-cell receptor excision circle (TREC) level on newborn screening raises concern for primary immunodeficiency and should be evaluated by

flow cytometry for lymphocyte subsets

Pt with X-linked agammaglobulinemia. Infants clinically well for first few mos of life d/t maternal Abs that cross placenta. A 6-9 mos, infants develop recurrent or chronic infections with S pneumoniae, H influenza, S aureus, pseudomonas. Pt with these features should undergo what testing ...

flow cytometry testing

An infant with normal growth and development is seen in the clinic for a health supervision visit. She is initially fearful and looks to her mother when the examiner enters the room. With time, she grows calm, smiles, and laughs when the examiner plays peek-a-boo with her. After squirming in her mother's arms she is placed standing on the ground. In response, she holds onto the seat of a chair and takes a few steps. She turns when her mother says her name during the visit and makes a variety of vocalizations using vowel and consonant sounds as well as shrieks and "growls." Of the following, this infant's fine motor abilities MOST likely include

grasping a cube with her thumb and fingertips 9-month-old infant - At this age, fine motor skills include the ability to grasp a cube with the thumb and fingertips ("radial-digital grasp"), and bang 2 objects together - An inferior pincer grasp of a small object like dry cereal typically develops around 9 to 10 months of age as well

What is most commonly associated with biliary colic in kids?

hemolytic anemia - sickle cell; hereditary spherocytosis

16 yo M + rash on neck that started 2 days ago + initially had burning pain in neck 4 days ago + IUTD + PE = 20 vesicular lesions of neck (3 mm in diameter over erythematous base). Cause?

herpes simplex virus, type 1 - herpes gladiatorum

lab abnormalities of tumor lysis syndrome

high K low Ca high P high uric acid

A 13 year old girl presents with irregular menstrual cycles. She had menarche at 12 years of age. She has no complaints but is worried because she has skipped her period 3 times in the past year. What is the most common time frame for ovulation to begin in this patient?

highly variable but often following menarche by 12-24 mos

Contact precautions are recommended for

hospitalized patients with - parainfluenza virus - respiratory syncytial virus

A 10-year-old girl presents with a 6-month history of constipation and occasional right-sided abdominal pain. She has no history of fevers, night sweats, urinary frequency, dysuria, emesis, or hematuria. Her appetite and activity have been normal. She has no drug allergies and she is taking no medications. Physical exam reveals a well-developed, well-nourished girl in NAD. Vital signs are: BP 100/62 mm Hg; pulse: 90; RR: 20; T: 98.7. HEENT exam is normal. Neck is supple without lymphadenopathy; chest and heart exams are within normal limits. Abdominal exam reveals a 5 cm by 7 cm smooth mass in the right upper quadrant. No tenderness is elicited. No hepatosplenomegaly is noted. Some hard stool is palpated in the left lower quadrant. Rectal exam is heme negative. Neurological exam is within normal limits. Computed tomography shows a large cystic mass in the right upper quadrant contiguous with the right renal collecting system. No solid soft tissue component to the mass is noted. The remaining renal parenchyma on the right enhances normally with contrast and is otherwise unremarkable. The left kidney is normal; so are the remaining structures of the abdomen. What is the most likely cause

hydronephrosis - An abdominal mass in a child should trigger further investigation, especially to rule out malignancy. - Hydronephrosis, as in this vignette, is usually due to ureteropelvic junction obstruction and is the most common obstructive lesion of the urinary tract. The CT scan shows the dilated renal collecting system, lack of solid soft tissue involvement, and normally functioning renal parenchyma. These findings are quite suggestive of an obstruction of the normal outflow of urine from the collecting system. Imaging studies to determine renal function would be helpful in deciding whether to repair the affected kidney or remove it altogether.

pityriasis rosea tx'ed with

hydroxyzine - usually: initially larger lesion followed by smaller lesions/tinea that was previously treated with antifungal but resistant to med

Recognize Pityriasis Rosea, in which the management includes:

hydroxyzine Pityriasis rosacea - self-limited papulosquamous disorder (ie, lesions are elevated and have scale) - may be infectious (human herpesvirus types 6 and 7 have been implicated) - usually occurs in the spring and fall - eruption begins with a herald patch; this lesion is round or oval, often has central clearing, and exhibits scaling - within 2 weeks, a more widespread eruption develops that is composed of erythematous papules and plaque

Typical findings of pneumonia seen with C trachomatis in young infants ...

hyperinflation with bilateral interstitial infiltrates

An 18-month-old girl is brought to the emergency department for evaluation of a left arm injury. Twenty minutes before arrival, she was walking down the street holding her father's hand when she slipped. She never fell to the ground because her father tightened his grip on her wrist; however, since then she has been crying and not moving her left arm. On physical examination, she cries when approached and is holding her left arm to her side with the elbow slightly flexed. The parents are informed that their daughter's condition can be resolved with a quick maneuver. Of the following, the intervention MOST likely to be successful is

hyperpronation

Radial head subluxation is a clinical diagnosis; radiography is not warranted if the mechanism of injury is consistent with the diagnosis and there are no findings of swelling, ecchymosis, bony tenderness, or deformity. Reduction of radial head subluxation can be achieved via either

hyperpronation of the forearm or supination of the forearm and flexion of the elbow

6 yo F + short stature and constipation + ht at 10th %ile + wt at 60th %ile. Ht for age has deviated downward by 2 percentiles over past 2 years. Bone age is 2 years behind her chronological age. Mom is 5'2" and dad is 5'7". Cause of short stature?

hypothyroidism - poor growth, cold intolerance, wt gan, constipation, skin/nail changes

Most common cause of seizures in t term newborn

hypoxia - asphyxia

Interventions for varicella-zoster virus exposures are only considered for what individuals?

individuals without evidence of immunity - Interventions can include varicella vaccine, varicella-zoster immune globulin, and antiviral medications including acyclovir and valacyclovir - For individuals who are vaccine-eligible (have no vaccine contraindications and are ≥12 months of age), vaccination should be administered ideally within 3 days but up to 5 days after the exposure - Varicella-zoster immune globulin is indicated for immunocompromised individuals, pregnant women, and certain newborns. Ideally, it should be administered within 96 hours of exposure, but can be administered up to 10 days afterward. Immunocompromised individuals include those with T-cell immunodeficiencies (congenital, HIV), neoplasms of the bone marrow or lymphatic system, hematopoietic stem cell transplant recipients, and those receiving immunosuppressive therapy. - Newborn candidates include infants whose mothers developed primary varicella disease between 5 days before and 48 hours after delivery, hospitalized preterm infants (≥28 weeks' gestation) whose mothers lack evidence of varicella immunity, and all hospitalized preterm infants of less than 28 weeks of gestation. - If varicella-zoster immune globulin is unavailable, intravenous immune globulin can be substituted.

Preferred tx for PDA + pulmonary overcirculation + feeding intolerance in infant

indomethacin --> inhibits cyclooxygenase-1 and decreasing prostaglandin synthesis

Otherwise asymptomatic 3 yo boy, recently adopted from orphanage in Thailand, found to have induration at site of tuberculin skin test placed 48 hours previously. His IZZ records indicate he received once BCG vaccination when he arrived at orphanage. What defines positive TB skin test in this pt?

induration of 11 mm - induration of >/= 10mm = positive in children <4 yo; in those with chronic med conditions; in those with close contact with high risk individuals; those who have been to high-prevalence areas

Acts as a muscarinic antagonist in pts with asthma exacerbations

ipratropium

Presents with lethargy, vomiting within first few days of life. Urine that smells like sweaty feet ...

isovaleric acidemia

A 5-year-old boy is brought to the emergency department after being hit on the head with a tennis racquet. He has a 3-cm superficial laceration on his left eyebrow that will require suture repair. The remainder of his physical examination findings are unremarkable. Procedural sedation is planned with goals of providing sedation to facilitate the laceration repair as well as pain control during suturing. Of the following, the BEST medication to use for this boy's procedure is

ketamine - dissociative anesthetic - effects are mediated primarily by noncompetitive antagonism at the N-methyl-D-aspartate receptor and also agonism at the μ- and κ-opioid receptors - ideal for procedural sedation because it has analgesic, amnestic, and sedative properties without loss of protective airway or hemodynamic reflexes - important adverse effect of ketamine is the emergence phenomenon, which can manifest as disturbing hallucinations in older children and adults; it is often mitigated by the use of low-dose midazolam

Pts with hereditary tyrosinemia type 2 present with early eye and skin abnormalities, including corneal ulcers and dendritic keratitis. Tx involves...

limiting tyrosine (and phenylalanine, a tyrosine precursor) intake

Infant with pointed chin, butterfly vertebrae, heart murmur + jaundice = Alagille. Next diagnostic step? ...

liver biopsy - paucity of bile ducts

Contraindications for OCPs with estrogen + progestin

liver disease breast cancer being less than 21 days postpartum hx of thromboembolism vascular dz hx of stroke complicated valvular heart disease impaired cardiac function severe HT migraine with aura smoking SLE

apha1-antitrypsin deficiency (AATD) is an inherited disorder that can cause lung disease and liver disease in children and adults. Alleles responsible for defect are located on Pi locus of SERPINA1 gene. Severely depressed levels of protein (10-20) are found in those with Pi^ZZ phenotype. About 15% of those with Pi^ZZ get ....

liver fibrosis and cirrhosis - especially common in children

Salicylates stimulate respiratory drive, which leads to an increase in respiratory rate and depth, and thus respiratory alkalosis. This leads to ...

loss of bicarb in urine, as well as K --> as time progresses, K is depleted and kidneys attempt to conserve K at expense of H = paradoxically acidic urine

Nephrotic syndrome most commonly occurs due to minimal change disease. Loss of protein in minimal change disease is d/t ...

loss of negative charge in glomerular filtration barrier

2 yo M + abdominal pain and diarrhea + mom concerns he took some of her prenatal vitamins. Most appropriate next step?

measure serum iron concentration - measure iron 4-6 hours after ingestion

Findings on CSF of transverse myelitis include...

mild lymphocyte pleocytosis - and normal or slightly elevated levels of protein

When can a mom with TB begin breastfeeding?

minimum of 2 weeks of treatment

4 mo old infant + crying and blue lips + diagnosed with a heart prob at birth and is scheduled for surgery + perioral cyanosis, hyperpnea, tachycardia, hypoxia. No murmur today; although he had one weeks ago. Increasingly hypoxia and hypotension. Immediate initial management?

morphine and phenylephrine - Tet of fallot - morphine can be used too

Teen with benign paroxysmal vertigo of childhood. Presents in school-age. Out of blue nausea, dizziness. Lasts about 1 minute and goes away when child rests. For this kid, has lasted a few days. Next steps?

observation - can run in families - if longer or inhibiting with function, can treat with antihistamines

Most common tumor associated with retinoblastoma ...

osteosarcoma

Adolescents require hospitalization for PID if ...

patient is pregnant surgical emergency that cannot be excluded suspected pelvic/tuboovarian abscess nausea/vomiting that precludes oral therapy failure to respond to oral therapies after 24-48 hours

What causes body lice?

pediculus humanus corporis

predictive genetic testing involves ...

performing genetic test on a person who is at risk for developing a genetic disorder

Example of QI project that tests impact of change in real work setting ...

plan-do-study-act

Athletes who report undue fatigue or chest pain with physical activity during or after a URI or other febrile illness should be evaluated for

possible myocarditis

Radiographic visualization of what indicates an occult elbow fracture ...

posterior fat pad - blood or joint fluid in elbow joint via fracture

What is first seen in acute liver failure

prolonged prothrombin time

Baby experiencing recurrent SVT episodes that resolve with ice to face suggesting arrhythmia that depends on AV node. Most likely AV nodal reentrant tachycardia. Preferred preventative medication?

propranolol

Considered the reference standard of research design

randomized controlled trial

In kids with d-transposition of great arteries, why is early arterial switch surgery preferred?

retains left ventricular mass so that it can normally perform when attached to systemic circulation

Drug FDA approved for tx of irritability in children with autism ...

risperidone, aripiprazole - side effects = wt gain, high sugar, dyslipidemia, fatigue

Tx for verucca/warts on fingers of anxious 6 yo boy.

salicylic acid topically - takes a long time --> need to counsel (could take 3 months!) - would not cooperate with painful tx since he is anxious - cryotherapy hurts

Important tool for diagnosing Lyme dz ...

serologic testing

Abnormality in the brainstem receptor of what is linked to SIDS?

serotonin

rare disorder characterized by episodic hypothermia, hyperhidrosis, and agenesis of the corpus callosum - Though poorly understood, the absence of the corpus callosum in this condition is believed to lead to hypothalamic dysfunction and thus altered thermoregulation.

shapiro syndrome

umbilical granuloma tx ...

silver nitrate (salt works too)

Boy in clinic has findings consistent with preseptal (or periorbital) cellulitis, including unilateral erythema and swelling of the anterior eyelid with associated eye pain. Preseptal cellulitis is associated with

sinusitis dental infections trauma to the skin (eg, insect bite, atopic dermatitis, impetigo) ocular conditions such as hordeolum or dacryocystitis - Preseptal cellulitis can be treated with oral antibiotics and close follow-up, whereas patients with orbital cellulitis typically require admission to the hospital for multidisciplinary care - Common treatments include trimethoprim-sulfamethoxazole or clindamycin (for coverage of methicillin-resistant Staphylococcus aureus) plus amoxicillin, augmentin, or a third-generation cephalosporin like cefdinir or cefpodoxime (for coverage of Streptococcus pneumoniae, other streptococci, and anaerobic organisms)

Teen + overdose of tricyclic antidepressant. Sz, resp depression, tachycardia, dry skin. Anticholinergic symptoms. EKG with ventricular tachycardia. Tx?

sodium bicarb

Tx for ethylene glycol intoxication ...

sodium bicarb fomepizole pyridoxine thiamine

A pt that is struggling to avoid purging may find it beneficial to have accountability through ...

supervision for 2-3 hours after eating meal

CF leads to pancreatic exocrine insufficiency. Long-term management with what is necessary?

supplemental pancreatic enzymes and fat-soluble vitamins

Initial step in management of caustic substance (like drano) ...

supportive care and close observation

Should be considered in any child with severe, unexplained lung disease in infancy ...

surfactant dysfunction / deficiency - consider surfactant mutation analysis

Giving infants cow's milk (with a low of P) results in ...

symptomatic hypocalcemia

Successful reduction for intussusception will lead to rush of air reflux where?

terminal ileum / proximal to ileocecal valve

When immunizations are not current, what abx are used for community acquired pna ...

third-gen ceph - ceftriaxone or cefotaxime

Treatment of mild folliculitis

topical mupirocin and topical clindamycin

Infant + chronic diarrhea (>14 days) + FTT. Next step for evaluation?

upper endoscopy - to look for organic etiologies

15 yo boy + intermittent red or purple blood mixed in his stools for past several years. Does not have associated abdominal pain or rectal pain and he has not lost weight. On PE, you note prominent brown-black macules on his lips and buccal membrane. Most important to perform next?

upper endoscopy and colonoscopy - painless rectal bleeding = colonic polyps - additional freckling of lips = Peutz-Jeghers syndrome - AD - hamartomatous polyps in GI tract - risk of adenocarcinoma of bowel increased

High doses of vitamin B9 (folic acid) may mask

vitamin B12 deficiency

The initial evaluation for the cause of cholestasis in infant should include obtaining

α1-antitrypsin phenotype and level thyroid function tests abdominal ultrasonography reviewing the newborn screen results

Roseola is caused by ...

human herpes virus 6

For children with BWS, the risk for embryonal tumors is highest in young children through age 8 years. Surveillance is recommended, including

- abdominal ultrasonography every 3 months until age 8 years - serum α-fetoprotein concentration is recommended every 2 to 3 months in the first 4 years of age for early detection of hepatoblastoma

Recognize esophageal web

- can cause reflux-like symptoms and chest pain

Recognize myringosclerosis

- characterized clinically by calcium and phosphate crystal deposits within the tympanic membrane that appear as whitish plaques on otoscopy and may demonstrate decreased mobility on pneumatic otoscopy -b 32% of patients developed myringosclerosis after tympanostomy tube extrusion = typically asymptomatic and rarely requires treatment, as hearing loss that involves the ossicles is uncommon

In HOCM, ICD is necessary prevent fatal ventricular arrhythmias in what scenarios?

- sudden death or likely attributable to HCM in >/= first-degree relative or close relative who are less than 50 yo - massive LVH >/= 30 mm in any LV segment - >/= 1 recent episode of syncope suspected to be d/t arrhythmia - LV apical aneurysm - LV systolic dysfunction (</= 50)

sturge-weber syndrome CT results often show ....

dilation of ipsilateral ventricle calcification cerebral atrophy

Recognize Eczema Coxsackium

- While usually a clinical diagnosis, an enterovirus polymerase chain reaction (PCR) or culture of the skin can provide confirmation - Enteroviral rash seen in infants and children with underlying atopic dermatitis. - It is characterized by vesicles, bullae, and/or erosive lesions of skin affected by atopic dermatitis - Caused by coxsackievirus A6 generally results in a less painful rash, and affected children typically have a milder and shorter course of illness than those with herpes simplex virus infection. - Generally more widespread, consists of vesicles and erosions, and includes more than 10% of the body surface area without oral involvement.

Surgical correction of a cleft lip and/or palate varies based on the infant's other medical needs, weight gain, and severity of the defect. Typically - cleft lip can be repaired between: - Palate repair often takes place between:

- Cleft lip can be repaired between 2 to 6 months of age - Palate repair often takes place between 9 to 19 months of age; repair before 7 months of age is associated with an increased risk of midface hypoplasia - A maxillary bone graft to fill the alveolar cleft is often needed between 6 to 8 years of age - It is common practice for myringotomy tubes to be placed at the time of palate repair. All children with cleft palate require frequent evaluation by an audiologist.

Girl has signs and symptoms of fatigue, decreased appetite, weight loss, tachycardia, hyperpigmentation, and lower blood pressure has primary adrenal insufficiency (Addison disease) associated with autoimmune polyglandular syndrome type 1 (APS-1). Addison disease is caused by damage to the adrenal cortex. In the United States, autoimmunity is the most common cause of Addison disease. There are several distinguishing features of primary (ie, a problem with the adrenal gland itself) and secondary (ie, a pituitary or hypothalamus problem) adrenal insufficiency. In primary adrenal insufficiency, what are the following lab values: - glucocorticoid (cortisol) - mineralocorticoid (aldosterone) - Adrenocorticotropic hormone (ACTH) levels - plasma renin activity - Serum sodium levels - Serum potassium levels

- Deficiency of both glucocorticoid (cortisol) and mineralocorticoid (aldosterone) - Adrenocorticotropic hormone (ACTH) levels are high in response to low cortisol levels - Plasma renin activity is high in response to low aldosterone levels - Serum sodium levels are low due to both glucocorticoid and mineralocorticoid deficiency - Serum potassium levels are high due to the mineralocorticoid deficiency *Mineralocorticoid deficiency manifests as signs of dehydration. *Skin hyperpigmentation occurs due to the high ACTH levels. *If adrenal insufficiency is suspected, a bedside blood glucose test should be performed given the high risk for hypoglycemia. Serum electrolytes, glucose, cortisol, and adrenocorticotropic hormone levels should be obtained.

Recognize Ependymoma

- Ependymoma W.H.O. grade 11 = most common in children - Mean age is 6 years. - 70% of all ependymomas occur in the posterior fossa. - It is derived from the ependymal lining of the ventricular system. - It is usually non invasive and extends into the ventricular lumen displacing the normal structures.

A consensus statement from the American Heart Association and American College of Cardiology makes the following recommendations for the care and activity of athletes with Marfan syndrome:

- Evaluation of aortic root dimensions should be performed every 6 to 12 months - May consider participation in class IA or IIA sports if they do NOT have any of the following: Aortic root dilation; Ventricular dysfunction; Moderate or severe mitral regurgitation; Family history of aortic dissection at an aortic diameter of <50 mm - Avoid participation in contact/collision sports

Infants with PWS on the face should be evaluated for Sturge-Weber syndrome, which is characterized by at least 2 of the following:

- Facial PWS (forehead and upper eyelid usually) - Leptomeningeal angiomatosis - Abnormal intraocular vascular development

Most common cause of in-toeing in children - older than 3 years - younger than 3 years

- Femoral anteversion is the most common cause of in-toeing in children older than 3 years - Internal tibial torsion is the most common cause in children younger than 3 years

Acute hepatitis B infection - HBsAg: - HBsAb: - HBeAg: - HBeAb: - HBcIgM: - HBcIgG: - ALT:

- HBsAg+ - HBsAb- - HBeAg+ - HBeAb- - HBcIgM+ - HBcIgG- - ALT increased

Febrile seizures are common, occurring in 3% to 5% of all children. They are classified into 2 categories: simple and complex. For a febrile seizure to be designated as simple, it must meet all of these 5 criteria:

- Occur in a child between 6 months through 5 years of age - Occur in a developmentally normal child - Be generalized with no focal features - Last less than 15 minutes - Occur once in a 24-hour period If all criteria are not met, the seizure is classified as complex. In a neurologically normal child with a simple febrile seizure, routine electroencephalography and neuroimaging are not recommended. - Laboratory evaluation should be focused on the cause of the fever rather than the seizure. - If signs or symptoms of meningitis or encephalitis are present, the seizure cannot be classified as a simple febrile seizure, and a lumbar puncture should be performed.

seborrheic dermatitis treatment for infant scalp

- Selsun Blue wash (with scalp massage and allow to sit on scalp for two minutes) twice a week followed by moisturizing scalp with an olive oil or mineral oil - Lay off the eco gel and leave in conditioner for a while - Gels and petroleum based moisturizers can breed more seborrhea - Could culture In summary: If the seborrheic dermatitis is mild, an OTC topical antifungal cream or medicated shampoo with ketoconazole, selenium sulfide, coal tar or zinc pyrithione, may be enough to control symptoms.

Recognize seborrheic dermatitis around nose

- chronic and relapsing inflammatory disorder that affects areas in which sebaceous glands are concentrated - especially common in adolescents and young adults in whom sebaceous gland activity is greatest - although the cause is not fully understood, it may be the result of an inflammatory response to the yeasts of the genus Malassezia - typical findings are scaling of the scalp (ie, dandruff) or scaling and erythema of the eyebrows, eyelids, glabella, alar or retroauricular creases, beard or sideburn areas, or ear canals - for skin affected by seborrheic dermatitis, treatment is with a low-potency topical corticosteroid (eg, hydrocortisone 1% or 2.5%) or an agent active against yeast (eg, clotrimazole, miconazole nitrate, or ketoconazole) applied twice daily as needed - scalp involvement is treated with an antiseborrheic shampoo containing pyrithione zinc, selenium sulfide, or ketoconazole - if the scalp and face are involved, some shampoo should be allowed to contact affected areas and then rinsed off - if signs of scalp inflammation are present (eg, erythema or erosions), a topical corticosteroid solution (eg, fluocinolone acetonide 0.1%) may be applied at bedtime.

Recognize osteosarcoma

- classic = painful mass near growth plate - new bone formation = "sunburst" + cortical destruction - classic = adolescent, probably tall (volleyball), presents after trauma

Recognize eustachian tube dysfunction

- clear peak shifted negatively/left = negative pressure in middle ear - peak compliance may be normal - measurement of energy transmission through middle ear

Recognize Neurocysticercosis

- contrast-enhanced CT scan of the head shows a single ring-enhancing lesion with perifocal edema in the left parietal region - previously asymptomatic child who has presents with a sudden onset of complex partial seizures associated with a motor deficit (in the form of postictal right-sided hemiparesis) - diagnosis is confirmed by the contrast-enhanced CT scan of the head - most common parasitic infection of the central nervous system; it is caused by the larval stage of Taenia solium, or pork tape worm - eggs develop into larvae in the small intestines; they migrate across the intestinal wall into the bloodstream, and are carried to target organs (e.g., brain, muscles, and subcutaneous tissues) where they mature into cysticerci - most common cause of a single ring-enhancing lesion seen on a CT scan of the brain

Effect of ACEIs taken during pregnancy on surviving infants ...

- decreased fetal urine production/oligohydramnios - limb and hand anomalies - widely separated eyes - small, low-set and posteriorly rotated ears - facial deformities (small chin, beaked nose, unusual facial creases)

Differential for prolonged PT

- early liver failure - early/mild vitamin K deficiency - early DIC - factor 7 deficiency - factor 7 inhibitor

Recognize ascaris lumbricoides

- egg presentation = hard shell, oval, round - tropical climate, like Burma

Strategies to decrease ventilator-associated pna ...

- elevation of bed to at least 30 degrees - chlorhexidine oral rinses every 4 hours - extubation as soon as possible - suctioning of oral secretions before suctioning endotracheal tube - using separate catheter for oral suctioning - using closed suctioning systems for ETT suctioning - performing hand hygiene between pt contacts

Symptoms of galactosemia include FTT, jaundice, hepatosplenomegaly, liver failure, hypotonia, cataracts, vomiting, sepsis. Tx?

- eliminate galactose from diet - switch from BF'ing or dairy-based diets to soy or hydrolyzed formulas

Recognize scabies

- generalized eruption composed of erythematous papules - eruption involves the hands and feet, including the palms and soles - permethrin 5% cream topically - hydrocortisone and hydroxyzine could be used adjunctively to relieve pruritus, and cephalexin might be used if there was evidence of secondary bacterial infection

Nasal septal hematomas, caused by nasal trauma, management

- immediately drain septal hematoma - pack nasal cavity to prevent bleeding and recurrence of hematoma - broad-spectrum abx

Puberty is considered delayed when...

- in boys when there is a lack of testicular growth = 4 mL in volume (or 2.5 cm in length) by age 14 years - in girls, puberty is delayed when there is a lack of breast development by age 13 years

Urine sodium in ... - children with extrarenal losses of sodium (diarrhea, vomiting, burns) or increased proximal renal tubular reabsorption (nephrotic syndrome, CHF): - children with diuretic use, salt-losing nephropathy, or SIADH:

- low (<20 mEq/L) - high (>40 mEq/L)

Recognize staphylococcal pneumonia

- multiple pneumatoceles in the left lung - staphylococcal infection of lung may be a primary infection of the lung parenchyma or may be secondary to generalized staphylococcal septicemia - may be a complication of measles, influenza, or cystic fibrosis of the lungs; in addition, it may follow minor staphylococcal pyoderma - initially, the pneumonic process is diffuse - soon the lesions suppurate leading to formation of multiple microabscesses. These erode the bronchial walls and discharge their contents into the bronchi. This leads to formation of multiple thin-walled, air-filled cysts, which are called pneumatoceles. - Small pneumatoceles ultimately resolve and disappear within a period of a few weeks or months. - A large pneumatocele can rupture and lead to pyopneumothorax, which requires intercostals drainage under water seal. - Pneumatoceles are characteristically seen in X-ray films of the chest in cases of staphylococcal pneumonia as well as in cases of pneumonia due to Klebsiella.

Side effects of lithium

- psoriasis - acne - hypothyroidism - hypercalcemia - DI - leukocytosis - nausea - tremors

The typical 9-month-old infant can do what developmentally?

- pull to a stand and may begin to cruise - grasp a cube with a radial-digital grip and bang 2 objects together - say "mama" nonspecific and imitate sounds - orient to name and enjoy gesture games - recognize familiar people visually and follow when someone points

Recognize grade 3 vur

- reflux into collecting system with mild dilatation, slight ureteral tortuosity, no or slight blunting of fornices

Diabetes insipidus d/t craniopharyngioma: - serum Na - serum osm - urine osm

- serum Na: increased - serum osm: increased - urine osm: decreased

Girl has a clinical diagnosis of pneumonia based on findings of crackles and wheezing on lung examination, tachypnea, and mildly decreased oxygen saturation. The primary cause of pneumonia in preschool children is viral infection, and no additional evaluation or antibiotic treatment is needed. The most appropriate approach for the girl in the vignette is supportive treatment and monitoring clinical status. If she does not improve in 48 to 72 hours or worsens at any time, further evaluation for a secondary bacterial infection or complicated pneumonia is indicated. According to the Infectious Diseases Society of America/Pediatric Infectious Diseases Society guidelines for the management of community-acquired pneumonia, chest radiography or laboratory studies are not indicated for the initial evaluation, and antibiotic treatment is not needed for uncomplicated pneumonia in this age group. If there were reason to suspect a bacterial pneumonia, amoxicillin would be the antibiotic of choice rather than a macrolide. Indications for the use of antibiotics might include

- sudden onset of high fever after several days of mild symptoms - focal rather than diffuse findings on chest examination signs of respiratory distress - presence of another underlying condition making bacterial pneumonia more likely In school-aged children and adolescents, atypical organisms become more prevalent and should be considered in the differential diagnosis and treatment planning. In this situation, a macrolide antibiotic would be appropriate.

Recognize granuloma annulare

- the absence of erythema and scale supports a diagnosis of granuloma annulare (GA) - the lesions of GA resolve spontaneously within 2 to 4 years; no intervention is required - relatively common disorder with an unknown cause - it usually occurs in school-aged children and affects girls more often than boys - characterized by papules that form a ring; occasionally, the ring is incomplete - lesions may occur at any body site but commonly involve areas that are prone to trauma like the dorsa of the hands or feet - the lesions of GA may be skin-colored but often are erythematous or violaceous - most individuals have a single lesion, but multiple lesions may occur

Recognize Lamellar Ichthyosis

- thin, transparent film on body - eyelashes missing - eyelids seem inverted

Recognize pericardial effusion

- typical history of fever, cough, and chest pain marked while lying down and relieved while sitting up and leaning forward - low pulse volume, low BP, distended neck veins, hepatomegaly, and pulsus paradoxus of >10 mm Hg point toward a moderately large effusion - pulsus paradoxus of >20 mm Hg indicates cardiac tamponade - the diagnosis is further supported by low voltage ECG with ST-segment elevation and flattened T waves and by the chest X-ray showing generalized cardiomegaly with "water bottle" configuration, with no specific chamber enlargement and clear lung fields

DRESS syndrome has been associated with reactivation of what virus?

human herpesvirus 6 - DRESS syndrome can manifest 2-6 weeks after onset of drug therapy

Infant botulism should be treated with...

human-derived botulism immunoglobulin

Cardiopulmonary compromise can be seen in patients with severe scoliosis and Cobb angles >90 degrees. You would expect these patients to have restrictive pattern on pulmonary function test. What would expect to see on echo?

cor pulmonale

Foster kids should be given no more than how many atypical antipsychotic medications at a time?

1

For children 6 to 17 years of age with ADHD, the starting dose of guanfacine ER is

1 mg once daily - this amount may be increased in increments of 1 mg/week, up to 4 mg, the recommended maximum daily dose

Full-term, healthy, breastfed babies should receive daily supplementation at 4 mos of age with ...

1 mg/kg of elemental iron - continue until iron-rich complementary foods are regularly consumed

Pts receiving 2 mg/kg/day (or >20 mg/day if they weight >10 kg) of oral prednisone or an equivalent should delay vaccination with any live vaccine for how long after completion of steroid therapy?

1 mo

Newborn male, born at 30 weeks of gestation and weighing 1.7 kg, is admitted to the NICU following delivery by emergency c-section secondary to placental abruption. Prenatal records show his mother is HBsAg negative. At what chronological ages should this pt receive first in series of hepatitis B vaccines?

1 mo of age - By 1 mo of chronological age, all medically stable preterm infants, irrespective of initial birth weight or gestational age, respond with seroconversion rates similar to that of term and larger (>2 kg) preterm infants

The starting Miralax dose for children ages 1-5 years is

1 teaspoon per year of age daily - For example, a 3 year old child would get 3 teaspoons of Miralax. - This dose can then be increased or decreased by 12 teaspoon every third day until the child is having daily soft stools.

Hand-foot-and-mouth disease is a self-limiting illness with resolution by

1 week - Herpangina is characterized by painful, discrete yellow or white ulcers on the soft palate, uvula, tonsils, and posterior oropharyngeal area, often associated with abrupt onset of high fever, headache, and sore throat

General course of resistant/recurrent otitis media therapy

1) amoxicillin 2) augmentin 3) cefdinir 4) ceftriaxone x 3 days - If > 3-4 infections in 6 months, then go to ENT for consultation re possible T-tubes.

Severity and danger of neutropenia is related to ...

1) how prolonged is the neutropenia 2) what marrow reserves are there 3) are other components of immune system or host impaired - very severe example = Kostmann syndrome = prolonged period of neutropenia + no marrow reserves

The four key clinical features of PCD/primary ciliary dyskinesia are

1) year round, daily, productive cough 2) year round, daily non-seasonal rhinitis 3) neonatal respiratory distress in a term infant 4) laterality defects (eg, situs inversus totalis) - Low exhaled nitric oxide is found in both PCD and CF , therefore this test is only diagnostic of PCD when CF has been ruled out

Incontinetia Pigmenti phases

1. crops of inflammatory vesicles and bullae over trunk and extremities ~ peripheral eosinophilia 2. several mos later = vesicles clear spontaneously and replaced by irregular linearly distributed verrucous lesions on extremities, hands, feet --> resolve by 2 yo 3. thin bands of "swirly" brown, bluish-gray pigmentation that coalesce and darken further before slowly fading by adolescence --> leave streaks of atrophy and hypopigmentation (4th stage) - delayed dentition - pegged or conical teeth - partial adontia - alopecia - nail changes

1st step in emergency treatment of hyperkalemia

10 ml of 10% calcium gluconate over 10 minutes - followed by glucose and insulin

Child hospitalized presents with sz activity. Serum sodium is 120. Before hospitalization, she weighed 30kg. Rate per hour of sodium infusion required to prevent symptoms of hyponatremia in this pt?

10-20 ml/hr of 3% NaCl - (Desire Na - Pt's Na / 2) = (140 - 120 /2) = 10 mEq - Replace volume = NaD x (wt x0.6) = 10 x 30 x 0.6 = 180 mEq ~ 10 mEq / 0.5 mEq/hr = 20 hr --> Na will be replaced over 20 hours - 3% NS = 0.513 mEq/ml concentration - 180 mEq / 0.513 mEq/ml / 20 mEq = 17.5 ml/hour of 3% saline

At what age do kids lift a box to find a toy?

12 mos

At what age can you typically give pseudoephedrine?

12 yo

50-90% of kids can use a spoon while eating and stack four blocks by what age ...

18 mos - can walk backwards - walk up stairs with one hand held (16 mo skill) - kicks ball - uses spoon and fork - 10-25 words

A newborn boy is evaluated in the delivery room at 1 minute of life after an emergency cesarean section due to late decelerations. He is limp, pale, and unresponsive; he has a heart rate of 78 and a slow irregular respiratory rate. What is his Apgar score?

2 Appearance (Skin): 0: Entire body pale blue 1: Body pink and extremities blue 2: Entire body pink Pulse: 0: No heartbeat 1: Heart rate <100 beats per minute 2: Heart rate >100 beats per minute Grimace (response or irritability): 0: No reaction 1: Grimacing 2: Grimacing and a cough, sneeze, or vigorous cry Activity (Muscle tone): 0: Muscles are loose and floppy 1: Some muscle tone 2: Active motion Respiration: 0: Not breathing 1: Respirations are slow or irregular 2: Infant cries well This patient would receive 1 point each for heart rate and respiratory effort, and no points for muscle tone, reflex response, or color. Immediate resuscitation efforts would be initiated due to the low score.

An adolescent girl is seen for a health supervision visit. Her 15th birthday is tomorrow. She has no significant medical history and is healthy. She has received all of her routine childhood vaccines with the exception of the human papillomavirus (HPV) vaccine. Although she and her parents are amenable to the vaccine, she has a softball game today and would like to delay the immunization until her next visit because she is worried that the injection site will be sore. Of the following, the MOST accurate statement regarding this adolescent's immunization schedule is that she will need

2 doses if the first dose is given today and 3 doses if she delays - Two doses are required if the vaccine series is started before the 15th birthday and 3 doses if it is started on or after the 15th birthday, regardless of the HPV vaccine type (ie, quadrivalent or 9-valent)

A female newborn has a positive screen for cystic fibrosis. Genetic testing has identified a mutation in each of the patient's CFTR genes. Parental studies confirm that the patient's mother and father are both carriers. The parents are concerned about the risks of their other child (age 6) being a cystic fibrosis carrier. The 6-year-old child has been healthy their whole life. What is the risk that the patient's healthy sibling is a cystic fibrosis carrier?

2 in 3 This patient is affected, and the parents are each obligate carriers. The typical Punnett square for this family will indicate— a 1 in 4 (25%) chance of having an unaffected non-carrier child a 1 in 2 (50%) chance of having a carrier child a 1 in 4 (25%) chance of having an affected child This patient's sibling is unaffected, so the 1 in 4 chance that they could be affected is eliminated as a possibility; they are unaffected, so they cannot be affected. There are three remaining possibilities from the original Punnett square: 1 in 4 that they are unaffected non-carriers 1 in 2 that they are carriers These results constitute 3 quadrants of the Punnett square. 2 of these 3 quadrants result in the sibling being a carrier, so the risk is 2 in 3.

A healthy infant who was born at full term is seen for a routine health supervision visit. Her father reports that she coos and has begun to smile. When placed prone, she is able to keep her head up and is beginning to push herself up on her forearms. She is also able to bring her hands together at midline. She has not yet started to roll. Of the following, the MOST likely developmental age of this infant is

2 months - An infant with a developmental age of 2 months should be able to lift up its chest when prone, hold its hands at midline, smile, and coo.

Clinical diagnosis of NF1

2 of the following - >/= cafe au lei macules (>/=5 mm in diameter) in prepubertal individuals and >/= 15mm diameter in postpubertal individuals - >/= 2 neurofibromas of any type or 1 plexiform neurofibroma - freckling in the inguinal or axillary region - >/= 2 lisch nodules (iris hamartomas) - optic gliomas - distinctive osseous lesion - 1st degree relative who also meet criteria *Inheritance = autosomal dominant*

Child can: stand on tiptoes + throw ball overhand + kick ball. Age?

2 years

Peak height velocity usually occurs at what Tanner staging for females?

2-3

Most DRESS syndrome reactions occur when?

2-6 weeks after exposure to offending drug - common lab findings = elevated serum transaminases, eosinophilia, atypical lymphocytosis

For what degree of scoliosis is bracing warranted?

25 - 45 degrees

In general, an infant should gain how much each day in the first 3 months of life?

25 to 30 g

Pt presents with overdose of drug X. Plasma [ ] = 75mg/L. Insulin clearance rate = 100ml/min. Tubular reabsorption rate = 5mg/min. Excretion rate?

2.5mg/min Excretion rate = filtration rate - reabsorption rate - 7.5mg/min - 5mg/min = 2.5 *GFR = 100ml/min; plasma [ ] = 75ml/L --> 7.5mg/100ml

Vernix covers body in what age premature infants?

24-38 weeks

Patient most likely has Cushing syndrome, as evidenced by significant obesity with rapid weight gain, fatigue, exercise intolerance, muscle weakness, and hyperandrogenism. The growth curve demonstrates excessive weight gain with decreased linear growth velocity is concerning for hormonal cause. Best initial screening test for Cushing syndrome is

24-hour urinary free Cortisol level - negative = no Cushing

Leg length discrepancies greater than what length warrant evaluation for possible surgical intervention?

2cm

Recommended for empiric therapy as initial drug of choice for tx of bacterial cystitis in kids ...

2nd-gen (cefuroxime, cefprozil) or 3rd-gen ceph (cefdinir, cefixime, cefpodoxime, ceftibutin)

A 10-year-old female patient weighs 25 kg and is admitted to the intensive care unit (ICU) with headache and vomiting 2 days after emergency surgery for right-sided ovarian torsion. She received 2000 mL lactated Ringer's solution during surgery, then continued on 0.45% saline with 5% dextrose and 20 mEq/L KCl at 200 mL/hr in the post-operative period. Blood pressure is 134/92 mm Hg, and she has brisk deep tendon reflexes. At the time of transfer to the ICU, Na+ level 113 mEq/L, plasma osmolality 236 mOsm/kg, urine osmolality 900 mOsm/kg. She now exhibits seizure activity. What is the best initial management of this patient?

3% saline bolus of 75 mL - hyponatremia due to a combination of excessive fluid administration and syndrome of inappropriate antidiuretic hormone secretion (SIADH) caused by the stress of surgery - since she is now showing features of cerebral edema and seizure activity, her serum sodium requires quick correction before cerebral edema increases further and leads to brainstem herniation - Use of 3% saline is indicated in patients with acute-onset and symptomatic hyponatremia. A bolus of 3-5 mL/kg administered every 10-15 minutes is appropriate and should raise the serum sodium concentration by 2.5-4 mEq/L. Once seizure activity resolves, an infusion of 1-2 mL/kg/hr of 3% saline may be used to reduce cerebral edema.

25 yo M survives house fire. Has second- and third-degree burns over much of his body. Former heroin addict and wants to avoid taking narcotics. He is in severe pain. New non-narcortic called Painbegone. Volume of distribution of med is 7.2 L/kg, and clearance is 10,080 ml/kg/hour. Half-life of med?

30 minutes - half-life of drug = (0.7 x volume of distribution) / clearance rate = 0.7 x 7200 / 10,080 = 0.467 hours = 30 minutes

Lanugo covers entire body until what age gestation?

32 weeks gestation - absent from face between 33 and 37 weeks - present on only shoulders from 38 to 41 weeks

Chronic draining lesions in combo with water exposure (fishing tank, swimming pool) are indicative of

mycobacterium marinum

A 3-month-old female infant is brought to the clinic for difficulty with feeding and poor weight gain. She was born at 39 weeks' gestation with weight at the 50th percentile and had no complications. The mother states that the infant has been waking to feed about 2 oz every 3 hours. She often takes up to 40 minutes to feed, gets sweaty during her feeds, and seems tired afterwards. Her weight is 4.6 kg (second percentile), and she is thin. She has crackles in the lower lung fields and a grade 3/6 systolic murmur heard best at the left lower sternal border. The liver is palpable 2 cm below the right costal margin. Echocardiography findings are significant for a ventricular septal defect. A nasogastric tube is placed and bottle/gavage feeds are initiated with standard formula while awaiting a return call from the cardiologist. Of the following, the BEST feeding plan for this infant is

4 oz every 3 hours - Infant has systolic murmur, crackles in the lungs, and hepatomegaly, which are consistent with congestive heart failure likely related to her ventricular septal defect. - Her weight percentile for age has decreased from the 50th to the 2nd, indicating failure to thrive. - The recommended intake goal for infants with congestive heart failure is 140 kcal/kg/day. Since the infant's weight is 4.6 kg, she needs 644 kcal/day (140 kcal/kg/day × 4.6 kg = 644 kcal/day). - Standard formula contains 20 kcal/oz, so the infant requires 32 oz/day (644 kcal/day divided by 20 kcal/oz). - Feedings are typically every 3 hours at this age for a total of 8 feedings daily. A total daily intake of 32 oz divided by 8 feedings is 4 oz every 3 hours, making it the best response listed.

By what age can most children walk up and down stairs with 1 foot on each step?

4 yo

Metopic, sagittal, coronal, lambdoidal sutures fuse by ...

4 yo

Social smile happens at 1-2 mos of age. Infants can track objects by 2 mos of age. Smiles at mirror by what age?

4-5 mos

What is the risk of adverse pregnancy outcome in the case of a first-cousin mating in the context of an unremarkable family history?

4-5% - First-cousins share 1/8 of their genes in common - Couples who are first-cousins have a 1.7 to 2.8% increased risk above the 2-3% general population risk of adverse fetal outcome for an overall risk of approximately 5%

At what age do children respond to a change in the tone of voice ...

4-6 mos

If a child is uncooperative for vision testing, a second attempt should be made when?

4-6 mos later for 3 yo 1 mo later for 4 years and older - children unable to be tested after 2 attempts should be referred to an ophthalmologist experienced in care of children

Peak age for acute pharyngitis

4-7 yo - most commonly cause by viruses

Interval recommended for children younger than 7 yo who are "catching up" on their vaccines...

4-week interval

6 yo boy noted to be below 3rd percentile for height. His weight is at 30th %ile. Several dysmorphic features also identified, including webbing of the neck, downward slanting palpebral fissures, hypertelorism, and micrognathia. He also has prominent cubitus valgus. Murmur is noted, and echo reveals pulmonary valvular stenosis. What represents the most likely karyotype of this pt?

46, XY - normal karyotype with abnormal facial features + 2 clinical signs often observed in Turner syndrome (webbing of neck + cubitus valgus) = AD + variable expression + chromosome 12 = NOONAN SYNDROME - often have right-sided cardiac lesions - occurs in both girls and boys - has some intellectual disability - delayed sexual maturation - increased risk for bleeding diatheses

A 1-year-old girl is being evaluated for unusual facies, growth deficiency, significant developmental delays, hypotonia, hearing loss, and a previously diagnosed generalized tonic-clonic seizure disorder. Her facies are remarkable for microcephaly, "Greek warrior" helmet nose, high anterior hairline with prominent glabella, hypertelorism, epicanthus, high-arched eyebrows, downturned mouth, small jaw, and small ears with bilateral ear pits. Prenatal history is remarkable for intrauterine growth restriction. Evaluation for other anomalies includes echocardiography, which shows an atrial septal defect, and brain magnetic resonance imaging, which shows thinning of the corpus callosum. A diagnosis is made using a high-resolution karyotype. Of the following, the MOST likely diagnosis for this girl is

4p− deletion 4p− deletion, also known as Wolf-Hirschhorn syndrome commonly presents with - unusual facies - prenatal and postnatal growth deficiency - significant developmental delays - hypotonia - hearing loss - antibody deficiencies (69%) - cardiac defects (50%) - urinary tract anomalies (30%) - central nervous system malformations (80%) - epilepsy or electroencephalographic abnormalities

A 2-month-old male infant is seen for his health supervision visit. He is tracking, smiling socially, and holding his head up without support for a few seconds. He has a 4-year-old sister who is developmentally normal. His mother's brother died at 20 years of age with complications related to a muscle disease. He had calf hypertrophy and used his hands to crawl from a lying to a standing position. He required the use of a wheelchair by 10 years of age. The mother reports that she has no muscle weakness or cramps. The mother underwent echocardiography 5 years ago for chest pain that showed mild dilated cardiomyopathy felt to be related to her brother's disorder. The parents are concerned about their son's risk of experiencing the same condition. Of the following, the MOST accurate percentage chance that this infant has the same condition as his uncle is

50% - Duchenne muscular dystrophy (DMD) - This disorder of the muscle protein dystrophin is inherited in an X-linked recessive manner - Tests to confirm the diagnosis include elevated serum creatine kinase and molecular analysis of the DMD gene to look for the disease-causing variant. - Female carriers are reported to have mild muscle weakness and dilated cardiomyopathy; hence, cardiac surveillance also should be offered to female carriers in the family. - Patient's uncle had DMD, the mother is symptomatic with dilated cardiomyopathy, and the maternal grandmother is an obligate carrier because two of her children are affected. The risk of the infant's having DMD is 50%, because the mother could pass on either the mutated or the normal DMD allele to her son.

Functional peripheral pulmonary stenosis is a common, benign finding in young infants and typically resolves around ...

6 months old - 2/6 systolic murmur best heard at left upper sternal border - radiates to right upper sternal border and into posterior lung fields and axillae

Chronic urticaria can occur and persist for how long?

6 weeks or more - is most often idiopathic

At what age does AAP currently recommend parents administer OTC cough and cold medicines for children without a prescription ...

6 years old

Frontal sutures fuse by ...

6 yo

Severe side effects of ecstasy

autonomic dysfunction coagulopathy

At a boy's health supervision visit, his father wishes to know how to manage the child's recent behaviors. Last week, after a trip to the store, he found a new toy in his son's jacket pocket. When asked, the boy stated that he did not know how the toy came to be there. He later admitted to taking the toy because he did not think that his parents would buy it for him. The boy's father has also noted that when playing board games, his son will change the rules to his advantage. The child has been in good health and his development and behaviors have been age appropriate up to this point. Of the following, this child's behavior is MOST like that of a

6 yo - The behavior of the child in the vignette is most like that of a typical 6 year old who may understand that there are rules but may have a hard time controlling his impulses.

Prednisone or prednisolone is the first-line treatment for idiopathic NS. The recommended starting dose for an initial episode is

60 mg/m2/day (2 mg/kg/day) in two divided doses for 6 weeks, followed by 40 mg/m2 (1.5 mg/kg/day) as a single dose every other day for 6 weeks. - Nephrotic syndrome is known to have relapses and remission. - The treatment of relapse includes prednisone at 60 mg/m2/day (2 mg/kg/day) in two divided doses until the urine protein is negative or at a trace amount for 3 days, followed by 40 mg/m2 (1.5 mg/kg/day) as a single dose every other day for 4 weeks

Overhead throwing should be limited to

8 months per year - with break intervals lasting at least 2 consecutive months to allow for adequate recovery and to minimize injury risk - Injury risk is markedly increased in throwers participating on multiple teams simultaneously, and current recommendations are to limit participation to 1 team per season.

During a routine visit, an infant turns when her name is called and looks at her father for reassurance when frightened. Although she is silent in the office, her parents report that she says "mama" and "dada" in a nonspecific way. These milestones are most typical of a child of what age?

9 mos - The ability to attach meaning to a specific sound; seeks reassurance from a parent or caretaker

Normal separation anxiety, making it hard for children to go back to sleep, happens at appx what age?

9 to 18 mos old - can use "transition" object at appx 12 mos (blanket or stuffed toy) for comfort to child

Recognize rotavirus ...

= excess fluid and electrolyte secretion

In high prevalence regions of the world, positive TB skin test is ...

>10mm

Group A streptococcal pharyngitis can be diagnosed with a rapid antigen detection test or a throat culture. Antigen testing has false negatives; culture is considered the gold standard and should be ordered if the rapid antigen test is negative. Antimicrobial treatment of GAS pharyngitis decreases the length and severity of illness, prevents acute rheumatic fever, and reduces the risk of local suppurative complications like peritonsillar abscess. What is the preferred treatment?

A 10-day course of penicillin (or amoxicillin because of its superior palatability in suspension form) - Group A streptococcal pharyngitis is almost universally sensitive to penicillin. - Children who are allergic to penicillin can be treated with a first-generation cephalosporin (if no history of anaphylaxis), macrolide, or clindamycin.

4 yo with hx of recurrent seizures has left hemispheric atrophy associated with leptomeningeal angioma on cranial MRI. What is most likely to be identified during additional evaluation of this pt?

A cutaneous port wine stain involving 1st and/or 2nd divisions of trigeminal nerve - Sturge-Weber syndrome - leptomeningeal angioma (large tortuous venous structures) usually occurs on same side as port-wine stain - focal tonic-clonic seizures by 1 yo in most pts - pts at high risk of glaucoma (require ongoing monitoring of intraocular pressure)

Prolonged symptoms. 2-3 weeks of cough, etc. Bilateral crackles on lung exam. No focal findings. Dx?

mycoplasma pna

A 16-year-old adolescent girl is seen for completion of paperwork for an overnight school trip. She has no significant medical history and takes no medication. Her review of systems is significant for weight gain of 9 kg over the past 2 years and irregular menses. Menarche was at age 12 years and menses have occurred every 2 to 3 months since that time. She does not eat breakfast, eats lunch at school, snacks when she comes home from school, and eats dinner at home. She does not exercise regularly. Her family history is significant for hypothyroidism in her mother and diabetes in her father and paternal grandmother. Vital signs show a heart rate of 96 beats/min and blood pressure of 126/84 mm Hg. Her weight is 92 kg (98th percentile), height is 168 cm (80th percentile), and body mass index is 32.6 kg/m2 (98th percentile). She has acanthosis nigricans over the nape of her neck and flesh-colored striae over her abdomen. Her physical examination findings are otherwise unremarkable. Of the following, the BEST test to order is a

A1C - Screening for prediabetes and type 2 diabetes is recommended in children and adolescents with a body mass index ≥85th percentile and at least 1 other risk factor (family history or evidence of insulin resistance) - Screening options for prediabetes and diabetes include obtaining levels for fasting plasma glucose, 2-hour plasma glucose during a 75-g oral glucose tolerance test, or hemoglobin A1c. - Components of metabolic syndrome include obesity, glucose intolerance, hypertension, and dyslipidemia.

18 yo transported to ED 1 hr after intentional overdose of his brother's med. Pupils are small, and he is difficulty to arouse. HR is 50, RR is 8, BP is 70/30. Temp and glucose are normal. Medication that most likely cause his symptoms is used to treat what disorder?

ADHD - clonidine overdose - treatment = supportive

Ewing sarcoma is more common in what race?

Caucasian - small, round, blue cell tumor - neural crest origin - onion skin appearance - more common in children <10 yo - osteoid production by spindle cells - typically presents in diaphysis - slightly more common in males

Best medication to administer for organophosphate toxicity:

Atropine - Organophosphates stimulate both the sympathetic and parasympathetic nervous systems; however, the typical clinical scenario of organophosphate toxicity involves symptoms of overstimulation of the parasympathetic system, like urination, emesis, bradycardia, bronchospasm, and miosis (pupillary constriction).

Abnormal physical examination findings for a newborn with osteogenesis imperfecta include

blue or gray sclera triangular face macrocephaly with large fontanelle pectus excavatum or carinatum

3 yo pt with hx of recurrent pulmonary infections presents with onset of cough, dyspnea, and chest pain. No fever or stridor on exam. Chloride sweat tests in past have been negative. Chest radiograph reveals pneumothorax. Likely cause?

Abnormal budding of tacheal diverticulum in utero = bronchogenic cysts = via abnormal budding of tracheal diverticulum of foregut before 16 weeks gestation --> can cause recurrent infections, pneumothorax (from cyst rupture), and undergo malignant transformation - diagnosis via CT and MRI - tx = excision

A 2-year-old girl presents for a well-child visit. Her mother has a question concerning a newer form of pneumococcal immunization. Past medical history and family history are unremarkable. Growth and development are normal and immunizations are current, including a series of 4 doses of pneumococcal conjugate vaccine (PCV7); the last dose was administered at approximately 15 months of age. Vital signs are normal. Weight and height are at the 50th percentile. The examination is entirely normal. What advice should be given regarding pneumococcal immunization?

Administer a single dose of PCV13 now - Currently, routine immunization with PCV13 (which has supplanted PCV7) consists of 1 dose at 2, 4, and 6 months, with an additional dose at 12 to 15 months to complete the series. - For healthy children ages 14 - 59 months who have completed an age-appropriate series of PCV7, a single supplemental dose of PCV13 should be given at least 8 weeks after the last dose of PCV7. - When a full or age-appropriate series of PCV7 has been given, a full series of PCV13 is not needed.

A 17-month-old female child is brought to the emergency department after her mother noted 3 hours ago that some of her prenatal vitamins were missing. The mother has brought the bottle with her. It originally contained 100 tablets, of which the mother has taken approximately 30. Only 55 tablets remain. Each tablet contains 30 mg of elemental iron. The child has been complaining of abdominal pain and has vomited 3 times. Physical examination revealed an 11 kg girl in mild distress. Vital signs are normal without signs of dehydration. There is mild generalized tenderness of the abdomen without guarding or rebound. Bowel sounds are mildly hyperactive. Brown stool in the rectal vault was guaiac negative. You have obtained a CBC, serum chemistries, liver enzymes, and iron level, the results of which are pending. KUB revealed about 10 radio-opaque tablets in the intestine. Based upon the presumed maximum iron ingestion and the clinical features above, what should be the next step in management?

Administer polyethylene glycol/electrolyte solution and await iron level before deciding whether to administer deferoxamine - If a significant amount of elemental iron is ingested, severe toxicity, including liver damage, gastrointestinal scarring, and coagulopathy can occur. - When the radio-opaque pills are visible on x-ray, intestinal lavage with polyethylene glycol/electrolyte solution is warranted. Here, the maximum dose of elemental iron is 450 mg, or approximately 40 mg/kg. - In an ingestion of less than 60mg/kg, one can wait for iron levels before undertaking chelation with deferoxamine unless clinical signs and symptoms otherwise indicate.

16 month old girl brought to ED with generalized tonic-clonic seizures. For past 3 days, she has been vomiting and has had several episodes of diarrhea. Has been tolerating some liquids but refusing all solids. This morning, her parents found her unresponsive with tonic-clonic movements of extremities. Currently unresponsive. Tachypneic and tachycardic. Afebrile. Anterior fontanelle is closed. Liver moderately enlarged and smooth. Remainder of exam is normal. CMP reveals normal anion gap acidosis with glucose of 18. AST and ALT are 3x normal. CBC and UA are normal without ketones present. CT of head is normal. CSF cell counts and protein are wnl. You infuse dextrose solution and start CTX. Child's glucose normalizes and acidosis improves slightly, but improvement in her mental status is very slow. Serum organic acids are ordered but results will not be available for a week. What should you consider?

Administration of carnitine - MCAD deficiency - hypoketotic hypoglycemia - Reye-like illness in first 3 years of life - asymptomatic until placed under metabolic distress - ex = severe presentation after gastroenteritis - plasma and tissue carnitine levels depleted tx = frequent carb feeds

A 6-month-old Caucasian female infant presents with developmental delay; the parents have concerns because she is not responsive to sounds. You review her chart and read that she was hospitalized 2 months ago for pneumonia treated with IV gentamicin. She has also been prescribed glasses by an ophthalmologist for exotropia, but she had an otherwise ocular negative exam. Your physical examination is negative for any overt dysmorphology. An audiogram diagnoses profound bilateral sensorineural hearing loss. A temporal CT scan is unremarkable. Urinalysis, CBC, comprehensive metabolic panel, and TORCH titers, which are negative. The family history is significant for a paternal male cousin recently discovered to have a unilateral multicystic dysplastic kidney. A maternal aunt has mild unilateral hearing loss that is presumed to be isolated with childhood onset. What is the most likely explanation for this patient's hearing loss?

Aminoglycoside ototoxicity - Aminoglycoside ototoxicity (AO) is due to one of two known mutations in the mitochondrial 12S ribosomal RNA gene. - An individual with such a mutation is susceptible to irreversible AO due to primarily vestibular or (less frequently) cochlear toxicity secondary to aminoglycoside production of inner-ear free radicals that damage the sensory cells and neurons.

A 30-year-old Caucasian woman elects a chorionic villi sampling (CVS) procedure secondary to a first trimester test that was screen positive for Down syndrome, risk 1 in 75. She has a history of polycystic ovaries and is obese. The CVS revealed a normal female karyotype, and the family was given the good news. A level II ultrasound 4 weeks later revealed normal fetal anatomy with the fetal gender visualized as male. What is the most appropriate and direct follow-up testing to clarify the true gender of the fetus?

Amniocentesis for fetal karyotyping - A CVS procedure carries a 1% risk of requiring a follow-up diagnostic test, such as amniocentesis, to further interpret initial results. There is a less than 1% risk of maternal cell contamination on CVS, which is a possible explanation for the gender discrepancy. - If the amniocentesis result reveals male gender, then this leads the clinician to postulate that maternal cell contamination or confined placental mosaicism are the likely culprits. If the amniocentesis reveals female gender, further ultrasound analysis and consideration of a possible sex-reversal condition would then be appropriate.

A 2-month-old Caucasian female infant is seen for a well baby visit. The mother is concerned that the child is floppy, but is uncertain, since this is her first child. Birth weight was 7 pounds 2 ounces at term. Prenatal and perinatal histories are unremarkable except for occasions in the third trimester during which the mother felt the baby kicked less frequently. The mother was 30 years old at the time of delivery. Prior medical history revealed almost continual nasal congestion. Physical examination revealed normal vital signs and growth parameters. Findings included a slight upward slant of the palpebral fissures, extra folds of skin at the inner ocular canthi, small ears, tongue thrusting, short fifth fingers bilaterally, and a single palmar flexion crease on the left. Muscle tone was moderately decreased throughout, with a pronounced head lag. Chromosome analysis performed upon peripheral blood lymphocytes revealed a 46,XX, t(15q;21q) karyotype. The results of chromosome analyses performed upon the parents are as follows: Mother: 46,XX Father: 45, XY, t(15q;21q) How should you advise the parents?

Amniocentesis is indicated for future pregnancies, as well as peripheral blood leukocyte chromosome analyses for all of the father's first degree relatives - The father, who is clinically normal, is a carrier of a balanced Robertsonian translocation and therefore has only 45 chromosomes. - His blood relatives may be carriers as well. - Amniocentesis is indicated for all future pregnancies to rule out a recurrence of the unbalanced translocation.

Abx for infant <28 days + fever, serious bacterial illness.

Amp + cefotaxime or amp + gent. + acyclovir (in ill-appearing infants until results back)

Recognize Cryptococcus neoformans and the tx ...

Amphotericin B and flucytosine - soil and pigeon droppings - cryptococcal meningitis

Contraindications to rotavirus vaccination include:

Anaphylaxis to the vaccine or components (ie, latex in RV1) History of intussusception Severe combined immunodeficiency - Infants are at slightly higher risk of intussusception for up to 7 days after receiving the rotavirus vaccine

9 yo M + 1 mo hx of tender, dusky skin nodules. He also has necrotic lesions of skin and intermittent fevers. Mother reports he lost 22 pounds in last 6 mos. BP 140/105 mmHg. PE = palpable purpura and livedo reticularis. Most likely to be found on imaging of this pt?

Aneurysm - polyarteritis nodosa - necrotizing vasculitis - associated with hep B

Parents of 3 yo girl concerned about her dev't. Delays were first noted in early infancy. She only recently began walking with aid of reverse walker. She has no speech and communicates with gestures and vocalizations. She is happy and easily excitable child. Her height and wt plot 25 %ile, but her HC is <3%ile. She ambulates with support; however, she has a wide-based ataxic gait. Most likely etiology of this child's features?

Angelman syndrome - microcephaly and seizures common

Can present with heart failure. Prominent Q waves on electrocardiogram are a characteristic feature, given the ischemia from decreased left coronary artery blood flow as the pulmonary vascular resistance drops.

Anomalous coronary artery from the pulmonary artery (ALCAPA)

Should be suspected in any child with new-onset painful torticollis.

Atlantoaxial rotary subluxation, a subluxation of the facet joints between C1 and C2 - Peak incidence is between 6 and 8 years of age, and is attributed to the relative ligamentous laxity in this age group. - Atlantoaxial rotary subluxation can be seen after trauma; in patients with Down syndrome, rheumatologic disease, or other congenital or acquired abnormalities of the upper cervical spine; and rarely after an upper respiratory infection, tonsillectomy, or other surgery involving the upper airways. - A child with any evidence of myelopathy should be referred for neurosurgical consultation and possible magnetic resonance imaging (MRI). Although MRI shows superior visualization of the spinal cord and soft tissue without radiation exposure, it typically does not demonstrate sufficient detail of bony anatomy to diagnose AARS. Computed tomography is the imaging modality of choice in the absence of myelopathy or focal neurologic deficits. - Atlantoaxial rotary subluxation will typically resolve within 1 to 2 weeks with conservative management, which may include rest, soft collar, nonsteroidal anti-inflammatory drugs, stretching exercises, and/or physical therapy. Prolonged persistence of this subluxation makes definitive treatment more difficult, and these patients should be closely followed to ensure resolution. Children with symptoms that persist despite these measures, or those with delayed presentation, should be referred to a neurosurgeon or pediatric orthopedic surgeon for initiation of traction therapy. Surgical reduction and fixation is indicated for children who do not respond to traction therapy, have neurologic deficits, or have recurrent episodes of AARS.

A young mother brings in her 1-week-old uncircumcised male newborn with concerns about his penis. It has become red and swollen, and the newborn has been increasingly fussy and irritable. She tells you that her mother-in-law instructed her to wash well under the foreskin and to keep it clean by pulling it back as much as possible. When she did this 2 days ago, she noted some minor bleeding. Last night, it began to look a little puffy and pink. Today, it has become worse, prompting this visit. The newborn has been otherwise well, and he was eating well with normal number of wet and soiled diapers. On exam, his vitals are normal. His genitalia show a moderately swollen distal penile shaft and glans, with a constricting band of foreskin just proximal to the swelling. There is no discharge or bleeding. The rest of his exam is normal. What would be the most appropriate next step in the management of this infant?

Attempt to relieve the constriction manually. - The infant foreskin (fold of skin covering the head of the penis) begins to grow forward at 8-9 weeks gestation to eventually cover the glans penis. - Incomplete separation of the adjacent epithelial layers between the foreskin and glans results in adhesion-like attachments throughout most of the first postnatal year, impeding full retraction of the foreskin. - Separation of the layers by natural lysis occurs over the first few years, with a fully retractile foreskin by age 3. - Paraphimosis occurs when a tight foreskin is retracted and cannot be returned to its usual position due to its constricting effect on the shaft of the penis. Painful swelling of the shaft and glans distal to the constriction results from venous stasis. This condition requires immediate manual attempts to relieve the constriction and to prevent obstruction of the urinary tract, which can impede voiding and can cause ischemia and engorgement with ultimate gangrene. - Relief is best accomplished by sustained circumferential pressure on the distal swelling of the penis with lubrication and forward traction of the foreskin. - Topical anesthetic or penile nerve block may minimize the pain. - If manual attempts fail, an emergency dorsal slit of the foreskin will prevent ischemic injury to the penis.

14 yo girl + frequent and prolonged menses over last 8 mos. Noted heavy menstrual bleeding for last 9 days. Menarche was at 13 yo. Resting heart rate is 88. BP is 115/78 without orthostatic changes. Abdominal exam is unremarkable. Blood is emanating from cervical os on pelvic exam. Labs include 10.2 and negative urine pregnancy test. Most appropriate tx of this pt's condition?

Begin combined monophasic estrogen-progestin oral contraceptive and administer at set intervals until bleeding stops - moderate abnormal menstrual bleeding (>7 days and/or frequent menses every 1-3 weeks + moderate to heavy flow and hemoglobin >/= 10) - combined OCP's for those actively bleeding - combined OCP's (with min of 30 mcg ethinyl estradiol) = preferred in adolescents --> estrogen provides hemostasis Regimen for moderate HMB/anovulatory uterine bleeding - 1 pill every 8 hours until bleeding stops - 1 pill every 12 hours for 5 days - 1 pill once daily for total of at least 21 days

11 yo evaluated for sore throat + sores in her mouth and skin rash + recently adopted from Southeast Asia + limited diet + swelling of lips and gums, seborrheic dermatitis of scalp and eyelids and glossitis. Deficiency of what vitamin?

B2/riboflvin - angular cheilosis - keratitis - sore throat - edema of mucus membranes - seborrheic dermatitis

Recognize bullous impetigo

BI - begins as a single red macule or papule that quickly becomes a vesicle. The vesicle rapidly enlarges into flaccid bullae with sharp margins without surrounding erythema. The bullae may rupture, ooze, and leave yellow crusts. The rash spreads by autoinoculation. - Moist, intertriginous areas, such as the diaper area, axillae, and neck folds, are commonly affected. - Systemic symptoms are uncommon. Most cases are self-limited and resolve without scarring within several weeks. - Bullous Impetigo in a Neonate

Infant (6 weeks old) comes to ED. RR 8 breaths/minute. SBP in 60s. O2 sat 86%. Next step?

Bag-mask ventilation and prepare for intubation - could actually help BP a bit with ventilation - airway most important currently

Crying 3 yo + macroglossia + microcephaly + hx of umbilical hernia. Most likely dx?

Beckwith-Wiedemann syndrome

Syndromes associated with Wilms tumor

Beckwith-Wiedemann syndrome Denys-Drash syndrome WAGR syndrome

Young child drinks unknown amount of mouthwash and is now unresponsive. Next step?

Bedside blood glucose - ethanol in mouthwash --> impaired hepatic gluconeogenesis = hypoglycemia in ethanol toxicity in younger children

A 3-month-old male infant presentS for his first well-child visit. He was born at 32 weeks gestation and experienced hyperbilirubinemia in the nursery, requiring phototherapy and exchange transfusion. He has had rhinorrhea without fever for 2 days. Physical examination revealS a normal-appearing infant at 25th percentile for height and weight. Vital signs are normal. There was mild clear rhinorrhea without respiratory distress. Two types of immunizations against rotavirus are available: RotaTeq and Rotarix. Should you administer rotavirus vaccine at this point?

Begin either immunization series now. - Rotavirus is the most common cause of gastroenteritis and diarrhea before age 2, especially at 3-15 months. Two types of immunizations are available: Rotarix is administered orally at 2 and 4 months; RotaTeq is given orally at 2, 4, and 6 months. Either series can be initiated after 6 weeks and before 15 weeks.

Most important sequelae of Chagas disease are bradyarrhtyhmias, heart block, dilated cardiomyopathy. First-line therapies?

Benznidazole and nifurtimox hints - dilated cardiomyopathy new after trip to South America - cardiomyopathy may resolve if treated early

11 yo child + hx of asthma + gets easily tired with exercise that resolves when resting. Vitals and PE are normal. Lab = microcytic hypochromic anemia. Hemoglobin analysis decreased amounts of HbA and increased amounts of HbF. Most likely cause of lab abnormalities?

Beta thalassemia minor

Dx? Microcytic hypochromic anemia. Raised ferritin. Mentzer index (MCV/RBC) classically less than 13. Elevated HbA2 up to 5%. Variable elevation of HbF. HbA >90%.

Beta-thalassemia trait

6 mo old boy, new to your practice, presents with fusion of index, middle, ring, and 5th fingers and common nail bed. Plain radiograph confirms the involved fingers share fused bone and soft tissue components. Thumb is relatively well formed and has full range of motion. Similar deformity present in both feet. What is most likely to be identified upon additional evaluation of this patient?

Bicoronal synostosis and maxillary hypoplasia - mitten-hand deformity associated with Alpert syndrome = one of several craniosynostosis syndromes - 10% have cardiac and/or renal probz - 50% have IQ <70

While BF'ing, a 2 day old girl with VSD suddenly becomes lethargic with associated generalized hypotonia and decreased respiratory effort. After stabilization, a cranial US reveals a large intracranial hemorrhage. Pt also has micrognathia; hypertelorism with broad forehead; low-set, posteriorly rotated ears. Labs include platelet count of 15,000 and hemoglobin of 15.2. Most likely to be identified upon further evaluation?

Bilateral radial aplasia - bilateral radial aplasia + congenital heart disease + severe secondary hemorrhage secondary to thrombocytopenia = thrombocytopenia with absent radium syndrome - many become symptomatic within first week of life - those who survive to 2 yo have spontaneous resolution of thrombocytopenia

Administered as cofactor in tx of propionic acidemia, an inborn error of metabolism that often presents in first month of life with acute onset of poor feeding, FTT, vomiting, lethargy, tachypnea, ketoacidosis, elevated anion gap, moderate hyperammonemia ...

Biotin

Adoptive parents of 2 yo twin boys presents for their first visit after their return from Africa. Parents report that the twins' biological mother attempted to breastfeed for as long as possible, but she estimates their nutritional intake has been extremely poor for at least 6-9 mos. On PE, the twins have a rash, are markedly edematous, and have evidence of severe hypoalbuminemia on lab eval. What best describes the likely characteristics of the rash noted on PE?

Blanching erythema with overlying reddish-brown scaling and sharply demarcated raised edges - Kwashiorkor = severe form of chronic protein malnutrition = apathy, anorexia, irritability, edema, dermatitis, photosensitivity, easy bruisability, hypoalbuminemia - "flaky paint dermatitis"

Neonate has Down syndrome or trisomy 21. It is the most common chromosomal disorder, with an incidence of 1 in 800 births across the world. What is the best test to confirm the diagnosis for any aneuploidy, including trisomy 21?

Chromosome analysis - Evaluation of a neonate diagnosed with trisomy 21 includes 2-dimensional echocardiography, ophthalmological evaluation, free thyroxine and thyroid-stimulating hormone levels, and hearing evaluation.

14 yo male returns to office 2 days after starting cipro otic drops for acute otitis externa. He has had severe pain and tenderness of his right ear for past 2 days. He is swim team coach and hopes to return to swimming soon. He has T1DM and uses insulin pump for management. He is febrile and auricle is swollen, red, and painful to touch. External auditory canal is swollen shut with purulent drainage at opening. Most appropriate abx management?

Ciprofloxacin PO - extensive inv't of auricle + occlusion of canal + immunocompromised --> tx with abx that cover P aeruginosa - if continues to worsen, consider malignant otitis externa

A 3-year-old boy presents with intensely pruritic skin lesions that have appeared intermittently since the age of 4 months. They have been most prominent at the elbow and knee creases. Family history is notable because his older brother, who had similarly severe skin lesions and who suffered from easy bruisability, passed away 2 years ago at the age of 10 years due to abdominal lymphoma. Past medical history is remarkable for prolonged bleeding at circumcision and an episode of pneumonia due to Streptococcus pneumoniae at the age of 6 months. Growth and development are normal, and immunizations are current. Vital signs are normal. The skin is generally dry, with thickening and fissuring at the antecubital and popliteal areas, along with excoriations and crusting. Similar changes are present to a lesser extent at the cheeks and extensor surfaces of the legs. Multiple ecchymoses are present, especially overlying the anterior tibiae. What is your next step in diagnosis of this patient's underlying condition?

CBC - Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder featuring persistent thrombocytopenia, often with small platelets, a variety of defects of the immune system (including decreased IgM with fairly normal IgG and possibly elevated IgA and IgD levels, impaired response to polysaccharide antigens and often to protein antigens, anergy, impaired lymphocyte proliferation in response to mitogens, and decreased T-cell number), significant eczema, and an increased risk of malignancy. - Severe and recurrent infections due to encapsulated organisms are frequent, along with infection due to viruses and Pneumocystis carinii. - The 1st sign may be prolonged bleeding at circumcision, as in this case.

An 8-year-old girl is being evaluated for repeated episodes of pneumonia. During these events, the girl is usually afebrile, but develops cough and sometimes spits out pink, frothy mucus. Chest radiographs have shown diffuse multifocal infiltrates. These episodes are usually treated with antibiotics and corticosteroids, and improve over several days. The girl has no history of asthma or wheezing, chronic or recurrent upper respiratory congestion with rhinorrhea, or kidney disease. Physical examination findings are normal today, and the point-of-care urinalysis is normal. Of the following, the laboratory test MOST likely to determine this girl's diagnosis is

CBC + diff - Pulmonary hemosiderosis characterized by repeated episodes of subclinical pulmonary hemorrhage that may present with cough and pink frothy sputum. - Chest radiography will demonstrate multifocal infiltrates. - The characteristic finding, other than the radiographic infiltrates, is anemia, which may be variable and exacerbated by episodes of hemorrhage. - Idiopathic pulmonary hemosiderosis is characterized by repeated episodes of intrapulmonary bleeding without evidence of autoimmune disease. - In some infants with idiopathic pulmonary hemorrhage, an association with mold exposure has been identified. - For most children with idiopathic pulmonary hemosiderosis, there is no identifiable trigger and treatment is targeted toward nonspecific inflammation.

Mom with chorio. High fever in mom. 36 weeks gestation infant. Mom wants early discharge. Patient is currently 2 hours old. Next step in management...

CBC, blood cdx, empiric abx - high sepsis risk in late preterm infant for early onset sepsis - height of fever correlates with risk of infection

A 10-year-old boy went scout hiking 2 weeks ago and pulled an engorged tick off his left calf. He later developed a round rash around the bite site that was treated with cool compresses and calamine lotion. He has had worsening headaches, fatigue, and joint achiness, especially to his knees. He has felt feverish, though a temperature has not been obtained. This morning he began to complain of neck stiffness and his mother noticed that the right side of his face seemed droopy compared to the left. There is no history of upper respiratory illness, vomiting, or diarrhea. His appetite has been fair to poor. No one else is sick. There is no history of drug allergies. On exam, his temperature is 101.8° F, pulse 115 bpm, BP 102/72 mg Hg, respirations 32/min. He seems pale, lethargic, and in mild distress. There is mild facial asymmetry with weakness on the right that is more pronounced upon forced smile and eye closing. There is mild nuchal rigidity, photophobia, and mildly positive Kernig and Brudzinski signs. Lungs, heart, and abdomen exam are normal. His skin exam reveals a large, round, erythematous, blanching rash with central clearing over the ventral surface of his lower left leg. Which

Ceftriaxone IV for 14-28 days - Lyme meningitis which is best treated with ceftriaxone IV in a single daily dose of 50-75 mg/kg per day for 14-28 days. - The current recommendation for the dose of ceftriaxone for Lyme meningitis is 50-75 mg/kg IV for 14-28 days.

A 4-year-old boy is seen in the emergency department for 6 hours of progressive restlessness, abnormal movements of the extremities, blurred vision, and drooling. His parents report that the boy was in good health until he felt something sharp touch his right great toe as he put on his shoes. His parents examined his toe at the time but were unable to see any marks. Over the course of the next hour he complained of a "funny feeling" in his right leg. Shortly thereafter, the other symptoms commenced. On physical examination, the boy's temperature is 38.2°C, heart rate is 140 beats/min, respiratory rate is 22 breaths/min, blood pressure is 110/60 mm Hg, and his room air oxygen saturation is 100%. He is alert and oriented and able to answer questions appropriately. The boy appears uncomfortable and is intermittently writhing on the stretcher. His pupils are equal, round, and reactive to light; however, he has abnormal extraocular movements. His mucous membranes are moist and he has minimal drooling. On auscultation, his heart has a normal rhythm with no murmurs, scattered expiratory wheezes can be heard; and his abdominal examination findings are normal. On skin examination, no marks are found and there is no bruising. Palpation of his right great toe produces intense pain. Of the following, the creature MOST likely to have caused this child's symptoms is

Centruroides sculpturatus (Arizona bark scorpion) - extremely painful and can also cause local numbness and swelling without significant erythema at the site of the sting or elsewhere on the body - palpation or percussion of the affected area causes an intense pain - in addition to significant local pain, scorpion stings have the potential to cause severe systemic symptoms; the venom contains neurotoxins that result in both sympathetic (eg, tachycardia, hyperthermia) and parasympathetic (eg, drooling, wheezing) symptoms - various neuromuscular symptoms, including restlessness, muscular fasciculations, ataxia, and cranial nerve abnormalities such as abnormal eye movements - presence of cranial nerve and/or somatic skeletal muscle dysfunction are evidence of a severe envenomation - other severe sequelae of scorpion envenomation include myocarditis, pancreatitis, and respiratory failure

The newborn nursery calls to report that a full-term infant girl born 10 hours ago has begun to have mild respiratory distress with nasal flaring and slight retractions. Though prenatal history was normal, the delivery was accomplished with low forceps because the baby's head had an oblique presentation. She also had a tight nuchal cord and required brief bag and mask ventilation with good response. Birth weight was 3.4 kg. On exam, the baby has a poor suck and cry. Deep forceps marks are present on both cheeks. There is mild nasal flaring, intercostal retractions, and faint abdominal excursion upon inspiration. Breath sounds are equal bilaterally and capillary refill is good. Real-time fluoroscopy reveals right hemidiaphragmatic paresis. What spinal nerve injury is most likely responsible for the patient's respiratory distress?

Cervical 3, 4 and 5 - Phrenic nerve injury is due to overstretching of the anterior roots of cervical nerves 3, 4 and 5. This results from lateral hyperextension of the neck, especially during breech births or difficult forceps delivery. - Most cases are associated with ipsilateral brachial palsy, although 25% are not. 0 Fluoroscopy will reveal elevation of the affected hemidiaphragm. - Treatment is supportive because most cases are mild with spontaneous recovery within 1-2 months.

Fully vaccinated 5 yo presents to ED with his parents, who are concerned about "rash all over his body." During preceding 48 hours, he had been uncharacteristically irritable with increased temp and complaints of headache, stomachache, and sore throat. On PE, he appears unwell, choosing to sit in his mom's lap instead of playing video games. Temp is 101.7F. Positive findings include fine, reddish-pink, papular rash on face, trunk, upper extremities, and thighs (but it spares his palms and soles). Most likely to be identified during further eval of pt?

Circumoral pallor - Scarlet fever - scarlatiniform rash via strep erythrotoxin - sometimes pruritic, fine, pinkish-to-erythematous, papular, sandpaper-like rash (goodflesh) on face, trunk, and extremities - spares circumoral area, palms, soles - rash commonly accentuated by linear petechiae = pastia lines in flexural surfaces of arms, legs, and trunk - desquamation of large sheets of skin 10-14 days after onset = prominent on fingertips and toes

The exhausted parents of a 9-week-old girl report that their newborn has been very fussy and crying "all day and night long" for the past 3 weeks. She has not been sick, there is no fever, and her appetite remains good. On further questioning, they report that she cries non-stop for 5-6 hours a day almost every day. They have tried rocking, bundling, singing, going for long car rides, and some over-the-counter drops, but nothing seems to help. She takes cow milk protein; her mother tried changing to a soy formula without much improvement. On exam, she is fussy, crying, and hard to console. Vitals are normal, and full physical exam is completely normal. There are no known allergies or positive family medical history. Her parents are exhausted and desperate; they are wondering about the use of complementary and alternative therapies after a relative suggested this, but they would like your opinion first. What complementary or alternative medical treatment with possible beneficial effects would be safest to recommend?

Chamomile - Infantile colic (crying >3 hours a day ≥3 days a week for ≥3 weeks) affects 26% of infants and resolves by about 3 months of age in approximately 50%. - Separate published scientific studies assessing the efficacy and safety of common CAM therapies have found that a botanical herbal tea blend including chamomile reported that crying was reduced in a significant percentage of infants compared with control. It should be avoided or used with caution in children allergic to ragweed, asters, and chrysanthemums. - Other CAM therapies showing potential benefit include infant message and parental behavioral training.

You are called to see a term baby in newborn unit for seizure-like activity. She was born yesterday via spontaneous vaginal delivery with no complications during pregnancy or delivery. Nurse described 30 seconds of clonic activity of right upper extremity that self-resolved. She is afebrile with stable vital signs. Baby appears to be sleeping but arouses to painful stimuli when you arrive. No focal deficits on exam. Best next step to determine etiology of pt's seizures?

Check serum glucose and calcium - to assess for neonatal seizures

Using random sample, epidemiology researchers conduct study to assess relationship between participants' zip codes and presence of bipolar disorder. Goal is to assess relationship between socioeconomic factors and risk of bipolar disease. What statistical test should be used to analyze data?

Chi-square test - compares samples with non-measurable nominal or categorical variables

You are evaluating a 5-day-old male infant due poor feeding and irritability. Birth weight at term was 3.1 kg, with an occipitofrontal circumference (OFC) of 35 cm (25th percentile). A lumbosacral meningomyelocele, diagnosed at birth, was repaired on the 2nd day of life. Prenatal, birth, and family histories are unremarkable. Vital signs are normal. Examination reveals an obviously large head with frontal prominence, a large, bulging anterior fontanelle, and dilated scalp veins. OFC is now 40.5 cm (> 95th percentile). Neurologic examination shows increased tone of the lower extremities, irritability, and poor suck. The eyes are deviated downward, with retraction of the eyelids and scleral show bilaterally above the irises.Cranial ultrasound reveals enlargement of the lateral, 3rd, and 4th ventricles. What associated anomaly is the most likely cause of this child's hydrocephalus?

Chiari II malformation - Failure of fusion at the stage of posterior neuropore closure between days 26 and 30 results in meningomyelocele, with subsequent maldevelopment of the mesoderm that forms the muscular and bony covering of the neural elements. - The presentation here is that of a lumbosacral meningomyelocele, a fluid-filled sac covered by a thin membrane of arachnoid infiltrated by blood vessels, topped by the neural placode containing splayed spinal cord and nerve roots. - Hydrocephalus develops in approximately 90% of patients with meningomyelocele. Ventriculomegaly is present at birth in about 20% of cases, and it develops after closure of the defect in about 70 % of cases. - Leakage of CSF from the sac may be related to absence of ventriculomegaly at birth, but it is not protective after closure. It is usually associated with a Chiari II malformation, which consists of downward displacement of the lower brainstem through the foramen magnum. In this case, obstruction occurred at the level of the foramina of Luschka and Magendie, causing dilatation of the lateral, 3rd, and 4th ventricles.

4 mo old had prolonged sz 6 days after his 2 mo WCC. Received standard 2 mo vaccines. He now returns for his 4 mo WCC. What statement is true regarding DTaP?

Child should no longer receive DTaP but may receive DT component at this visit - If child has encephalopathy or prolonged seizure within 1 week of gettin DTaP, pertussis component is contraindicated in future vaccines

A 15-year-old boy has had episodic abdominal pains about 2-3 times a week for the past 3 months. The pains are described vaguely as dull and achy with no localization. They start gradually and end gradually. There has been no change in bowel movements, which are reported to be regular and no change in the pain with defecation. Appetite has been good without any weight loss. He has been otherwise well and denies any dysuria, vomiting, substance abuse, or excessive ingestion of spicy foods or caffeine, nor problems with sleep, headache, or other somatic complaints. He also reports these pains have not interfered with his daily activities. Based on these findings, which of the following is the most likely diagnosis for this patient's chronic abdominal pain?

Childhood functional abdominal pain - episodic or continuous abdominal pain with insufficient criteria for other functional gastrointestinal disorders and in the absence of an inflammatory, anatomic, metabolic, or neoplastic process that explains the symptoms - must be present at least once per week for 2 months before diagnosis

Primary cause of ophthalmia neonatorum and usually occurs days 5-25 after birth.

Chlamydia - dx via NAAT

18 yo male + right inguinal lymphadenopathy + extremely painful to palpation. Noticed "bump" on penis several weeks ago that "never hurt and went away by itself." Additional findings on PE include tender right femoral LAD that is clearly distinct and separate from inguinal nodes. Most likely cause of pt's findings?

Chlamydia trachomatis - groove sign = lymph nodes above and below inguinal ligament = secondary/inguinal stage of lymphogranuloma venereum via L1, L2, L3 serovars - tx = doxy PO 100mg BID for 3 weeks

The parents of an 8-year-old boy are worried about his height because he is the shortest child in his class. The boy was born at full term and had a normal birth weight. The neonatal period was uneventful, and he achieved his developmental milestones on time; his elder brother, who also achieved his developmental milestones normally, is of normal height. His anthropometric measurements are as follows: Height: 104 cm (41 in) (<1st percentile) Weight: 20 kg (3rd percentile) Head circumference: 53 cm (average adult male head circumference: 56 cm to 61 cm) Span: 100 cm Upper segment: Lower segment ratio 1.5: 1 His bone age is appropriate for his chronological age. What is the most likely diagnosis?

Chondrodystrophy - also known as 'achondrosplasia' - short height, span less than height - upper segment: lower segment (US:LS) ratio of more than 1 - bone age appropriate for chronological age

Typically ordered as a first step in the medical evaluation of a child with intellectual disability (ID) ...

Chromosomal microarray - The most common genetic cause of intellectual disability is Down syndrome whereas the most common inherited cause of intellectual disability is fragile X

Recommended first-line test for multiple congenital anomalies not suggestive of a specific syndrome, autism spectrum disorder, and unexplained non-syndromic developmental delay.

Chromosomal microarray analysis

A 14-year-old boy presents for a sports physical. History is significant for a high degree of myopia bilaterally first diagnosed at age 4 and a dislocated shoulder at age 10 that was easily reduced. Family history is significant for several unidentified ancestors having died in their 40s of an unidentified cardiovascular disorder. Vitals are normal. Height is 6'1'' and weight 145 lb. The upper to lower segment ratio is 0.65 (decreased). Arm span is 77". The palate is highly arched. Mild pectus excavatum is present. A 2/6 early diastolic murmur is present and best heard at the second intercostal space at the right sternal border. Arachnodactyly of the fingers and toes, generalized loose jointedness, and pes planus are also present. Echocardiography revealed a tricuspid aortic valve with grade 1 out of 4 aortic regurgitation with a normal aortic root diameter. What is the recommendation for this patient?

Clear him and advise echocardiography every 6-12 months. - The finding of a low-grade of aortic regurgitation in an asymptomatic patient with a likely diagnosis of Marfan syndrome does not preclude athletic participation, but warrants follow-up for progression of aortic regurgitation and evidence of aortic root dilatation. - Low-grade aortic regurgitation in the absence of symptoms or aortic root dilation is not a reason to prohibit participation in athletics.

Acute recurrent pancreatitis (ARP) is diagnosed following at least 2 episodes of AP and 1 of the following:

Complete resolution of pain (lasting longer than 1 month) between AP episodes Complete normalization of pancreatic enzyme measurements (amylase, lipase) between AP episodes

A 4-year-old boy presents with a 5-day history of skin lesions of both lower extremities that began as reddish spots, progressed to bumps, then purplish spots. He notes a mild headache, bilateral knee pain, and loss of appetite. He had an episode of streptococcal pharyngitis 2 weeks before the rash. PMH and family history are unremarkable. Growth and development are normal; immunizations are current. Temperature is 101.2°F; vital signs otherwise normal. There is a bilateral symmetrical purpuric rash of both legs up to mid-thigh, edema to just above the ankles, and mild scrotal edema. There is no joint redness, heat, or effusion. Laboratory evaluation reveals platelet count 625,000/mm3, erythrocyte sedimentation rate 30 mm/hr, 2+ proteinuria, and no hematuria. Serum albumin, cholesterol, blood urea nitrogen, and creatinine are normal. Stool is guaiac-negative. What is the most likely outcome of this condition?

Complete resolution with significant chance of recurrence - Henoch-Schönlein purpura (HSP) is an acute IgA-mediated disseminated vasculitis affecting small vessels. - Up to 2/3 of patients will have a history of upper respiratory tract infection or other infection 1-3 weeks before presentation. - It is usually an acute self-limited illness, with most cases resolving within 8 weeks, though approximately 1/3 of patients will have one or more recurrences. - Full resolution is most likely if renal involvement is mild, there is no CNS involvement, and duration is <4-6 weeks. Prognosis is poorer if there are bloody stools or if the rash persists longer than 3 weeks.

Imaging modality of choice for confirmation of splenic injury in the setting of blunt abdominal trauma

Computed tomography with intravenous contrast

Characterized by pattern of aggression, deceit, property destruction, onset in childhood or adolescence, lasting at least 12 mos. Behavior include violation of norms, rules, rights of others, physical aggression.

Conduct disorder

17 yo with positive Hepatitis C virus Ab. Next best step in management?

Confirm Ab test with recombinant immunoblot assay (RIBA) - HCV Ab testing has 3% false-positive rate

2 yo + very severe clinical course of condition that resulted in formation of vesicles and bullae, ulcerations, crusting, hyperpigmentation, hypertrichosis in sun-exposed areas. Pink staining in diaper. Erythrodontia and hepatosplenomegaly. Hemolytic anemia. Dx?

Congenital erythropoietic porphyria - rare - manifests in infancy or first decade of life

Newborn + "dusky" appearance + O2 84% on RA. PE = cyanosis. No improvement with 100% O2. Lab = dark brown blood. Cause?

Congenital methemoglobinema - rare

Most common cause of short stature and delayed puberty in children, especially in boys. These children are otherwise healthy. A family history of this is common. Bone age is delayed, is consistent with pubertal stage, and predicts catch-up growth.

Constitutional delay - Management of constitutional delay consists of reassurance regarding future pubertal development and catch-up growth, in addition to clinical observation. - Constitutional delay, however, can cause significant psychosocial distress. - Referral to a pediatric endocrinologist for a short course of sex steroid therapy is a treatment option once a boy is 14 years old, or a girl is 13 years old, and has no or minimal pubertal development. The goal of sex steroid therapy is to facilitate pubertal progression and promote earlier initiation of the pubertal growth spurt. There is no significant effect on final height.

A 5-month-old HIV positive infant presents with a 4-day history of fever, cough, and difficulty in breathing. Physical examination reveals that his weight is 4kg. He is dyspneic with a respiratory rate of 60/min and has chest retractions. Bilateral crackles and ronchi are present. X-ray chest shows diffuse bilateral infiltrates more in the perihilar region. What is the antimicrobial of choice for this infant?

Cotrimoxazole - most likely cause of pneumonia in HIV infected infants is Pneumocystis carinii (Pneumocystis jiroveci) infection. It is the most common and is a serious opportunistic infection in these infants, commonly occurring between the ages 3-6 months. - Diagnosis can be established by bronchoalveolar lavage or lung biopsy. - Cotrimoxazole, which is a combination of Trimethoprim and Sulphamethoxazole (TMP-SMX), remains the drug of choice for treating Pneumocystis carinii (jiroveci) infection in immunocompromised children. - Treatment consists of TMP 15-20 mg/kg/day I.V. or orally for 3 weeks. For prevention, TMP is administered 5mg/kg/day on alternate days or thrice a week and continued until the patient remains immunocompromised.

A 15-year-old boy presents with a 6-month history of 3-4 loose bowel movements per day with blood and mucus and intermittent cramping abdominal pain around the umbilicus. Pain is felt more severely after meals. He frequently has loss of appetite and low-grade fever, and he has lost 2 kg over the last 6 months. Physical examination shows the patient is undernourished and has mild pallor. Oral examination shows cheilitis and stomatitis. Abdominal examination reveals tenderness in the right lower quadrant. Perianal examination shows a deep anal fissure and an anorectal fistula. Blood counts: Hb.9gm%; WBC-12,500/mm3; P 70%; L 25%; E 3%; B 2%; ESR 30 mm/hr; and elevated C reactive protein (CRP). Stool examination is negative for parasites and pathogens. Anti-Saccharomyces cerevisiae antibodies (ASCA) are positive, and perinuclear anti-neutrophil cytoplasmic autoantibodies (pANCA) are negative. Colonoscopy shows patchy areas of edema, hyperemia, and friable mucosa with strictures and fissures with normal areas between. What is the most likely diagnosis?

Crohn's disease - young adolescent presenting with features of inflammatory bowel disease (IBD), made more likely due to involvement of the oral mucosa, perianal fissure, and anorectal fistula - Investigations like elevated CRP, positive anti-Saccharomyces cerevisiae antibodies (ASCA), and negative perinuclear anti-neutrophil cytoplasmic auto-antibodies (pANCA) - Colonoscopic findings of patchy involvement of colonic mucosa and presence of strictures and fissures are typically seen

4 yo child discovered to have microscopic hematuria on dipstick during well child check when she complained of "stomach pain." She is otherwise completely healthy, growing and developing normally, and has a normal physical. Several f/u UA continues to demonstrate microscopic hematuria without casts or crystals. Urine calcium to creatinine ratio is <0.2. Most appropriate next step in evaluation?

Dip parents' urine to look for hematuria - Familial hematuria = microscopic hematuria without proteinuria - transmission = AD

2 day old newborn + rapid onset of unilateral left eye mass with associated swelling + restless + temp to 99.2 F + warm, erythematous mass over left nasolacrimal sac + mass is below medial canthal tendon + matting of eyelashes, mucopurulent discharge from punctum, and epiphora. Conjunctiva white and quiet. Dx?

Dacrocystitis - complete obstruction of nasolacrimal duct - high morbidity and mortality - IV abx

16 yo boy comes to pediatrician with concerns related to symptoms he experiences during every soccer practice. He is able to keep up with peers while playing, but at the end of practice, he experiences shortness of breath and cough. His symptoms peak appx 15 minutes after practice ends and self-resolve over the next hour. His medical hx is notable for seasonal allergies. Next best step in treating this pt's symptoms?

Daily leukotriene receptor antagonist - may be used in those who experience symptoms frequently but should monitor closely for neurobehavioral side effects - Exercise-induced bronchoconstriction (vs vocal cord dysfunction) - Symptoms 5-30 minutes after intense exercise and resolve within 60 minutes - triggered by hyperventilation - 1st-line = SABA 15-20 minutes before exercise

13 yo girl presents with HA, nasal congestion, mid-facial pain. She reports onset of sore throat and rhinorrhea 5 days ago. Over past 48 hours, she noticed green nasal discharge. PE shows: - T 99.2 F - BP 110/70 - Swollen turbinates - No adenopathy of neck Chest exam clear. What would you recommend?

Decongestants + nasal irrigation Rhinosinusitis / common cold - begin without antimicrobial tx To clinically diagnose acute bacterial sinusitis, 1 of the following criteria is required - persistent nasal discharge of any quality or daytime cough, which may be worse at night, without evidence of improvement for >/= 10 days - worsening symptoms of signs characterized by new onset of fever, HA, or increased nasal discharge after initial improvement of typical viral URI symptoms that lasted 5-6 days ("double sickening") - fever >102 F (39 C) with either purulent nasal discharge and/or facial pain concurrently for 3 or more consecutive days at the beginning of the illness in a child who seems ill

5 yo black boy presents with 2 day hx of pruritic rash that began on face, neck, and scalp before spreading to his trunk. He is otherwise well with no hx of associated systemic complaints. 3 weeks earlier, he was started on griseofulvin for tx of tinea capitis. On PE, small lichenoid skin-colored papules are noted on the scalp, neck, face, and trunk. Most likely etiology of this rash?

Dermatophytid reaction = id reaction / autoeczematization = before or during tx for inflammatory tinea capitis - type 4 delayed HS reaction to dermatophyte - lymphocytes become sensitized locally in scalp --> circulate = diffuse dermatitis - should not discontinue tx - tx = emollients and antipruritic reaction - topical or oral steroids --> taper over 2 weeks - without tx, can improve over 2-4 weeks

3 yo boy found holding open bottle of "grandpa's pills." He is taken to ED following acute onset of confusion associated with vomiting. PE incudes BP of 65/30, HR 48, multiple extra systole beats heard. T-wave inversions , short QT interval, multiple ectopic ventricular beats, short runs of atrial tachycardia noted on EKG. Blood potassium 5.5. Most appropriate management?

Digoxin-specific Fab Abs - acute ingestion of digitalis - clinically shows up 6 hours after ingestion - ectopic beats most commonly

A 17-year-old girl presents for routine evaluation. A breast examination was indicated as part of the evaluation due to the complaint of occasional tenderness of the right breast. The patient requested a female chaperone other than her mother, but no female personnel was available at that time. You are a male physician. What would you do?

Discuss alternatives to and risks of not performing the breast examination today and not perform it - The decision whether to employ a chaperone should be shared among the physician and the patient and/or the patient's parents. Considerations of patient privacy, propriety, and the physician's comfort and protection must be balanced. - When examinations of adolescents involve the genital or anorectal areas or the female breast, use of a chaperone is recommended. - If a chaperone is desired by the patient but is not available, that portion of the examination should not be conducted, alternatives should be discussed with the patient (such as return at date in the near future when a suitable chaperone will be available), the risks, if any, of not proceeding should be discussed, and the discussion should be documented in the record.

14 yo girl presents with 5-6 days of malaise, headache, sore throat, increased temp. Erythematous maculopapular morbilliform-like eruption of trunk, upper arms, and thighs, and periorbital and eyelid edema associated with petechial lesions at the junction of the hard and soft palate. Most likely cause of pt's clinical signs and symptoms ...

EBV

A 12-year-old previously healthy, developmentally normal girl is seen in the office after an emergency department visit for a first-time seizure. The previous evening her mother heard a thud and found her daughter on the bedroom floor shaking, with her eyes open and rolled back. This lasted for about 2 minutes after which she fell asleep. Her mother called 911 immediately after the shaking ended. On arrival at the emergency department, the girl's physical examination findings and laboratory results were normal, including a complete blood cell count, bedside glucose level, comprehensive metabolic panel, and serum and urine toxicology screen. She was discharged after several hours of observation. In the office, the girl and her mother report no prior history of paroxysmal events, family history of seizures or epilepsy, and no recent medication changes. The girl denies drug or alcohol use. Findings of her physical examination, including vital signs, and neurologic examination are normal Of the following, the BEST next step in this girl's diagnostic evaluation would be

EEG - is the best next step in this girl's diagnostic evaluation for new-onset seizure - seizure, which is ultimately a clinical diagnosis, but when abnormal, this test can support the diagnosis

A mother reports to the nurse that her newborn is not feeding well. He was born 28 hours ago at 39 weeks' gestation via spontaneous vaginal delivery after an uncomplicated pregnancy. Her group B Streptococcus status was positive and she received 3 doses of penicillin before delivery. The neonate's Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The neonate latched on and fed well yesterday, but has not been interested in breastfeeding today. On physical examination, his temperature is 36°C, heart rate is 145 beats/min, respiratory rate is 65 breaths/min, and blood pressure is 65/43 mm Hg. He is unresponsive. His pupils are reactive to light; anterior fontanelle is flat; heart has a regular rate and rhythm, with no murmur noted; lungs are clear with normal work of breathing. He has no hepatomegaly; +2 peripheral pulses; capillary refill 2 to 3 seconds; generalized decreased tone; limited active movement; no clonus; and decreased reflexes throughout. His glucose level on dextrose stick is 65 mg/dL (3.6 mmol/L). He is brought to the neonatal intensive care unit, where a blood culture, cerebrospinal fluid culture, arterial blood gas, and ammonia level are obtained. He is started on treatment with intravenous ampicillin, gentamicin, and acyclovir. Of the following, the BEST next test to confirm this neonate's diagnosis is

EEG - most likely seizures, which can be diagnosed with electroencephalography - neonatal seizures affect 1 to 3 per 1,000 neonates born at term gestation - most common cause is hypoxia resulting from perinatal events or cardiac dysfunction. Hypoglycemia, intracranial hemorrhage, and intracerebral infection are other important reasons for neonatal seizures. - neonatal seizures are often subtle - focal tonic clonic movement of the extremities or facial muscles is the most common presentation - eye deviation, tongue thrusting, and other facial movements are other presenting signs - the initial evaluation of neonatal seizures should include the measurement of serum glucose and electrolyte levels - diagnosis is confirmed with continuous electroencephalographic findings correlated with clinical movements concerning for seizure - because meningitis, viral or bacterial, can present with new-onset seizures, a lumbar puncture must be performed - pharmacologic treatment typically begins with phenobarbital. seizures that are difficult to control may require the addition of a second agent such as fosphenytoin

A 3-week-old female infant presents with a 1-week history of difficulty feeding, duskiness with crying, and perspiration when feeding. She was delivered via a normal, spontaneous vaginal delivery. Her postnatal course was unremarkable and newborn exam was normal. Family history of congenital cardiac disease is negative. Her physical exam is normal except for a harsh, loud, left parasternal holosystolic murmur. A systolic thrill is palpable at the apex. A chest X-ray shows cardiomegaly and increased pulmonary vascular markings. What most closely correlates with the abnormal findings?

EKG suggestive of biventricular hypertrophy - Ventricular septal defects (VSD) - Small defects are typically restrictive in nature and result in normal pulmonary pressures and no left-to-right shunting. These patients are usually asymptomatic and have a characteristic loud, harsh or blowing left holosystolic murmur heard at the left of the sternum. - Left precordial prominence, an apical thrust, and a systolic thrill may be observed. - EKG will show biventricular hypertrophy and P waves may be notched or peaked.

9 month old infant presents to clinic for rash and inability to sit up. He has diffuse scaly rash on most skin surfaces. He is unable to sit without support. He feeds without difficulty. On further questioning, his mom relates that he was initially breastfed for about 1-2 mos but was then begun on a diet exclusively consistent of skim cow's milk. He has not been interested in taking any solid foods. You suspect nutritional deficiency. What other common findings would you expect with this nutritional deficiency?

Eczematous dermatitis - classic sign of essential fatty acid deficiency - low levels of linoleic acid - decrease in arachidonic acids - reduced growth velocity, delayed dev't, scaly dermatitis, increased susceptibility to infection

9 yo girl + hx of intermittent daily fever up to 103.3 F for preceding 11 days. On day before admission, she developed swollen, warm, and tender right ankle and left knee, along with nonpruritic, erythematous, macular rash associated with pale centers on posterior trunk and extremities. Heart murmur is noted. EKG shows prolonged PR interval. Most likely to be identified during additional eval of pt?

Elevated antistreptolysin O Abs - acute rheumatic fevers = must have evidence of recent group A strep infection

8 week old with firmly attached umbilical stump. Has chronic gingivitis + FTT. Lab testing ordered. What is most likely associated with this condition?

Elevated leukocyte count in in peripheral blood Leukocyte adhesion deficiency - defect in leukocyte migration and adherence - TMP/SMX prophylaxis recommended

12 yo M + easy blistering at sites of friction + dad has similar issues with blistering on his feet when walking long distances. Dx and inheritance patternb?

Epidermolysis bullosa simplex, AD - Weber-Cockayne subtype

Clinical features of type 1 Gaucher disease include hepatomegaly, splenomegaly, pancytopenia, and skeletal disease. Bone marrow shows the characteristic storage cells of Gaucher disease. Diagnosis via bone marrow aspiration and biopsy has been largely replaced by measurement of the glucocerebrosidase enzymatic activity level in peripheral blood leukocytes. Children with Gaucher disease may have delayed growth and menarche. Medullary infarction of long bones leads to excruciating pain (bone crises). Complications include aseptic necrosis of the femoral head and spontaneous fractures. Bone marrow infiltration with Gaucher cells is seen as under tubulation in the distal femur, also called

Erlenmeyer flask deformity. - Treatment includes enzyme replacement therapy or substrate reduction therapy for type 1 and some type 3 patients.

2 medications approved by the United States Food and Drug Administration (FDA) for treatment of depression in adolescents

Escitalopram Fluoxetine - Common adverse effects of SSRIs include headaches, gastrointestinal upset, and sleep disturbance

X-linked disorder of sphingolipid metabolism characterized by periodic pain crises, angiokeratomas, corneal and lenticular opacities, stroke, left ventricular hypertrophy, sweating abnormalities, and renal disorder progressing to end-stage renal disease.

Fabry disease

4 yo girl presents with 2 day hx of subjective fever and rash. She previously had not received any childhood vaccines and returned 1 week ago from a trip to Angola. On PE, prominent postauricular and suboccipital lymphadenopathy noted. What best describes the rash most likely identified during additional evaluation of this patient?

Fine, discrete, irregular coalescing macules located on face and trunk - prominent LAD located in postauricular and suboccipital areas = rubella / German measles - rash = discrete, pinkish-red macular lesions: begin on face and spreads rapidly to trunk and LE's - small, pinkish-red spots of soft palate (Forchheimer spots) are common

EKG findings in tricuspid atresia

LVH and left axis deviation

You are evaluating a 12-year-old Caucasian boy who has been followed at a rural clinic for a history of recurrent hematomas of the thighs. Despite this history, he has remained active in athletics and has suffered 4 thigh hemorrhages severe enough to cause compartment syndrome within the past 3 years. He has no history of gingival bleeding or bleeding into joint spaces, gastrointestinal tract, or the urinary tract. He appears to bruise easily. His older sister has a similar history. Growth and development are normal. On exam, there is palpable induration overlying both quadriceps muscles. A few ecchymoses are noted at the anterior thighs. No petechiae are present. Platelet count is 220,000/mm3. Prothrombin time (PT) is 28 sec, and activated partial thromboplastin time (aPTT) is 26 sec. What is the most likely cause of his bleeding disorder?

Factor VII deficiency - autosomal recessive coagulation disorder caused by mutation of the gene coding for factor VII on chromosome 13 - wide range of severity, manifesting in milder cases as recurrent soft tissue and mucosal hemorrhages (e.g., epistaxis) and easy bruisability - more severe cases feature hemarthroses, intracranial hemorrhage, and gastrointestinal bleeding - Platelet count, aPTT and thrombin time are normal, while PT is prolonged since factor VII is part of the extrinsic pathway - Acute bleeding episodes are treated with factor VII replacement or recombinant factor VII. - Prophylactic treatment is indicated for recurrent intracranial hemorrhages and hemarthroses.

You are evaluating an 8-year-old boy at a well-child visit. Prenatal and past medical histories are unremarkable. Growth and developmental are normal. Immunizations are up to date. Examination is normal. His father notes that, because of his job responsibilities as a courier in the jewelry district, it is necessary to keep a firearm in the home. What advice would you give regarding the presence of a firearm in the home?

Firearms should be kept unloaded, with gun locks, in a locked cabinet; ammunition should be stored separately. - In the case described above, it would be counterproductive and impractical to recommend that firearms be completely removed from the home when their presence is necessary for the family's livelihood. - Under these circumstances, each firearm, unloaded, should be fitted with a gun lock, the weapons should be stored in a locked location, the ammunition should be locked away separately and the location of the keys should never be made known to the child.

Healthy adolescent, active teen. 2+ protein in urine. Everything else normal. Next step?

First morning urine protein:creatinine ratio - if no protein first thing in morning, you're good - r/o orthostatic proteinuria = spills over urine when active; none when sleep --> benign

Adolescent has femoral anteversion. Femoral version describes the relationship between the head and shaft of the femur. In femoral anteversion (ie, medial femoral torsion), the femoral shaft is internally rotated relative to the femoral head. Older children with femoral anteversion may have anterior knee pain and concerns about the impact of anteversion on their appearance and sports performance. Although femoral anteversion in children older than 10 to 12 years of age is likely to persist through adulthood, what's the management?

Families can be reassured that there is no association with impaired function, diminished athletic performance, or increased risk for hip osteoarthritis in adulthood. - Hip radiography should be performed if the child has significant knee pain, hip pain, or restricted hip range of motion - Femoral anteversion is the most common cause of in-toeing in children older than 3 years whereas internal tibial torsion is the most common cause in children younger than 3 years

A 14-year-old girl has a 2 to 3-week history of a foul smelling, blood tinged, yellow/white vaginal discharge. She did not tell her mother, who recently found her hidden stained underwear and had noted a bad odor in her room. Her periods started at 12.5 years of age and have been regular; her last period was about 3-4 weeks ago. In private, she denies any sexual activity. She has had vaginal discomfort with occasional mild dysuria. She denies any abdominal pain or diarrhea. On external exam, she has mild tenderness, erythema, and swelling of the labia minora and vulvar area. A scant malodorous purulent, blood tinged vaginal discharge is noted. Abdominal exam is without any masses or tenderness. Based on these findings, which of the following is the most likely diagnosis?

Foreign body - A malodorous purulent discharge in a young girl, especially if blood tinged, is most commonly due to a foreign body, usually toilet paper or a forgotten tampon, which may also cause vaginal ulcerations. - Vaginal bleeding is the most sensitive and specific symptom. - Removal is imperative for resolution and prevention of complications, including lacerations that could cause rectovaginal or vesicovaginal fistulas as well as stenosis from fibrotic reactions of the foreign body and ascending infection resulting in endometritis, salpingitis, uterine synechiae, pelvic adhesions and infertility. - Visualization of the vaginal vault in the young girl may be best accomplished with the patient in the knee-chest position. A foreign body may be removed using a cotton-tipped swab or tissue forceps. Saline irrigation is an excellent method of removing an object atraumatically; however, surgical removal may be necessary and requires sedation.

A 2-week-old female infant presents to your office for a post-nursery health supervision visit. The parents do not report anything remarkable or unusual about the delivery or nursery stay. The infant is alert and smiling, and generally appears to be well and healthy. Exam is notable for a 2cm mass in the center of the left clavicle. Judging from the infant's behavior during the exam, the mass is painless. There is full and active range of motion of the left upper extremity and the Moro reflex is present and symmetrical right to left. Which of the following choices represents the most likely diagnosis?

Fracture of the clavicle - The clavicle is the bone most commonly fractured during labor and delivery. - In simple greenstick fractures, there may be no abnormality except for a palpable lump, representing callus formation. - A large callus forms quickly and is generally palpable by age 1 week. Prognosis is excellent. Treatment, if any, is limited to immobilization of the arm and shoulder on the affected side. - A palpable lump in the center of the clavicle that does not change or heal with any amount of time and is characteristic of congenital pseudoarthrosis of the clavicle. The cause is unknown. There is a bony defect in mid-clavicle with palpable motion of the bony ends and a painless lump. The condition is most common on the right. It is regarded as cosmetic and no treatment is indicated.

15 yo boy + increased temp, headache, chills, generalized malaise. Ulcerated lesion noted on left leg associated with 3 tender, distinct, 2-x4 left inguinal lymph nodes. Upon further hx, it is learned he removed several ticks from his leg during recent camping trip. Most likely etiology of this pt's clinical signs and symptoms?

Francisella tularensis - ulceroglandular syndrome - maculopapular lesion at site of tick bite (ulcerates and slowly heals) - painful, acutely inflamed regional lymph nodes - tx = gentamicin, amikacin, streptomycin - mild illness tx = cipro, doxy

11 yo boy + increased proximal muscle weakness of LE's. Gait unsteadiness, decreased visual acuity, displacement of point of maximal impulse on palpation of chest. Mild scoliosis and increase in arch of both feet. Muscle mass appears to be normal for his age. Diminished deep tendon reflexes but bilateral upgoing toes (positive Babinski sign). Decreased physical activity over past few years and no longer plays with his friends as he previously did. No family hx of similar disorder. 2 older siblings appear well. With which disorder is his presentation most consistent?

Friedreich ataxia - AR

Clearance for return to participation in contact sports after concussion requires:

Full symptom resolution No symptom recurrence with high-intensity physical activity Return to baseline neurocognitive function and academic performance

A 3-year-old boy is seen for a 14-day history of intermittent, watery diarrhea without blood or mucus. The stools smell foul and appear greasy. He also has low-grade fever, anorexia, weight loss, and occasional bouts of abdominal pain and emesis. He attends daycare. He has not been exposed to pets or traveled internationally. No other family members have had diarrhea. The boy's temperature is 37°C, and his remaining vital signs are normal. His physical examination findings are significant only for evidence of mild dehydration and abdominal distension. Of the following, the MOST likely cause of this boy's illness is

Giardia duodenalis - history of prolonged diarrhea associated with weight loss, low-grade fever, and abdominal distention has a clinical picture suggestive of giardiasis - flagellated protozoan that infects the small intestine and the biliary tract - giardia cysts are the infective form of the parasite; they are viable in the environment for prolonged periods - human-to-human infection occurs via fecal-oral transmission through consumption of contaminated water or food products, or exposure to animals in petting zoos or farms - the infection cycle begins with ingestion of cysts. Excystation in the small intestine results in the replicating trophozoites, which attach to the small intestinal epithelium, causing inflammation, flattening of villi, and chronic diarrhea due to malabsorption. Encystation then occurs in the large intestine, resulting in release of infective cysts in the stool. - common risk factors for giardiasis include daycare center attendance, foreign travel, exposure to animals, and sexual transmission (ie, men having sex with men) - outbreaks of giardiasis can occur in children attending daycare centers. Community-wide outbreaks due to contaminated drinking or recreational water have been reported. Children under age 5 years and adults aged 45 to 49 years have a higher incidence of giardiasis - the disease is more common during the late summer and early fall months in the United States. - symptomatic infection may manifest as acute, watery diarrhea or chronic diarrhea with malnutrition. - giardia-associated diarrheal illness is accompanied by nausea, vomiting, abdominal cramps, malaise, loss of appetite, bloating, and mild elevations in temperature. - the stools are typically explosive, foul-smelling, and greasy in nature; the presence of blood is rare. Malnutrition due to malabsorption is a frequent occurrence in protracted illness

A 12-year-old boy's height was at 50th percentile until age 9, when it began to increase. He began to report headaches twice weekly 6 months ago. Past medical history is otherwise unremarkable. Family history is negative for tall stature. His father is 5'10", and his mother is 5'2". Development has been normal, and immunizations are current. Height is 6'1"(>95th percentile), and weight is 150 lb (>95th percentile). BMI is 19.8. Arm span is 74", and upper to lower segment ratio is 0.95. Head circumference is 58 cm (>95th percentile). The general facial appearance is somewhat coarsened, with frontal prominence and mild prognathism. Visual field examination by confrontation and funduscopic examination are normal. The fingers and toes appear somewhat thickened. Genitalia are Tanner stage II. What is the most likely diagnosis?

Gigantism - abnormal increase in linear growth to >2 standard deviations above the mean for age, sex, and Tanner stage due to increased insulin-like growth factor-1 (IGF-1) activity while the epiphyses are still open - macrocephaly (which may precede the onset of growth acceleration), coarsening of facial features, frontal bossing, prognathism, soft tissue hypertrophy, increased size of the hands and feet, thickening of the fingers and toes, headaches, visual field deficits, optic atrophy, peripheral neuropathy, and other endocrine disorders

24 hour female, born at term, has not yet passed meconium and is noted to have bilious vomiting associated with abdominal distention. Plain radiograph of abdomen shows distended loops of bowel with air-fluid levels. Contrast enema then performed, which shows normal-caliber rectum, small caliber sigmoid, and descending colon with abrupt caliber transition at splenic flexure. What is most likely to complicate this pt's early neonatal course?

Hypoglycemia - small left colon syndrome - associated with maternal diabetes - low Ca and low Mg also often seen

An 8-month-old male infant presents with a 2-week history of seizures. His mother describes the episodes as generalized stiffening and jerking; they last approximately 1 minute and occur shortly before his next scheduled feeding. Mild hypotonia was noted at his last visit, which was at 6 months of age. Past medical history is otherwise unremarkable, as is the family history. Immunizations are current. Examination reveals length at the 5th percentile and weight at the 50th percentile. The face had a rounded, cherubic appearance. The liver is palpable 5 cm below the right costal margin, with a span of 8 cm. Bilateral flank masses are palpable. Muscle tone is generally mildly decreased. Fasting blood glucose is 30 mg/dL, and serum bicarbonate is 15 meq/dL. What is the most likely cause of this child's hepatomegaly?

Glycogen storage disease type I - GSD-I, von Gierke disease, hepatorenal glycogenosis - autosomal recessive condition, is most commonly caused by deficiency of the enzyme glucose-6-phosphtase (type Ia), which converts glucose-6-phosphate (G-6-P) to glucose - results in increased conversion of G-6-P to glycogen of normal structure, causing hepatomegaly, or to lactic acid, resulting in decreased serum bicarbonate; it also results in hypoglycemia, which may lead to seizures - type Ib is due to number of mutations of various translocases of glucose-6-phosphatase - presentation is usually shortly after birth; it most commonly presents as seizures due to hypoglycemia - facial features are often described as doll-like due to increased fat deposition in the cheeks - renal enlargement occurs as well - hypotonia is usually present in infancy

Most common disorder of muscle glycogenosis. A classic feature of this is the second-wind phenomenon, where rest after a brief period of exercise-induced myalgia can improve exercise tolerance. Encouragement of good hydration with glucose-containing fluids prior to exercise can help prevent rhabdomyolysis.

Glycogen storage disease type V - also known as McArdle disease - The primary clinical feature is exercise intolerance, presenting with fatigue, pain, and cramps in the exercising muscle. Moderate exercise is usually fairly well tolerated. - Symptoms are increased during sustained aerobic (eg, stair climbing, jogging) or isometric exercise (eg, carrying weights). - Onset usually occurs in childhood, but recognition is frequently delayed, as pain and fatigue can be overlooked. - The majority of children with GSD V have minimal to no symptoms and no limitations of their day-to-day activities. - Approximately 50% of affected individuals have recurrent episodes of myoglobinuria and 25% have fixed muscle weakness. - Episodes of rhabdomyolysis can be triggered by high-intensity dynamic exercises (eg, soccer). Myoglobinuria places the child at risk of renal failure; adequate hydration is critical in these situations, and children at risk should be closely monitored. - Rarely, affected children can have difficulty with mastication, dysphagia, and oral motor function. - A classic feature of GSD V is the second-wind phenomenon, where rest after a brief period of exercise-induced myalgia can improve exercise tolerance. This increased tolerance after rest is due to the muscles' ability to metabolize free glucose and fatty acids in the bloodstream as exercise progresses. Some middle-aged adults report worsening of their symptoms accompanied by muscle wasting. - Inherited in an autosomal recessive manner. Diagnosis is made by genetic analysis with identification of 2 disease-causing changes in the PYGM gene.

Abnormal RUS. Voiding cysturethrography = reflux that reaches renal pelvis with dilation of ureter and distention of collecting system of right kidney. Grade?

Grade IV reflux - fills and grossly dilates ureter and collecting system with blunting of calyces

Only nonprescription expectorant available for use in children.

Guaifenesin - oral mucolytic that helps loosen phlegm and bronchial secretions by increasing respiratory-tract secretions, which leads to a more productive cough and better airway clearance - adverse effects associated with guaifenesin include nausea, vomiting, dizziness, drowsiness, headache, and rash - should be taken with a full glass of water, and adequate hydration during use should be maintained. Appropriate dosing for children aged up to 6 years is as follows: - age under 2 years, individualized dose (common dosing = 25-50 mg every four hours, with an MDD of 300 mg) - age 2 to 6 years, 50 to 100 mg every four hours, with an MDD of 600 mg

A 1-year-old child is being seen for her initial visit at your clinic. She and her parents have recently arrived from Nigeria and are living with relatives. Through an interpreter, her mother tells you that her daughter had a normal birth and has been fairly healthy; however, medical care was inconsistent in Africa. On further questioning, the mother reveals that she has been HIV positive for about 3 years. Her daughter was never tested. What would be the best test to perform on this child?

HIV nucleic acid detection - Maternal HIV antibody can persist in the infant for up to 18 months after delivery. Therefore, the preferred test to evaluate for HIV diagnosis in this age group is HIV nucleic acid detection (either DNA or RNA), HIV antigen, or HIV culture. If the test is positive, it should be repeated to confirm the diagnosis. = On children older than 18 months, HIV antibody testing (rapid or EIA) followed by positive confirmatory test (HIV nucleic acid detection, Western blot) is advised.

18 mo old boy presents with hx of "hoarseness" that has become progressively more pronounced over last few mos. He has coarse, muffled cry that does not change with position. His physical exam is otherwise normal. On fiberoptic laryngoscopy, a 1cm x 2.5 cm, localized, lobulated, soft tissue mass is identified on the left vocal cord. Most likely cause of this pt's findings?

HPV Type 11 - papilloma = most common type of respiratory tract neoplasm in children - recurrent respiratory pappilomatosis = most often via HPV 6 & 11 = via vertical transmission during delivery - dx = most common between 2&3 yo (usually before 5 yo) - multiple endoscopic procedures may be necessary since lesion recur

obesity medication treatment options for children

orlistat approved for >12 yo phentermine for >16 yo (short term) saxenda (approved for 12-17 yo in US)

26 mo old girl presents to ED with 2 day hx of "coughing and wheezing." Last several weeks, she has become "short of breath" with only minimal activity, and over the last couple of months has experienced progressive hoarseness. Past medical hx positive for recurrent episodes of wheezing usually associated with upper respiratory infections. She is afebrile. RR of 38. Breathing is labored, and she has biphasic stridor. She has mild, clear nasal discharge, is not drooling, and and is well hydrated. Radiograph of neck shows soft tissue irregularity at vocal cord level without narrowing of subglottic space. Most likely cause of the patient's findings?

HPV type 6 juvenile laryngeal papillomatosis - warty lesions often via HPV 6 and 11 - can be transmitted during vaginal delivery - onset of stridor usually preceded by hoarseness, which gradually worsens over time - dx via direct laryngoscopy and biopsy

During PPE, 12 yo boy noted to have axillary freckling and numerous cafe au lait spots that are >/= 18 mm in diameter. What physical exam finding is most likely to be additionally present?

Hamartomas of iris - NF1 - AD

Mutations in SCLC6A19 cause ...

Hartnup dz - ataxia - niacin def - AA disorder

Child has a new, symptomatic complete heart block. Additionally, he reports recent travel to New England. While not diagnostic, this raises suspicion for Lyme carditis. Next steps?

He should be admitted to the hospital for cardiac monitoring, given his symptomatic cardiac condition. While undergoing evaluation for the cause of his heart block, the boy should be started on intravenous (IV) therapy for possible Lyme disease. While there are several appropriate antibiotic choices to treat Lyme disease, including oral doxycycline and amoxicillin-clavulanic acid, Red Book® recommends IV ceftriaxone for those admitted to the hospital.

An 8-year-old boy presents for a health maintenance visit; there are no acute problems. Past medical history is unremarkable, except for a simple febrile seizure at age 4. His 45-year-old father has been treated for angina pectoris and hypertension since age 40. His total cholesterol has been as high as 320 mg/dL; he is currently being treated with a statin. The boy's development has been normal, with As and Bs in school. He participates in organized sports 4 times per week. Height is at the 75th percentile and weight is at the 50th percentile. Vital signs are normal, as is the remainder of the examination. His parents ask whether it is appropriate to perform lipid screening. What advice should be given to his parents?

He should have a fasting lipid profile. - The recommendation for universal lipid screening is between ages 9 and 11 and again between ages 17 and 21. - Targeted screening is also recommended beginning at age 2 for children with certain risk factors: family history in parents, grandparents, aunts, or uncles with premature cardiovascular disease (at age ≤55 for male relatives and at age ≤65 for female relatives); a history of myocardial infarction; angina pectoris; coronary artery bypass grafting; percutaneous coronary artery intervention; or sudden cardiac death. Another risk factor is having a parent with a total cholesterol level ≥240 mg/dL or with a known history of dyslipidemia; unknown family history is also a reason for early screening. - Indications for targeted screening include overweight status, BMI >95th percentile between ages 2 and 8, BMI >85th percentile between 12 and 16, hypertension, diabetes mellitus, cigarette smoking, and certain other medical conditions (e.g., chronic renal disease, renal transplant, nephrotic syndrome, heart transplant, Kawasaki disease, chronic inflammatory disease, and HIV positive status). - For universal screening, a non-fasting non-HDL cholesterol level is recommended. If this result is abnormal—or for targeted screening—a fasting lipid profile is recommended. - This patient should have a targeted screening using a fasting lipid profile due to the family history of premature cardiovascular disease and total cholesterol >240 mg/dL. - Even in universal screening, non-fasting non-HDL cholesterol level is used, not non-fasting total cholesterol or triglycerides.

4 yo boy + 3 day hx of rash. Mom reports similar episode ~ 1 year ago. Symmetrically distributed, dull-red macules and large round wheals, many of which contain small central vesicle. Various lesions have prominent central duskiness. Lesions are generalized but most prominent over palms and soles, backs of hands and feet, and extensor surfaces of arms and legs. Condition most likely precipitated by...

Herpes simplex type 1 - erythema multiforme - 5-7 days before fading

18 yo male presents to ED for syncopal episode. Increased fatigue, SOB soon after beginning exercise, several recent episodes of hemoptysis. Clubbing and mild cyanosis present on PE. Right ventricular heave noted in association with loud pulmonic component of 2nd heart sound. Soft systolic murmur along left sternal border noted. Moderate cardiac enlargement, enlargement of pulmonary vessels in hilar areas , and relative pulmonary undervascularity in outer 2/3 of lung fields noted on CXR. What best describes pathophysiology of pt's clinical and radiographic findings?

High pulmonary vascular resistance - Eisenmenger syndrome via unrepaired cardiac defect - SOB, decreased exercise intolerance, lethargy, fatigue, hemoptysis, syncopal episodes, dysrhthmias

2 yo girl + IUGR at birth, FTT, dev'tal delay presents for evaluation. Ht, wt, and HC all below 3rd %ile. Facial features include low hairline; long, thin lashes; long philthrum associated with downturned upper lip, micrognathia. Hands are small, with a hypoplastic proximally shaped thumb, short 5th finger, mild clindodactyly. Most likely to be identified during additional evaluation?

Hirsutism - Cornelia de Lange syndrome via sporadic new autosomal dominant mutation - phocomelia may occur - cognitive impairment

Teen + fever, cough, nonpleuritic chest pain following month-long excursion exploring caves in Ohio Valley. CXR shows hilar adenopathy + focal alveolar infiltrates. What fungal infection?

Histoplasmosis - most common pulmonary mycosis in humans - south and midwestern - MS and Ohio Valleys

Catabolic pathway of lysine as well as tryptophan and hydroxyleucine is disrupted in glutaric acidemia (glutaric aciduria) type I. However increased amounts of glutaric and 3-hydroxyglutaric aciduria are seen in urine. Tx is with...

L-carnitine riboflavin special diet

An 8-year-old boy presents with a 15-day history of a low-grade continuous fever. He has decreased appetite and significant lethargy. On clinical exam, he is found to have gross pallor, significant bilateral cervical lymphadenopathy, right axillary apical group adenopathy, and two ecchymotic lesions over the abdomen and thigh. The liver is palpable 5.5 cm below the right costal margin, and the spleen is palpable 6 cm below the left costal margin. On auscultation, there is an ejection systolic murmur, grade 3/6 in the pulmonary area. There is no neurological deficit or any bony tenderness. A peripheral smear revealed a few blasts, and a bone marrow aspiration is done on which a diagnosis of ALL-L2 is made. He is started on chemotherapy. During the first week, the child was afebrile and tolerated the chemotherapy well. During the second week, he received his usual dose of vincristine and was monitored thereafter. 8 hours later, the child began speaking irrelevantly and had a generalized tonic clonic seizure lasting 1 minute. A 0.3 mg/kg injection of diazepam was given and the child was moved to the ICU. His blood pressure was normal, and examination of the fundus was normal. In anticipat

Hyponatremia and hypokalemia - Hyponatremia is a common side effect of vincristine, a drug used as part of a chemotherapeutic regimen that inhibits cell duplication by bonding to the protein tubulin. Hyponatremia (serum sodium values <100 mEq/L) can cause seizures. - Hypokalemia can be caused by lack of nutrition and can occur alongside hyponatremia. Chemotherapy often causes fluid retention, which can also contribute to hypokalemia. - Syndrome of inappropriate anti-diuretic hormone (SIADH) secretion can result in hyponatremia with low serum osmolality, high urine osmolality, and low urine volume. It can be caused by drugs like vincristine in some children and from direct insults to the brain, such as meningitis, encephalitis, brain tumors and abscesses, CNS leukemia, subarachnoid hemorrhages, and GB syndrome. = The classic constellation of laboratory findings consists of low values for serum sodium, potassium, chloride, and osmolality; serum bicarbonate levels may be normal with a high urine osmolality (250-1400 mOsm/kg) and low uric acid levels.

For children 4 years and older with moderate to severe persistent asthma, the panel now recommends single maintenance and reliever therapy (SMART) using

ICS-formoterol for both daily and rescue use - instead of either daily dose ICS with reliever SABA or daily ICS-long-acting beta2-agonist (LABA) with reliever SABA - The recommended LABA for this approach is formoterol because of its rapid onset of action and more frequently allowable dosing. - For example, children who qualify for SMART can use one to two puffs of ICS-formoterol once or twice daily as maintenance, with one to two additional puffs as needed for asthma symptoms. The daily maximum is eight puffs (36 micrograms [mcg] formoterol) for children ages 4-11 years and 12 puffs (54 mcg formoterol) for children 12 years and older.

14 yo F + began menstruating 8 mos ago + breast and areolar enlargement present with no contour separation of areola. Pubic hair dark. Tanner stage?

III - breasts more elevated and extend beyond borders of areola - pubic hair more coarse and curly

14 yo M + ICU for tylenol overdose + started on N-acetylcystie immediately. 7 days into stay, UOP decreases a lot + high BP + super elevated ALT and AST. Next step?

IV albumin - hepatorenal syndrome - IV albumin 1x daily for 2 days --> r/o hypovolemic renal failure - UOP will not improve with hepatorenal syndrome

2 wk old M infant + vomiting and poor weight gain. PE = scrotal hyperpigmentation. Labs = hyponatremia, hyperkalemia, metabolic acidosis. Glucose = 40. Next step?

IV bolus of 5% dextrose in NS - Classic CAH via 21 hydroxylase deficiency

Pts with digoxin toxicity present with cardiac arrhythmias and hyperkalemia. What is given to all pts with K >5, life threatening arrhythmias, or end-organ dysfunction?

IV digibind (Fab) admin

Pts with Ca channel blocker intoxication (ex nifedipine) primarily present with hypotension and bradycardia. Often have preserved mental status. Traditional tx ...

IV fluid resuscitation and atropine - next step = IV calcium salts

Teen with IBD. Likely Crohns (granulomas). Initial tx of both UC and Crohns =

IV steroids - to initially reduce inflammation - initial tx --> followed by other IBD therapies

ITP is supported by the low platelet count with large (increased mean platelet volume, mean corpuscular volume) platelets, normal white blood cell count and differential, and the fact that the child is otherwise well with no lymphadenopathy or hepatosplenomegaly. This diagnosis is one of exclusion. ITP is an immune phenomenon. In most cases, there is a history of recent viral illness. Autoantibodies created toward a glycoprotein on platelets lead to their destruction and clearance, mainly by the spleen and also the liver. Though platelet antibody testing is available, a negative antibody test result does not exclude the diagnosis. For an infant, who developmentally has a significant risk of head injury, it would be advantageous to administer a therapy that would raise the platelet count quickly and thereby decrease the bleeding risk. What is the best choice?

IVIG - may increase the platelet count rapidly, and its response can last for several weeks - Because ITP is an immune phenomenon, the autoantibodies could destroy any platelets administered; therefore, platelet transfusions are reserved for life-threatening bleeding episodes - Age, developmental stage, and bleeding signs and symptoms should be considered when choosing a treatment choice for acute idiopathic thrombocytopenic purpura

Examination of short stature usually requires minimal laboratory tests that include thyroid function tests. In a young girl, symptoms and laboratory test results suggest hypothyroidism. An increase in the serum level of TSH is considered the most sensitive test of thyroid dysfunction. What is the preferred treatment?

L-thyroxine sodium

18 yo freshman + "lump" in her left breast she discovered while showering. Dull, persistent pain several days in duration. Otherwise well, not sexually active. Took OCP's several years ago to treat dysmenorrhea and heavy menstrual bleeding but has not done so for the past year. Regularly has menstrual cycle every 28-30 days; last period for 4 weeks ago. On PE, there is a slightly tender, cystlike mobile mass associated with fibrotic tissue palpated in upper outer quadrant of left breast. Most appropriate initial tx in this pt?

Ibuprofen with f/u in 1-2 mos = fibrocystic breast changes - initial tx = NSAIDs in premenstrual period - imbalance between estrogen and progesterone - no increased risk of breast cancer - often resolve after 1-2 cycles - most often in left outer quadrant of breast - OCPs improve this in majority of women - limiting caffeine may provide some relief

A 4-year-old girl presents with a 1-week history of fever of 39.0°C, sore throat, and fatigue. No one else has been sick at home. On exam, her temperature is 39.3°C, she has bilaterally enlarged and tender posterior cervical nodes, and her pharynx is erythematous with scant grayish exudates. The rest of the exam is normal. Lab tests reveal a total white blood count of 14,000 cells/cmm with differential count as follows: 26% polys, 66% lymphocytes, 8% monocytes, and several atypical lymphocytes on peripheral smear. Her heterophile antibody test is negative, and her mother wants another test done to confirm your suspicions of infectious mononucleosis. What serologic test would be most valuable?

IgM - VCA - present in more than 80% of acute sera and is the most valuable serologic test - antigen is produced at the time of the acute infection and persists for weeks to months, although it can be present in low titer for 1 year, and does not reappear

A 7-year-old boy presents with pain and photophobia after his older sibling accidentally poked her finger in his right eye two hours prior. He has otherwise been well. On examination, he has increased tearing and redness of his right eye; no foreign object is visible. A dark red fluid level is seen at the lower 1/2 of the iris. What is the most appropriate next step?

Immediate referral to an ophthalmologist - A traumatic hyphema (the presence of blood in the anterior chamber) - can occur with either blunt or penetrating trauma - appears as a bright red or dark red fluid level between the cornea and the iris, or as a diffuse murkiness of the aqueous humor - pain is a prominent symptom - ophthalmologic consultation should be sought. Treatment - bed rest with elevation to allow settling and resorption of the blood - Topical mydriatics, miotics, or oral aminocaproic acid are occasionally used - Patching of the affected eye may also be done

What extremities are weaker in SMA?

LE weaker than UE

An initial evaluation for precocious puberty includes

LH, FSH, thyroid-stimulating hormone estradiol in girls testosterone in boys bone age radiograph

15 yo girl presents with 24 hour hx of increasingly severe lower abdominal and back pain and progressive weakness of both lower extremities, which she states are "tingling and numb." She had a "bad cold" several weeks earlier. On PE, muscles of legs are noted to be weak and flaccid with decreased sensation documented below the umbilicus. Most likely to be identified on further eval?

Inflammation and edema of spinal cord at T10 documented on MRI - transverse myelitis - abrupt onset of progressive weakness - sensory abnormalities in LE's

There is currently no monkeypox vaccine available for administration to all children. However, there is a vaccine available to children < 18 years who have been exposed to monkeypox. What is it?

JYNNEOS vaccine - may be recommended for children <18 years of age for post-exposure prophylaxis under a single patient expanded access investigational new drug (IND) protocol through CDC. - Clinicians should discuss use of vaccine in a child as post-exposure prophylaxis with the state or local health department and CDC. https://www.fda.gov/drugs/investigational-new-drug-ind-application/ind-applications-clinical-treatment-expanded-access-overview

9 yo boy complains of pain in his feet when playing outside. No hx of associated trauma. His parents state that this has been a recurrent problem and sometimes he has painful cracks in his skin. On PE, skin on distal portions of soles and toes appear smooth and shiny "glazed" with associated scaling. Severe painful fissures noted. Interdigital spaces spared. Most likely cause of presenting signs and symptoms?

Juvenile plantar dermatosis - recurrent flares - painful hyperkeratosis - often limited motility - via repeated wetting and drying of feet related to sweating and occlusive footwear - tx = white cotton socks and avoid occlusive footwear; emollients when shoes and socks removed; medium-potency steroids - often have spontaneous resolution with puberty

Should be suspected in a young child (typically ages 1-7 years) with intermittent and painless rectal bleeding.

Juvenile polyp(s) - Most juvenile polyps are found in the distal colon (sigmoid and rectum); once resected, follow-up colonoscopy is not indicated. - However, if more than 5 polyps are detected, or if the location of the polyps is in the proximal colon, juvenile polyposis syndrome should be suspected. - Evaluation should include genetics and oncology consultation, and repeat colonoscopy every 1 to 3 years.

17 yo F + constipation and body pain + bones hurt + worsening constipation for past 3 mos + kidney stones several mos ago that passed and resolved spontaneously. PE = nodule near left inferior pole of thyroid. Labs - calcium: - phosphate: - parathyroid hormone:

Labs - calcium: increased - phosphate: decreased - parathyroid hormone: increased primary hyperparathyroidism - parathyroid adenoma - hypercalcemia and hypophosphatemia

Associated with retinitis pigmentosa, obesity, dev'tal disabilities, polydactyly, genital hypoplasia, hypogonadism...

Laurence-Moon-Biedl/Bardet-Biedl syndrome

5 yo M + hearing concern + testing with tuning fork. Placed in middle of forehead. Hears it louder on right. When placed next to each ear, he again hears it louder on the right. Most likely etiology of hearing loss?

Left-sided sensorineural hearing loss - With sensorineural hearing loss, both Weber and air conduction lateral to unaffected side

Leg length discrepancies are common and usually asymptomatic. Leg length discrepancies should be monitored for progression during linear growth. What leg length discrepancies should warrant evaluation for possible surgical intervention?

Leg length discrepancies greater than 2 cm

Functional hypothalamic amenorrhea is due to relative energy deficiency in sport. AKA female athlete triad. What hormonal changes do you find?

Low LH and FSH Estrogen deficiency - hx of menstrual dysfunction = earliest finding

9 yo boy brought to ED because "something is wrong with his face." No hx of trauma or systemic symptoms. Unable to wrinkle his forehead on left and has also lost the ability to close his left eye and raise the left corner of his mouth. Decreased nasolabial fold prominence on left is also noted. What is the cause of this patient's findings?

Lower motor neuron dysfunction of left 7th cranial nerve - lower/peripheral motor neuron = unable to wrinkle forehead, close eye, raised corner of mouth on affected side

A newborn male presents with what appears to be difficulty breathing and seems to choke and gag upon feeding. The baby starts to suck, then seems to become agitated and starts to choke. The mother states pregnancy was without complications and labor progressed rapidly. The birth weight was 8 lbs 10 ounces with Apgar scores of 7 at one minute and 8 at five minutes. The baby required some suctioning and stimulation in the delivery room. Upon examination, the baby is resting quietly. The face appears to have some bruising and the nose appears to be deviated slightly to the left. You attempt to look in the nose, but cannot see much other than edema of the mucosa. The oropharynx appears normal. The lungs are clear, and the examination of the heart reveals a regular rhythm with no murmurs. The finger tips are slightly dusky, but the remainder of the examination is normal. With the assistance of the nurse, you attempt to pass a catheter through the nares. However, you are unable to advance it very far into the nose. You consult with the pediatric otolarngologist and then order a CT scan. The results of the scan confirmed the diagnosis of congenital nasal pyriform aperture stenosis. The otolaryn

MRI of the brain - Congenital nasal pyriform aperture stenosis (CNPAS) - uncommon disorder presenting with respiratory distress after birth - symptoms may mimic those of choanal atresia - CT scan may demonstrate a single central maxillary incisor - It can occur as an isolated anomaly, or it can be associated with a dental anomaly, most commonly solitary central maxillary incisor, craniofacial, ophthalmological, or central nervous system malformation - It is thought that the association with a single central maxillary incisor may be an autosomal dominant microform form of holoprosencephaly, have increased incidence of endocrine abnormalities, and should have an MRI of the brain - Associated endocrinopathies include anterior pituitary dysfunction or absence, growth hormone deficiency, short stature unrelated to growth hormone deficiency

Irritable 7 week old male noted to have tender, slightly warm and edematous periumbilical erythema associated with several distinct areas of ecchymosis and necrosis without evidence of purulent discharge. Umbilical cord remains firmly attached. Clinical findings most likely associated with what lab findings?

Marked neutrophilia - Leukocyte adhesion deficiency Type 1 (LAD1) - AR - recurrent and often severe bacterial and fungal infections in absence of pus formation - elevated circulating neutrophils - increased risk of staph and E coli - recurrent perianal and facial cellulitis = common - death by 2-5 yo unless stem cell transplant

3 yo + hx of menstrual bleeding. Sexual maturity rating Stage 1 breast dev't and SMR 1 pubic hair. Rest of her exam is nml except for large cafe au lait spot on her chest. Most likely reason for her menstrual bleeding?

McCune Albright syndrome - triad = cafe au lait skin pigmentation, polyostotic fibrous dysplasia, autonomic endocrine hyperfunction - via missense mutation in gene coding for alpha subunit of stimulatory G protein - isolated menstrual bleeding before any signs of precocious puberty = classic

A 12-year-old boy presents for a general physical exam. He is generally in good health and reports no issues. His height is 156 cm, which has increased 8 cm since his physical last year. Observation of the back with the patient standing reveals an S-shaped curve in the spine, with the shoulders displaced 4 cm to the left of the hips. The posterior superior iliac crests are not level, the right being 3 cm higher than the left. What is the most appropriate next step in the evaluation of this patient?

Measurement of leg lengths in supine position - The combination of a tilted pelvis and an opposite-direction alteration in spinal alignment is characteristic of leg length discrepancy with compensatory scoliosis. - Common during the adolescent growth spurt, it produces a tilt in the pelvis toward the short side, a compensatory curve in the spine, concave toward the long side, and a shift of the shoulders and upper body toward the short side. - Measurement of both legs in the supine position from anterior superior iliac crest to lateral malleolus will confirm the discrepancy. - Returning the patient to the standing position and placing a lift equal to the length discrepancy under the foot on the short side should eliminate the compensatory scoliosis.

15 yo M + 1 day of gross hematuria + sore throat 2 days earlier + eyes are puffy + BP elevated + UA = RBC casts, 1+ protein. Kidney biopsy planned. What is on histology?

Mesangial hyperproliferation with strongly positive IgA immunofluorescent staining - nephritic syndrome + sore throat within 48=72 hours = IgA nephropathy

An increased anion gap metabolic acidosis has a limited number of causes. The mnemonic "MUDPILES" helps to identify the common causes of an anion gap metabolic acidosis...

Methanol Uremia Diabetic ketoacidosis Paraldehyde Iron/isoniazid Lactic acid Ethanol/ethylene glycol Salicylates

Child with Entamoeba histolytica infection. These cysts are usually found in the lumen of the intestine, but they may also cause extraintestinal abscesses. Liver abscesses are the most common extraintestinal complication. Abscesses can extend or rupture into the lung, pleural or pericardial cavity, or peritoneum. Most appropriate drug therapy for this patient?

Metronidazole plus iodoquinol - requires treatment with a luminal agent that will target the cysts as well as another medication that will treat the liver abscesses - alternative luminal agent to iodoquinol is paromomycin - metronidazole is the most commonly used drug to treat amebic liver abscesses - other options include imidazole or ornidazole

A mother is wanting to breastfeed her newborn infant who was born at 33 weeks gestation. Mom is on lamotrigine for a seizure disorder and took the recommended dose of 4 mg/day of folic acid prior to and during pregnancy. Baby is doing well with no signs of neural tube defect. Mom is vegan and has had breast implants. What is the correct advice for this mom?

Mom needs to be taking a B12 supplement

A 13-year-old girl with known von Willebrand disease has recently begun menstruating. Her periods have been extensive and very heavy. Her mother inquires about the use of oral contraceptives to control her bleeding and provide future birth control. The patient denies any sexual activity, but she does have a boyfriend. What contraceptive choice would be best suited for this patient?

Monophasic ethinyl estradiol/levonorgestrel (Seasonale) - extended-cycle regimen - provides 84 days of continuous hormonally active pills, followed by 7 days of placebo - regimen may be particularly appropriate for adolescents with certain medical conditions, including anemia, severe dysmenorrhea, endometriosis, dysfunctional uterine bleeding, or von Willebrand and other bleeding diatheses

The American Academy of Pediatrics recommends pediatrician visits how often for children in foster care?

Monthly until age 6 months Every 3 months from age 6 to 24 months Every 6 months until age 21 years - Children entering foster care should be seen by a pediatrician within 72 hours for a complete physical examination and within 1 month of placement for assessment of the child's health care needs

4 yo with trisomy 21 presents to ED with 3rd seizure in past 4 mos. He appears to be in postictal state, and his initial neuro exam is concerning for lack of withdrawal of left upper extremity upon painful stimulation. After 48 hours, he is back to his baseline, except that he is unable to move his left upper extremity. No hx of trauma, and plain films of extremity are normal. Cranial CT shows hypodensity in right cerebral hemisphere. Additional imaging in this pt most likely to reveal what disease process?

Moyamoya disease - hypodensity on CT = stroke - Moyamoya associated with Trisomy 21, etc

18 month old girl referred to cardiology for echo d/t recently identified systolic murmur. She is taking meds for chronic rhinitis that has been attributed to seasonal allergies. She has myringotomy tubes placed d/y chronic otitis media and conductive hearing loss. Her parents report that she met her early milestones appropriately, but they feel her dev'tal progression is now slower than that of other children her age. She is not yet walking and has several distinct words. Her ht and wt are at 25th %ile. Her HC is >97 %ile. Anterior fontanelle has not yet closed and is large. Lips and cheeks are full. Mild contractures of fingers. Moderate-size umbilical hernia. Echo with thick mitral valve with insufficiency. Most likely etiology of child's features ...

Mucopolysaccharidosis Type I - AKA Hurler syndrome - coarse facial features, frequent URIs, macrocephaly, hernias, thickened heart valves

What are the diagnostic criteria for stage 1, pre-symptomatic type 1 diabetes (T1D)?

Multiple islet autoantibodies and normoglycemia - The American Diabetes Association developed staging of diabetes with the recognition that the disease starts not as a metabolic disease, but as an autoimmune disease with presence of multiple islet autoantibodies and normoglycemia (stage 1). - Then it progresses to stage 2 with dysglycemia or relatively mild abnormalities of blood glucose observed sometimes in fasting state, but more often after meals.

Boy has microcytic anemia and peripheral eosinophilia. These findings and his history of barefoot outdoor play in rural Indonesia are highly suggestive of what infection?

Necator americanus (hookworm) - transmitted through soil in endemic regions - prevalent in North America, South America, Central Africa, parts of India, South Pacific Islands, and Indonesia - Ancylostoma duodenale, another important hookworm, is endemic in the Mediterranean region, Northern Asia, and areas of South America - The diagnosis of hookworm is made by visualization of its eggs on microscopy. The eggs are 57-76 µm × 35-47 µm in size, have an oval or ellipsoidal shape, and have a thin shell - Treatment choices for hookworm include albendazole, mebendazole, and pyrantel pamoate - The period from skin exposure to development of noncutaneous symptoms is 4 to 12 weeks

Most common extracranial solid tumor malignancy in childhood. Usually presents within first 5 years of life.

Neuroblastoma - most frequently arises in adrenal gland and retroperitoneum - bony mets common --> pain and irritability - periorbital mets = common --> raccoon eyes - tumor sometimes secretes VIP --> diarrhea - calcification within tumor on CT

Mother calls your office concerned her child may have diabetes. Child is 2 years old with hx of increased thirst but no increased UOP. Her weight is stable, and her appetite is normal. She is acting normal. Child's grandmother has type 2 diabetes. Child's mother checked her glucose 2 hours after lunch (using her grandmother's meter) to find it was 139. Most appropriate next step in management?

Reassure and educate the mother about diagnosis and presentation of diabetes Diabetes criteria - fasting glucose >126 - classic symptoms of hyperglycemia + plasma glucose >200 - 2-hour plasma glucose >200 during standard OGTT - A1C >6.5

A 4-year-old girl presents with a 2-day history of a rash. Prior to the onset of the rash, she had been febrile with malaise and tenderness of the skin. She had previously been well, with no significant past medical history. Her only medication is acetaminophen. On examination, her temperature is 101.2° F, and her blood pressure, respiratory rate, and heart rate are within normal limits. Skin exam reveals a diffuse scarlatiniform erythema over her entire body, with accentuation around her mouth and the periorbital region. She has crusting and fissuring about her eyes, mouth, and nose. The mucous membranes do not appear to be involved. She also has some thin-walled blisters on her cheeks, the flexor area of the neck, and her groin. The remainder of her exam is normal, with the exception of a general irritability. What most closely correlates with the abnormal physical examination findings?

Nikolsky's sign - Staphylococcal scalded-skin syndrome presents with a prodrome of fever, irritability, malaise, and tenderness of the skin. - A scarlatiniform erythema develops, most prominently on the face, the flexor area of the neck, the axillae, and the groin. - Facial edema develops, with crusting about the eyes and mouth and sparing of the mucous membranes. - Nikolsky's sign (desquamation of the underlying skin with gentle rubbing) often occurs; sterile bullae are seen in more severe cases. - The syndrome is caused predominantly by phage Group 2 staphylococci. - Treatment consists of systemic antibiotics, local skin care, and fluid and electrolyte monitoring.

A 12-year-old boy with HIV presents with a 1-month history of progressive confusion, lethargy, and memory loss. CT scan of the head reveals an isolated hypodense cerebral mass. Histologic examination reveals blast cells. There is no evidence of metastases. What is the most likely tumor type?

Non-Hodgkin's Lymphoma - Isolated primary Non-Hodgkin's lymphoma is the most common central nervous system tumor in pediatric patients with HIV infection. - Neuroimaging is the most important diagnostic test for defining a suspected brain tumor, MRI with contrast being the more sensitive and specific.

7 yo M + persistent vomiting, abdominal pain. Abdominal x-ray = air-fluid level in dilated duodenal loop. Abd US = narrowing of duodenal lumen and thickening of duodenal wall. Most likely cause of these symptoms?

Non-accidental trauma - duodenal injury = synonymous with child abuse in kids < 2 yo with no hx of serious abdominal trauma

First stage of surgical repair for hypoplastic left heart syndrome ...

Norwood procedure - performed in 1st week of life

A 14-year-old girl presents 6 weeks after a visit for fatigue, weight gain, cold sensitivity, and declining school performance. 3 weeks after the initial visit, she was diagnosed by pediatric endocrinology with hypothyroidism; she is being treated with thyroid hormone replacement. At the initial visit, a T3, T4, and TSH were ordered, but there was no follow-up on the results. The results were available 2 days after the initial visit, and the TSH was significantly elevated at 15 mIU/L; her physician has seen this patient on two subsequent occasions for acute care visits and has written notes for those visits. What is the best next step?

Note the test results and notify the physician's professional liability carrier - This physician did not follow up on the results of tests ordered. Any attempt to recreate a note will only worsen the matter and appear as an admission of negligence. Such recreations can also be detected by various forensic techniques. It has not been established that failure to follow up on the abnormal test results caused a delay in the diagnosis of hypothyroidism or, if it did, if the delay altered the outcome.

You are evaluating a 3-year-old African-American boy at a well-child visit. Prenatal and past medical histories are unremarkable. Growth and developmental are normal. Immunizations are up to date. Examination is normal. When inquiring about living arrangements, the child's mother tells you that they live in a 3rd story apartment of an older building that has exterior fire escapes accessible by the windows. What should you advise regarding the windows?

Operable window guards should be installed on all windows - The leading cause of childhood injuries is a fall; they are rarely fatal. Childhood falls that are fatal usually occur from a height greater than 2 stories (approximately 22 feet). Falls from windows from the 2nd story or higher are still a serious problem in older buildings. - Operable window guards should be placed on all windows so that exit by a child is prevented, but exit under adult supervision in case of emergency, such as fire, can be readily accomplished.

A 6-week-old male infant is brought to your office with history of jaundice that started at the age of 1 week. He has been passing dark-colored urine and pale stools for the same duration. He was born at term, birth weight 2.8 kg. He currently weighs 4 kg. He has moderate icterus. Abdominal examination reveals liver palpable 6 cm below costal margin. Spleen is palpable 3 cm below costal margin. Both are firm in consistency. There is no free fluid. The rest of the examination is essentially normal. Serum bilirubin total is 15 mg; direct is 8 mg. What is the investigation of choice for establishing the diagnosis?

Operative cholangiography - clinical features of the infant (jaundice, dark-yellow-colored urine, pale stools, hepatosplenomegaly, conjugated hyperbilirubinemia) are highly suggestive of biliary obstruction - can be intrahepatic—commonly due to neonatal hepatitis—or extrahepatic—due to extrahepatic biliary atresia - cholangiography is direct visualization of the intrahepatic and extrahepatic biliary tract to evaluate the cause, location, and extent of biliary obstruction. It provides definitive information to guide the decision of whether operation is indicated or not. - Management consists of hepatoportoenterostomy (Kasai procedure). The success rate of Kasai operation is much higher if performed before age 8 weeks.

7 yo boy + uncomfortable pruritic rash that began a few days earlier during a family vacation at the beach. On PE, several erythematous papules are noted on dorsal surface of left foot and lateral malleolus. Brownish-red, palpable, well demarcated, serpiginous plaques extend from each papule. These plaques have continuously lengthened since first observed. Most appropriate tx in this pt?

Oral albendazole - cutaneous larva migrans - creeping eruption - via larval stages of dog and cat hookworms - tx = oral anthelmintic agent albendazole ~ 200-400 mg as single dose for 3 days

4 yo F + fever, sore throat, malaise. PE = nontender cervical LAD and posterior pharyngeal aphthous ulcers. Labs = WBC 12,000 and slightly elevated ESR. Rapid strep negative. Throat culture normal. Hx of recurrent fever, sore throat, negative throat culture (4 similar episodes in last year). Tx most likely to result in symptom resolution?

Oral prednisone 2 mg/kg once - PFAPA - occurs before 5 yo and usually resolves with age - typically aborted by single dose of oral prednisone taken once at beginning of attack

Recommended treatment for an initial episode of severe C difficile colitis

Oral vancomycin (40 mg/kg per day) every 6 hours for 10 days

7 yo M + recently adopted from Asian refugee camp + initial visit + Hx unknown + vaccine records incomplete + received only 1 dose of hepatitis B at birth + PE unremarkable. With respect to hepatitis IZZ, what is the best approach?

Order serological tests for HBsAg, anti-HBs, and anti-HBc - all refugees from areas with endemic hep B infection (Asian and Africa) should be screened for hepatitis B with serologic tests

14 yo M + rt ankle pain + pain started 3 mos ago + wakes up at night with sharp sensation in anterior aspect of right ankle. Ankle support minimally beneficial. Pain with running, jumping, basketball. Does not recall trauma to area. Ibuprofen helps reduce pain. X-ray = 6x7 mm area of increased cortical thickening and central nidus involving anterior talus; overlying cortex is intact. Dx?

Osteoid osteoma - benign tumor derived from osteoblasts - mostly boys in second decade of life - femur, tibia, spine - bone pain and swelling worse at night - responds to NSAIDs - well circumscribed radiolucent central core/ nidus surrounded by medullary sclerosis and cortical thickening - tx = observation or surgical resection

A 9-month-old infant presents with delayed milestones. Physical examination reveals brachycephaly, hypertelorism, mongoloid slant of eyes, and epicanthic folds. Mouth remains open with protruding tongue, and Brushfield spots are seen in the iris. Hands show a single transverse crease (Simian crease) with clinodactyly of the little finger. Examination of cardiovascular system reveals an accentuated first heart sound, wide and fixed splitting of the second heart sound, a pulmonary ejection systolic murmur, a rumbling mid-diastolic murmur heard at the lower left sternal border, and a harsh apical holosystolic murmur that radiates to the axilla. ECG shows left axis deviation (LAD) of -45° and left ventricular hypertrophy (LVH). Based on the patient's underlying condition, what is the most likely cardiac abnormality?

Ostium primum ASD defect - Down syndrome - Ostium primum atrial septal defect (endocardial cushion defect) is commonly associated with Down syndrome - An accentuated first heart sound, wide and fixed splitting of the second heart sound, pulmonary ejection systolic murmur due to increased flow across the pulmonary valve, and a rumbling mid-diastolic murmur at the lower left sternal border (due to increased flow across the tricuspid valve) suggest atrial septal defect - The presence of left axis deviation (LAD) and left ventricular hypertrophy (LVH) in the ECG strongly suggest ostium primum ASD

Gene mutation responsible for congenital central alveolar hypoventilation syndrome ...

PHOX2B - frequent episodes of cyanosis during sleep

2 yo F + developed normally until around 6 mos. Then developed severe eczema and seizures. Unable to talk and uses few gestures to communicate with her family. PE = microcephaly. UA = large ketones. Most likely dx?

PKU

3 yo boy + hx of multiple episodes of otitis media and acute bacterial sinusitis is hospitalized for 2nd time with pneumococcal pna. He also has a hx of moderate to severe asthma and severe eczema. Labs show decreased IgM and increased IgA and IgE. Immunodeficiency disorder is expected. Most likely to be identified during additional evaluation?

Reduced number and size of platelets - Wiskott-Aldrich syndrome (WAS) = x-linked recessive

Young child with symptoms over 3 mos. Gastrocnemius muscle atrophy and leg-length discrepancy. Positive ANA. Has JIA. Next step?

Refer to ophthalmology for slit lamp exam - uveitis can be asymptomatic - she currently is having a chronic dz process

You have been following a 13-year-old Caucasian male in your general pediatric clinic for the past 5 years. His history is significant for several palpable lipomas (on his limbs, flank, and back) that have been stable. He has macrocephaly that has been persistently 2 to 3 standard deviations above the mean with height and weight at the 50th percentile. This appears to be familial, with his father's head circumference measuring just above 2 standard deviations above the mean. Your patient required ear tubes early in childhood for recurrent otitis media. He is mainstreamed in school and has never been held back, although he has a mild learning disability. His mother is concerned he may be dyslexic. He has had no other health issues. The patient presents today with new concerns regarding a possible genitourinary infection. He recently noted a discoloration to the skin of his penis. Urination is reported as normal and without pain. On physical exam, he is Tanner IV. Testes are descended bilaterally, measure 14 cubic centimeters, and are normal-appearing. His penis measures a stretch length of 9 centimeters. Upon close examination, the glans penis has a patchy area of hyperpigmentation in a

PTEN hamartoma tumor syndrome Mutations in the PTEN gene can cause a number of conditions as part of the PTEN hamartoma tumor syndrome umbrella, which includes: - Cowden syndrome (an adult-onset syndrome characterized by macrocephaly, increased risk of thyroid, endometrial and breast cancer, intestinal polyps, fibrocystic breasts, lipomas, fibromas, genitourinary tumors, uterine fibroids, and facial papules) - Bannayan Riley Ruvalcaba (with features including macrocephaly, intellectual disability, hamartomatous intestinal polyps, lipomas and pigmented macules of the glans penis among others) Given the presence of macrocephaly, learning disabilities, and abnormal pigmentation on the glans penis of this patient, PTEN gene analysis to evaluate for PTEN hamartoma tumor syndrome, including the Bannayan-Riley-Ruvalcaba syndrome presentation, is the most appropriate consideration.

4 yo found to have heart murmur. No concerns about exercise tolerance. No meds, allergies, or hospitalizations. BP and HR normal. II/VI murmur and best heard over anterior portion of upper chest during both systole and diastole. As pt asked to move his head up and down and side to side, the intensity of the murmur is noted to vary with position of head. Pulses equal and symmetric. Most appropriate next step in evaluation?

Parents can be reassured that additional evaluation is not needed Venous hum - turbulent blood through jugular venous system

15 yo girl with several day hx of sore throat, arthralgia, malaise, fever presents with sudden onset of pruritic swelling in her hands and feet. Temp is 100.2 F, HR is 68, and BP is 110/75. She does not appear acutely ill. There is erythematous, symmetrical swelling of hands and feet that is well-demarcated at wrists and ankles. She also has painful pruritic papules, petechiae, and purpura involving hands and feet. Etiology of pt's clinical signs and symptoms most likely due to...

Parvovirus B19 = papular pruritic gloves and socks syndrome (PPGSS) - may still be infectious

13 yo girl presents with complaints of bilateral knee pain. She states symptoms are worse when she is going up or down stairs. On exam, there is no effusion, but there is pain with pressure on patella over distal femur. This is enhanced if quadriceps is subsequently flexed. Most likely diagnosis?

Patellofemoral syndrome - common cause of anterior knee pain - exaggerating pain between patella and distal femoral groove - reduction of symptoms with strengthening of quadriceps

12 yo F presents with chorea-like movements that she cannot seem to control. At first, her mom thought she was "just fooling around," but then the mom realized her daughter could not control her movements. She has no history of sore throat but has oral ulcers and the following lab abnormalities: proteinuria, anemia, leukopenia. On CXR, she has bilateral pleural effusions. ASO titer is 1:40 (negative), and anti-DNase B antibody is 200 (<375 is normal). The ANA titer is positive at 1:640. Most likely diagnosis?

SLE - pt meets 6/11 criteria

Teen with arthritis in wrists, sores in her mouth, fatigue. No fever. Dx?

SLE - Still's is not associated with mucus membrane changes = distinguishing factor

Gene responsible for initiating male sex determination

SRY - encodes sex-determining region Y protein (testes determining factor)

Sudden episodes of high HR suggest

SVT

8 month old healthy infant traveling to Europe in 3 weeks on a family vacation. He is up to date on vaccines per US immunization schedule. Family concerned after hearing about measles outbreak in Europe. Best method to protect infant against measles?

Single dose of MMR vaccine prior to departure Preexposure prophylaxis against measles - Prevention for infants 6-11 mos traveling internationally prior to receiving routine vaccine (usually at 12 mos) = single dose of MMR prior to departure --> these infants should still receive routinely recommended 2 doses of measles-containing vaccine at 12-15 mos (at least 28 days after travel dose) and at 4-6 years of age once they return to US

A 14-year-old adolescent girl is seen for a health supervision visit. Her mother has questions about possible scoliosis. She is otherwise healthy and has had normal growth and development, with menarche occurring 2 years ago. She has no family history of scoliosis. A scoliometer measurement is 10 degrees with an Adams forward bend test. Her physical examination findings are otherwise unremarkable. Standing radiography of the spine reveals a wedge vertebra at T11 with a Cobb angle of 15 degrees and a Risser score of 4. Of the following, the BEST next management step for this adolescent is to

Perform renal US - The adolescent in the vignette has scoliosis caused by a vertebral malformation (T11 wedge vertebra), and is considered to have "congenital scoliosis," even though her curve was not detected until adolescence. - Because congenital scoliosis is associated with genitourinary anomalies, renal ultrasonography should be performed. - Children with suspected congenital scoliosis should undergo anteroposterior and lateral radiography of the entire spine that includes all cervical, thoracic, and lumbar vertebrae. In infants, these radiographs can be obtained while supine; older children should be standing. - The progression of congenital scoliosis is highly variable. Affected children should be monitored closely during periods of rapid growth. Depending on the type of malformation and pattern of progression, radiography should be performed every 3 to 12 months while the child is still growing. Congenital scoliosis is not typically associated with spinal instability; therefore radiography in flexion and extension is not indicated. - The Risser score ranges from 1 to 5 with a score of 5 indicative of being fully mature. A Risser score of 4 or 5 indicates limited remaining linear growth and is associated with a low risk for curve progression. The adolescent in the vignette is approaching skeletal maturity and has a radiographic curve of only 15 degrees; therefore orthopedic consultation is not necessary. - Genitourinary defects occur in 25% to 39% of children with congenital scoliosis, and renal ultrasonography is universally recommended.

A mother has brought her 9-year-old daughter to you because the child has been suffering from a 1-week history of intense itching on her scalp since she returned from a summer camp with a group of other children. Physical examination shows a number of nits attached to the hair shafts and a few full-grown lice on the scalp. Due to scratching, there are raw excoriated areas on the scalp with a few areas of pyoderma. Occipital group of lymph nodes are enlarged. What is the treatment of choice for this child?

Permethrin 1% topical - Non-prescription 1% permethrin cream rinse is applied to the hair and scalp and left on for 10 minutes, then rinsed out. - Permethrin is both pediculicidal and ovicidal. - A second application 7-10 days after initial treatment may be necessary. - Children may return to school after initial treatment. - 5% permethrin cream is used for treatment of body lice or scabies, but not head lice.

A newborn infant was delivered by emergency cesarean section for fetal distress following umbilical cord prolapse and meconium stained amniotic fluid. Infant was cyanosed, apneic, and hypotonic at birth. He was resuscitated by endotracheal intubation, suction of trachea under direct vision, and positive pressure ventilation. At 12 hours of age, the infant was observed to have central cyanosis and tachypnea. Cyanosis appeared to be comparatively less over the head, neck, and right upper limb, as compared to the trunk and the lower extremities He did not show significant improvement with 100% oxygen by hood, but showed transient improvement with mechanical ventilation with 100% oxygen. Auscultation of the heart showed accentuated second sound. There were no murmurs. Chest radiograph showed normal cardiac size and diminished lung markings. Partial arterial oxygen tension (Pao2) in the right radial artery was 20 mmHg higher than in the umbilical artery. What is the most likely diagnosis?

Persistent fetal circulation - or persistent pulmonary hypertension (PPHN) - history of fetal distress (due to umbilical cord prolapse) with meconium stained amniotic fluid, and the infant required tracheal suction and positive pressure ventilation at birth - partial oxygen tension (PaO2) in right radial artery is 20 mmHg higher than the umbilical artery. This finding is specific of persistent fetal circulation (PFC) - persistence of right to left shunting of blood through ductus arteriosus or foramen ovale or both in the presence of elevated right ventricular pressure - can be triggered by several perinatal factors. Hypoxia and acidosis are known pulmonary vasoconstrictors. Any condition that disrupts uteroplacental circulation, such as placental abruption and placental insufficiency, causes hypertrophy and thickening of muscular layer of pulmonary vessels, leading to their narrowing. This makes the neonate more susceptible to PFC. - right arm and head and neck remain pink, while the left arm and lower body are cyanotic. This is called differential cyanosis and is due to the difference in oxygen content in pre-ductal and post-ductal blood and is specific for PFC. - Also, a positive PaO2 gradient of 20mm or more between the right radial artery and the descending aorta suggests PFC

Following transport to ED for suspected overdose, diaphoretic and agitated 17 yo is noted to have pain, tenderness, and edema of muscles of lower extremities, associated with a positive result for heme on urine dipstick and absence of red blood cells on urine microscopy. HR is 135 and BP is 170/110. His pupils are small. What drug is most likely to be the cause of his symptoms and would be picked up on a screen of urine metabolites?

Phencyclidine - lab evidence of rhabdomyolysis - tx'ed with IV fluids and low urine pH to prevent precipitation in kidneys - miosis sets it apart from other sympathomimetics

Found in babies with moms who had this medication. Neck abnormalities, microcephaly, brachycephaly, midfacial hypoplasia, low-set ears, mild hypertelorism, depressed nasal bridge, cleft lip and palate. Digits of hands are often hypoplastic or even absent. Coarse hair and hirsutism are common ...

Phenytoin - category D pregnancy risk factor - GU defects are common - growth defects with intellectual disability - increased risk of certain neoplasms

35 yo gravida 1 gestational diabetic mom presents to ED in labor and delivers 12 lb, 3 oz male via precipitous vaginal delivery. Left upper arm adducted and internally rotated with forearm extended; hand and wrist movement are preserved. Best plan of action at this time ...

Physical therapy and observation - Erb palsy

Is essential to preventing drug use in teens ...

Positive mutual parent-child relationship - Healthy parental attitudes and behaviors are likely to be internalized and expressed in adolescents' own lives; they are more likely to select a pathway of drug abstinence and not drift toward affiliations with drug-using peers.

Growth hormone therapy has been associated with sudden death in what syndrome?

Prader-willi syndrome - recommended that sleep study be performed and potential risks and benefits be considered and discussed prior to initiating GH therapy in pts with Prader-willi syndrome

Management of children under the age of 5 who are exposed to TB...

Should receive isoniazid prophylaxis even if the result of the initial TB test is negative

Teen with pain at bottom of patella, including when you push against them extension. Wrestles. Dx?

Sinding-Larsen-Johansson Disease - pain at apophysitis of inferior pole of patella

You are evaluating a 6-year-old child with a history of recurrent pneumonias. The child has a history of frequent ear infections and often gets sick due to pneumonias, requiring the frequent use of antibiotics. He has a chronic wet cough that is present most of the time, and he also has failure to thrive and poor weight gain. As part of the diagnostic work up, what would be most useful when differentiating between cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) in this patient?

Sweat chloride level of >60 mmol/L - Sweat chloride greater than 60mmol/L is diagnostic of CF, whereas it is normal in PCD patients.

Previously health 10 yo boy with severe diarrhea. Dry mucosa. SBP in low 80s. Abdomen is diffusely tender, but his PE is otherwise unremarkable. Labs generally normal with exception of elevated BUN, Cr of 1.8, urine spec gravity of 1.030 with otherwise bland U/A. Fractional excretion of sodium (FeNa) <1%. What diagnosis do these findings support?

Prerenal azotemia - via GI losses/intravascular volume depletion

Treatment of iron deficiency anemia dosing:

Preterm infants: 2-4 mg elemental iron/kg/day PO divided BID or may be given once a day Children: 3-6 mg elemental iron/kg/day PO divided BID - TID - Typically initiate ferrous sulfate, 3 mg/kg of elemental iron, once daily in the morning or between meals. - Iron may be given alone or with water, juice, or acidic fruits (eg, citrus, strawberries, or applesauce), with care to ensure that the entire dose is taken. - Milk and/or dairy products should be avoided for approximately one hour before and two hours after each dose. - We choose to use ferrous sulfate and the 3 mg/kg dose because it was effective in a clinical trial and may be better tolerated than higher doses that are sometimes used

Child has severe factor VIII deficiency, hemophilia A, as confirmed with the laboratory findings. He likely had an injury to the right knee, resulting in bleeding into the joint as evidenced by the pain, swelling, increase in knee circumference, and inability to ambulate. This complication, known as hemarthrosis, should be treated as soon as possible with factor concentrate administration. Repeated bleeding into this joint may lead to the development of a target joint, a joint at risk for deformity, limited range of motion, and decreased strength. What helps prevent this?

Prophylactic factor concentrate administration - Factor VIII deficiency = one of the most common congenital coagulation factor deficiencies = inherited in an X-linked recessive pattern (all males are affected)

4 week old exclusively breast-fed infant brought for WCC. Noticed streaks of blood in stool for past week. Baby is well-appearing and otherwise healthy. Growing along his growth curve. Most likely diagnosis?

Protein-induced proctitis - AKA milk protein allergy - presents in infancy with streaks of blood in stools - milk and soy protein = most common triggers

Male newborn had oligohydramnios on prenatal US + Few hours after delivery, unable to palpated testicles in scrotum + child has not yet produced urine + very soft abdomen with midline abdominal mass. Most likely dx?

Prune Belly syndrome - UTI abnormalities + cryptorchidism

What are the characteristic symptoms of Pediculosis?

Pruritus and macular rash on the scalp

Female + short stature. Wt percentile is greater than that of her height. 4th and 5th fingers appear short. Several members of her family have similar appearance. Calcium low at 6.8, P 7 (high), PTH 100 (high). Most likely diagnosis?

Pseudohypoparathyroidism

12 yo girl + arthritis of left hand and knee. Dactylitis, pitting of her nails. DIP joints of her hands most severely affected by arthritis. ANA returns positive. Most likely diagnosis?

Psoriatic juvenile idiopathic arthritis - arthritis and psoriasis + 2/3 of: dactylitis, nail pitting, 1st degree relative with psoriasis

Most common complication of L-transposition is complete heart block. Pts present with fatigue, bradycardia, exercise intolerance. EKG =

Q waves in right chest leads and no Q waves in left - echo = anatomic left ventricle connecting to right atrium at pulmonary artery on right side

Experimental studies that can determine causation between a treatment and a desired outcome...

Randomized controlled trials

During WCC, 6 week old male noted to have nonblanching, nontender, bluish, fluctuant swelling lateral to midline in lower right portion of his mouth, which displaces the tongue superiorly and slightly to the left. What is the most likely cause of this pt's clinical finding?

Ranula - pseudocyst associated with sublingual glands and/or submandibular ducts - appearance sometimes compared to belly of frog - congenital or via oral trauma - surgical excision often recommended

A 13-year-old African-American female presents for her annual well child check. The parents report there is a significant family history of cancer. The family history reveals that 2 of the patient's maternal aunts were diagnosed with breast cancer in their late 30's. One of these aunts recently had genetic testing for the BRCA1 and BRCA2 genes. The results revealed a mutation in the BRCA2 gene, which was also identified in the other affected aunt. The family wants their daughter tested for this mutation. What is the most appropriate recommendation in this case?

Refuse to test the patient until the child is an adult - Mutations in the BRCA1 and BRCA2 genes cause a hereditary breast and ovarian cancer syndrome. Individuals who have inherited such a mutation have a significantly increased risk of developing primarily breast and ovarian cancer. - Because not everyone with a mutation will develop cancer, this is considered predispositional testing for an adult-onset condition. - Numerous professional genetics societies have policy statements against presymptomatic or predispositional genetic testing for adult-onset conditions in childhood. It is recommended that such testing be pursued only in the event that the results would alter medical management. If medical management would not be altered, it is recommended that testing be pursued when the child is of adult age so that they may make their own autonomous and informed decision.

7 yo F with HIV and severe immune suppression is brought to your office because her friend has measles. Pt and her friend shared a bed during sleepover birthday party 2 days ago. What is appropriate for this pt?

Regardless of vaccine hx and serologic results, pt should receive dose of immunoglobulin by IV route - IVIG should be administered within 6 days for postexposure prophylaxis against measles for all HIV-infected pts with severe immune suppression who have significant exposure to measles

10 yo girl presents for routine evaluation. She has lived in multiple cities throughout the United States bc her father's job has often changed locations. Documentation of her immunization status cannot be found, and her father is unable to reliably state which immunizations she received or when. In light of her uncertain IZZ circumstances, what best describes the recommended IZZ schedule for this pt?

Reinstitute entire series for hepatitis B, hepatitis A, MMR, varicella, IPV, and Tdap, Td

A 2-year-old female presents to the emergency room having swallowed a coin approximately 2 hours prior to her arrival. She has some drooling and appears anxious during the examination. She has no history of esophageal injury. Her parents have noted no stridor or dyspnea. Physical examination is unremarkable. A chest radiograph reveals a radiopaque round object at the distal esophagus. Which of the following is the most appropriate treatment option?

Removal of coin via esophagoscopy - Coins are the most commonly ingested object, usually in children less than 5 years of age. - Initially asymptomatic patients may also develop edema and inflammation leading to esophageal obstruction. Esophageal perforation may also occur. Radiographs will show radiopaque objects readily. - Symptomatic patients, such as the one in the vignette, should have the object removed via esophagoscopy and the esophageal mucosa examined for injury. - Asymptomatic patients may be observed for 24 hours if the object is not sharp or have sharp projections. If the object has not moved after this period of observation, then esophagoscopy would be performed.

Intellectually disabled 8 yo, blind due to congenital aniridia, presents for health maintenance exam. At birth, chromosome studies, ordered as part of eval for ambiguous genitalia, confirmed a male karyotype. What has most likely been periodically performed on this patient in order to screen for a known complication associated with his clinical hx and current physical exam findings?

Renal US WAGR syndrome - Wilms tumor, aniridia, GU malformations, reduced intellectual ability - 11p13

X-linked disorder, predominantly affecting girls, characterized by a period of normal development followed by motor, language, and cognitive regression, loss of purposeful hand movements replaced with hand wringing, and an autistic-like phenotype

Rett syndrome - Serial head circumference measurements can demonstrate decelerating head growth during the time of normal development and be an early clue in patients with Rett syndrome. - Treatment of Rett syndrome is currently supportive.

3 yo in ED for fever and cough. Undergoing tx for ALL. Increased fussiness and difficulty breathing. F 101.5F. O2 90%. PE = bilateral inspiratory wheezing. Admitted to hospital and started on drug that inhibits inosine monophosphate dehydrogenase. What drug has been administered?

Ribavirin / virazole - RSV in setting of ALL - binds RNA polymerase --> inhibits IMDP - off-label use

14 yo boy + uncertain IZZ hx. Recently adopted from orphanage in remote area of former Soviet Union. Presents to pediatrician with 3 day hx of headache, generalized malaise, sore throat, cough. Temp 101 F. Findings on exam include prominent suboccipital and postauricular LAD + soft palate petechiae distributed among larger reddish spots. Following day while awaiting results of lab investigations and receiving supportive management at home, he develops fine, discrete, maculopapular rash that starts on face and rapidly spreads to whole trunk and arms. Most likely cause of clinical signs and symptoms?

Rubella virus - AKA German measles - prodromal period - enanthem = Forchheimer sign = soft palate petechiae and/or large reddish spots - exanthem ~ 72 hours - rash usually disappears by 3rd day - rash often fades as it spreads - rapidly changing nature of rash = diagnostic

Should be considered in patients who experience effortless and painless vomiting for 2 months ,,,

Rumination syndrome - Rumination syndrome can be treated with behavioral therapy

Low serum sodium level, low serum osmolality, inappropriately high urine osmolality, and high urine sodium level favor a diagnosis of

SIADH

Required to make a diagnosis of AOM

Symptoms of pain or fever, together with an inflammatory middle ear effusion

A 10-month-old female infant presents for gradual regression of milestones over the last 4 or 5 months. The infant, who could previously hold her head up, sit with support, and vocalize, can no longer do so. She is less responsive to her mother's voice and shows no apparent excitement or pleasure upon seeing her. The infant makes very little eye contact when held or spoken to. There is no history of convulsions. She has had 2 respiratory infections during the last 3 months. Physical examination reveals that the child has enlargement of the liver 5 cm below the costal margin and of the spleen, 6 cm below the costal margin. Cardiovascular and respiratory systems are essentially normal. Central nervous system examination reveals hypotonia of the limbs. Image shows cherry red spot. Dx?

Sandhoff - infant presents with a loss of motor and language milestones beginning at about 5-6 months of age - hypotonia and moderate hepatosplenomegaly - also has apathy, and both visual and hearing los - fatal, with most children dying by the age of 3 - can both be distinguished by individual estimations of beta-hexosaminidase A and beta-hexosaminidase B - moderate to severe hepatosplenomegaly is present in Sandhoff disease but it is absent in Tay-Sach's disease - confirmed by deficient levels of hexosaminidase A or B in serum and leukocytes

You are evaluating a 12-year-old girl at a health maintenance visit. She has no specific complaints. She is doing well academically and socially in the 7th grade and is a starting guard on her school's basketball team. Breast development began at age 10 years. She has not reached menarche. She has noticed a rapid and, to her, alarming, increase in height. Past medical and family histories are unremarkable. Growth and development have been normal and immunizations are up to date. Vital signs are normal, with height at the 75th percentile and weight at the 50th percentile. Prior to puberty, her height had been at the 50th percentile. Examination reveals the presence of areolae as secondary mounds above the breast contour and copious, long, dark, curled pubic hairs not extending to the medial thighs. She is wondering how far along into puberty she is since she does not want to be a "beanpole" like her older sister. How would you answer her question concerning progress in puberty?

She is at SMR breast stage IV, pubic hair stage IV - Sexual maturity rating (SMR) stage IV breasts display the areolae forming separate mounds above the contours of the breasts. - Stage IV pubic hair for females is of adult quality (copious, long, dark, curled pubic hair) that has not yet reached the medial thighs (mid-escutcheon), as seen in this patient. - Stage IV displays the areolae forming separate mounds above the contours of the breasts and adult quality pubic hair that has not yet reached the medial thighs (mid-escutcheon).

4 yo girl + 24 hr hx of diarrhea presents to ED after her mom noted blood in her underwear. Hx of potential trauma denied. PE reveals bloody serosanguineous discharge. Stool sample negative for occult blood. Culture of vaginal discharge most likely to be positive for what organisms?

Shigella flexneri - most common infectious causes of bloody and/or serosanguineous vaginal discharge and vulvovaginitis = shigella flexneri, shigella sonnei, group A strep

Mother of 6 lb 4oz male born at term is known to be positive for HBsAg. Additional lab findings obtained during her prenatal course include negative HBeAg and negative Ab to hepatitis B core antigen. Most appropriate plan for subsequent care of this pt?

Should receive HBIG vaccine at birth, Hep B vaccine at 1-2 and 6 mos of age, and will require testing for HBsAg and anti-HBsAg at 9 mos of age - HBsAg + moms --> HBIG and hep B vaccine at diff't sites within 12 hours of birth - anti-HBsAg levels >10 --> immune = no additional vaccination or f/u - anti-HBsAg < 10 + negative --> reimmiunize with 3-dose series of hep B vaccine at 2-month intervals --> retest levels 1-2 mos after 3rd dose

The parents of a 2-year-old girl present with their daughter for evaluation of the her abnormal sleeping behaviors, which are causing the parents and child to get insufficient sleep at night. The parents usually put the child to bed after a routine of changing clothes, brushing teeth, reading a book, and rocking her to sleep while one of the parents sings a lullaby to her. She will then wake up multiple times throughout the night, "like clockwork," approximately every 90 minutes. Due to her prolonged crying and inability to fall back asleep, one of the parents has to go to her room to try to calm her down by rocking her and then putting her to sleep. The parents state that there have been nights when they have slept holding her in a rocking chair. She is otherwise healthy, with no symptoms of reflux or concerns for sleep-disordered breathing. What is the most likely diagnosis?

Sleep-onset association disorder - condition in which the child has learned to fall asleep only under particular circumstances or associations; they cannot "self-soothe" - the parents rock the child to sleep and the child has learned the association of rocking to sleeping

A 9-year-old male reports he was poked in the eye accidentally by a classmate with a pencil at school today. There was immediate pain, tearing, and blurry vision and his mother was called to take him home. He says it still hurts though the tearing and blurriness have subsided. On exam, his pupils are equal and reactive, extraocular movements intact, sclera is mildly injected, visual acuity is 20/30 in both eyes, and there is no foreign body seen. Which of the following would be most appropriate for this boy?

Stain cornea with fluorescein - Minor injury to the eye is most likely to cause a corneal abrasion. These occur often in children of all ages and usually result in loss of one or several of the layers of the corneal epithelium. - Being richly innervated, a corneal abrasion will cause mild to severe discomfort depending on the size of the defect along with photophobia, tearing, irritability, blurry vision, conjunctival injection, blehpharospasm, irregular red reflex, and fluorescein staining of the defect. - Fluorescein dye, available in strips, will stain the exposed corneal stroma but washes off of intact epithelium. This yellow dye may be visible with regular white light, but is best seen using a blue or ultraviolet light. Examination may be facilitated by the use of topical anesthetics or combination anesthetic/fluorescein drops. - Most corneal abrasions will heal rapidly, often within 24 hours. - Antibiotic ointment helps lubricate the surface until healing occurs.

19 yo M + surgical repair of large secundum ASD. Postop course complicated by difficult-to-treat a fib. Discharged home on amiodarone. Meds include metoprolol, furosemide, spironolactone. 6 mos later, he visits his cardiologist and reports SOB with exertion. Denies palpitations. PE is normal. EKG = sinus rhythm. Echo shows interval improvement of chamber sizes with normal biventricular systolic function and no patch leaks. PFT = reduced diffusing capacity of lungs to carbon monoxide (DLCO) and reduced FEV1. Best course of action at this time?

Stop amiodarone - class III antiarrhythmic - many side effects, including pulmonary fibrosis

Similar to the microbiology of AOM, the most common etiologic agent of acute mastoiditis is

Streptococcus pneumoniae - The incidence and frequency of AOM caused by S pneumoniae has declined since the introduction of pneumococcal conjugate vaccines PCV-7 and PCV-13. - The diagnosis of acute mastoiditis is clinical. Imaging is not indicated in uncomplicated cases. - Treatment of mastoiditis includes systemic antibiotics and, in many cases, surgical drainage. An otolaryngology consultation should be obtained. - It is prudent to obtain a middle ear fluid specimen by tympanocentesis for culture and antimicrobial sensitivities. Intravenous antimicrobial agents should be targeted against pathogens causing AOM, especially S pneumoniae and S aureus (including methicillin-resistant S aureus), for example, vancomycin plus ampicillin-sulbactam. - In the setting of recurrent AOM and recent antibiotic use, some experts prefer a combination of vancomycin plus ceftazidime or cefepime (to cover P aeruginosa). - If complicated disease, including intracranial complications, is suspected, empiric treatment with vancomycin and meropenem, or vancomycin plus ceftriaxone and metronidazole, is recommended. A 4- to 7-day course of parenteral antibiotic therapy followed by an oral antibiotic regimen for a total of 14 to 21 days is recommended for uncomplicated cases of acute mastoiditis. Longer durations of intravenous antibiotic therapy (3-6 weeks) may be necessary for intracranial complications of mastoiditis (such as venous thrombosis or brain abscess). - If there is no clinical improvement after 48 hours of intravenous antibiotic therapy, a contrast-enhanced CT scan of the temporal bone is recommended to evaluate for suppurative complications.

9 yo F + right shoulder pain for 6 mos + pain after crashed bike and fell on outstretched extremity + episodes of shoulder giving out but no obvious dislocation. Anterolateral shoulder pain and weakness with overhead activities. Difficulties with bike riding, swimming, bball. Weakness in adduction and medial rotation of right arm. Positive lag test on right side as well. MRI = completion rupture of tendon of one of her muscles. What muscle is injured?

Subscapularis - adduction and medial rotation of humeral joint - positive internal rotation lag sign

About 15 minutes ago, a 3-year-old boy accidentally ingested a detergent containing a caustic alkali. The boy is irritable, and he now presents with pain in his mouth, throat, and abdomen. He has also vomited once and is having difficulty swallowing. Physical exam reveals that his vital signs are stable. Oral mucosa is red, and there is excessive salivation and drooling. What is the immediate treatment for this patient?

Supportive care and diagnostic endoscopy in 12 hours - The immediate treatment of choice for this child is supportive care followed by diagnostic endoscopy, usually performed 12 hours after ingestion. - The endoscopy evaluates the esophagus for burns and other injuries.

Uncommon form of left ventricular obstruction and is sporadic, familial, or associated with Williams syndrome

Supravalvular aortic stenosis

Female, born by c-section, for breech presentation at 38 weeks, is noted to have multiple deformities of proximal and distal segments of lower extremities. Plain radiograph reveals partial agenesis of sacrum and lumbar vertebrae; absence of acetabulum; and bilateral hypoplasia of femur, tibia, and fibula. What maternal factors most likely contributed to these physical and radiographic findings?

T1DM Caudal regression syndrome / caudal dysplasia sequence - moms with T1 or T2 DM

Previously unvaccinated 17 year old patients often require what vaccines?

TDaP IPV Hep A Hep B MMR varicella HPV MCV influenza - If not administered at the same time, MMR and varicella should be separated by >1 mo to prevent diminished immunogenicity and a less optimal immune response (this recommendation applies to all live vaccines)

Child noted to have jaundice on DOL 1 at appx 20 hours of birth. Fam hx of physiologic jaundice. Child is being breastfed by Rh+ mom. Dx?

TORCH infection - jaundice within first 24 hours of life = bad

6 mo old + dysmorphic features + worsening murmur + renal cyst + multiple intracardiac tumors without valve obstruction + large hypopigmented macule on back. Gene that causes this condition?

TSC1 - tuberous sclerosis complex - AD - TSC1 and TSC2 - clinical signs most often present in infancy

A previously healthy 14-year-old adolescent girl is seen in the office for fatigue and intermittent constipation that she has experienced for the past 2 months. Her last menstrual period was heavier than usual. She reports no sexual activity. She has a temperature of 37°C, a blood pressure of 110/68 mm Hg, and a heart rate of 56 beats/min. She has a weight at the 50th percentile, height at the 75th percentile, and body mass index at the 40th percentile. Her thyroid is enlarged to about twice the normal size and has a firm, heterogeneous texture, without palpable nodules. The remainder of her physical examination findings are unremarkable. A urine pregnancy test has a negative result, and a complete blood cell count is normal. Best next step

TSH - Her enlarged thyroid gland, with a firm, heterogeneous texture and no nodules, is consistent with autoimmune thyroiditis (Hashimoto thyroiditis), the most common cause of acquired hypothyroidism. - Obtaining a thyroid-stimulating hormone level is the best next test to make the diagnosis of primary hypothyroidism (a primary problem with the thyroid gland). In this case, the thyroid-stimulating hormone level would be elevated. - Thyroid peroxidase and anti-thyroglobulin antibodies are often detectable in Hashimoto thyroiditis, and test findings for them would likely be positive in this girl. - Thyroid ultrasonography is not indicated for this girl. The American Academy of Pediatrics Section on Endocrinology, in its Choosing Wisely recommendations, indicated that clinicians should "avoid routinely ordering thyroid [ultrasonography] in children who have simple goiters or autoimmune thyroiditis." Thyroid ultrasonography should be performed in patients with asymmetric thyroid enlargement, palpable nodules, or concerning cervical lymphadenopathy. - Thyroid nodules are more common with Hashimoto thyroiditis, but the majority are benign.

Blocks effects of estrogen on breast tissue. Results in regression of breast size in pubertal gynecomastia.

Tamoxifen

>/=7 yo incompletely immunized child with need for tetanus booster. Which tetanus vaccine to use?

Tdap - for catch up immunization

Treatment is available, particularly for those who have severe disease, are at risk for severe monkeypox disease, (ie, those who are less than 8 years of age, those with immunocompromising conditions, those who have a history of certain skin conditions), those who have accidental implantation or lesions in certain anatomical areas (ie. eyes, mouth, genitalia, anus), and children and adolescents with complications from monkeypox. What is the first-line treatment

Tecovirimat - being used under an investigational protocol - CDC recently streamlined the process to obtain it - available in both oral and intravenous forms.

5 yo male + some pubic hair. Testicular volume is 3 cc. Testicles are descended bilaterally. Mom has been giving his deodorant for odor. You suspect premature adrenarche but need to r/o more serious diseases. What labs should you include in your workup?

Testosterone dehydroepiandrosterone-sulfate androstenedione 17-hydroxyprogesterone = workup for late-onset CAH (17-hydroxyprogesterone) and androgen-secreting tumors or exogenous androgen exposure

Mother brings you 6 mo old daughter with hx of cyanotic spells. When baby gets upset, she turns blue. Once she quits crying, her color returns to normal. Prenatal care started at 4 mos. IUTD. Breastfeeds only. O2 sat 85%. CV exam: regular rate and rhythm. 3/6 systolic ejection murmur along 3rd intercostal space to left of sternum. CXR shows decreased pulmonary markings. EKG shows RVH. Dx?

Tetralogy of fallot = RVH, pulmonic/pulmonary stenosis, VSD, overriding aorta - this pt is havinbg tet spells = cyanotic spells due to shunting of blood right to left across VSD

Angelman syndrome is caused by ...

maternal microdeletion

Patients with toxic shock syndrome should receive therapy initially with ...

max doses of clindamycin (suppresses toxin synthesis) + nafcillin (or vancomycin when MRSA is suspected)

Pt with recurrent episodes of pharyngitis associated with positive culture for group A beta-hemolytic strep (GAS) despite adequate antimicrobial tx indicates ...

may be carrier of organism

Features include her short stature with declining growth velocity, recurrent otitis media, and difficulty with math.

Turner syndrome - Other common features include ptosis; epicanthal folds; low-set, prominent ears; high-arched palate; neck webbing; low posterior hairline; broad chest with wide-spaced nipples; multiple nevi; congenital heart disease (eg, coarctation of the aorta); renal anomalies (eg, horseshoe kidney); skeletal anomalies (scoliosis, shortened 4th metacarpals, high upper-to-lower segment ratio); and primary ovarian failure. - Short stature is the most common manifestation of Turner syndrome and may be the only presenting feature. Turner syndrome should be considered in all girls with short stature.

Newborn infant with choking and coughing after initial feeding. Full term vaginal delivery 1 hour ago. Complicated by single umbilical artery , absent right kidney, polyhydramnios. Apgars 8, 9. Saliva and mucus bubbling at mouth and hypoplastic right thumb. Cause of current symptoms?

Type C esophageal atresia + distal TE fistula

Mother of otherwise healthy 4 yo male is concerned about several palpable bruises over child's buttocks and lower extremities. Child also complains of ankle and abdominal pain. What lab is most likely to be abnormal?

UA IgA vasculitis / HSP - systemic vasculitis can cause glomerulonephritis d/t IgA deposition in kidneys

Teen with asymptomatic ADPKD. Fam hx too. Screening needed?

UA with microscopy - watch BP and make sure no proteinuria, etc

You are asked to evaluate a 4-day-old male infant; he was born at term. He is the 3.1 kg product of an uncomplicated pregnancy, labor, and unassisted vaginal delivery of a 24-year-old G1P0Ab0woman. There was a positive newborn screening test for phenylketonuria (PKU). The child's mother reports that he appears to be completely well; he has been breastfeeding without difficulty or vomiting. Physical examination is entirely normal. What would you tell this child's mother?

The child requires plasma phenylalanine and tyrosine levels - The family should be referred immediately to a center with a specialist in metabolic diseases. Plasma levels of phenylalanine and tyrosine should be obtained. - In classic PKU, a mutation of the PAH (phenylalanine hydroxylase) gene impairs conversion of phenylalanine to tyrosine, resulting in increased plasma levels of phenylalanine, increased urinary excretion of phenylpyruvic and phenyllactic acids, and decreased plasma levels of tyrosine. A phenylalanine level of > 20 mg/dL will confirm the diagnosis.

A 16-year-old girl presents with pallor, petechial rash over the lower limbs, and alteration of sensorium and seizures following a brief flu-like illness (fever, fatigue, arthralgia). Physical examination reveals BP145/90 mm Hg and hemiparesis of the right side. Spleen is palpable 2.5 cm below costal margin. Blood examination shows Hb 6 g/dL, TLC 12,000/cm3, platelet count is 30,000/cm3. Peripheral smear show presence of schistosis (fragmented RBC). Reticulocyte count-elevated. Serum lactate dehydrogenase levels are markedly raised. Prothrombin time (PT), activated thromboplastin time (APTT), and fibrinogen levels are normal. BUN and serum creatinine levels are raised. Urinalysis shows proteinuria and microscopic hematuria. What is the most likely diagnosis?

Thrombotic thrombocytopenic purpura - patient is an adolescent girl presenting with microangiopathic hemolytic anemia suggested by the presence of schistocytes in the peripheral smear, thrombocytopenia manifesting as petechiae and purpura, CNS involvement in the form of alteration of sensorium, and seizures and focal neurological deficits as hemiparesis of right side - Renal involvement is evident by the presence of hypertension, proteinuria, hematuria, and raised BUN and serum creatinine levels. - The diagnosis is further supported by an elevated level of lactate dehydrogenase.

A 17-year-old girl has severe nodulocystic acne vulgaris of the face. Routine topical preparations, topical and oral antibiotics, and topical Retin-A have not helped. She is being treated with oral isotretinoin. This medication is subject to an FDA Risk Evaluation and Mitigation Strategy (REMS) to ensure that the benefits of the drug in question outweigh the risks. What is the primary aim of the REMS of isotretinoin for this patient?

To prevent exposure of pregnant patients to isotretinoin - The main purpose of a REMS is to ensure that drug benefits outweigh risks. - Isotretinoin is highly teratogenic. The patient must commit to employing two effective forms of contraception beginning 1 month before starting isotretinoin, continuing throughout treatment, and for 1 month after cessation of treatment. Two pregnancy tests must be negative before starting treatment, and monthly pregnancy tests must be performed by accredited laboratories before the patient can obtain monthly refills.`

A 3-year-old boy presents with persistent jaundice. He has no history of dark urine, acholic stools, liver disease, blood loss, or easy bruising. He required an exchange transfusion as a neonate for severe hemolysis of uncertain etiology, and he has needed at least six transfusions per year for severe anemia; his last transfusion was 2 weeks ago. Height and weight are 10th percentile. No family history of similar symptoms. Vitals normal. Moderate scleral and cutaneous icterus, moderate pallor of oral mucosa, no hepatomegaly, and spleen palpable 4 cm below the left costal margin. Stool negative for occult blood. Moderate increase in total bilirubin, almost all indirect. Hemoglobin 9.4 gm/dL, normal MCHC, normal MCV. Reticulocytes 15%. WBC and platelets normal. Peripheral smear reveals few RBCs of normal morphology. Some elongated RBCs, predominance of microspherocytes, fragmented cells, and poikilocytosis: stomatocytes, budding erythrocytes, and pyknocytes. He is diagnosed with hereditary pyropoikilocytosis. What is the best recommendation regarding the splenomegaly?

Total splenectomy after age 5-6 - A CBC with peripheral smear and reticulocyte count helps distinguish between increased destruction (suggested by splenomegaly) and decreased production. The increased reticulocyte count also suggests increased RBC destruction. A normal MCHC and normal MCV (normochromic, normocytic anemia) are seen in hereditary elliptocytosis, confirmed by the peripheral smear. - In hereditary pyropoikilocytosis, elliptocytes are fewer than in more common forms, with a greater number of bizarre shapes. Splenectomy is indicated for severe anemia, frequent transfusions, or growth failure in this and other conditions causing increased erythrocyte destruction. - If possible, splenectomy should be deferred until after age 5-6 to reduce the risk of overwhelming post-splenectomy sepsis (OPSS). Before splenectomy, pneumococcal, meningococcal, and Haemophilus influenzae type B immunization should be initiated or updated.

1 year old girl was treated for a febrile Escherichia coli urinary tract infection (UTI). Imaging revealed right hydronephrosis and grade IV vesicoureteral reflux (VUR). Antibiotic prophylaxis is indicated because she is not toilet-trained and her VUR is at grade III or higher. What is preferred for prophylaxis?

Trimethoprim-sulfamethoxazole (TMP/SMX) - because of its bactericidal action - after the urine is made sterile with treatment doses of third-generation cephalosporins, TMP/SMX would be effective in preventing future infection for this girl despite any recent E coli infection showing resistance to TMP/SMX - Antibiotic prophylaxis is indicated for children with low-grade VUR with recurrent UTI, grade III or higher VUR, or non-toilet-trained children with any grade of VUR - The antibiotic is preferably given once daily at bedtime - Prophylactic antibiotic dosing is generally one-fourth to one-half of the daily treatment dose - Commonly used agents include TMP-SMX, trimethoprim alone, or nitrofurantoin. Trimethoprim-sulfamethoxazole is preferred for prophylaxis because of its bactericidal action - Amoxicillin prophylaxis is recommended for infants younger than 2 months, because both TMP/SMX and nitrofurantoin interfere with hepatic conjugation and increase the risk of hyperbilirubinemia

A 14-year-old girl presents for a routine health maintenance visit; the family has recently moved to the area from a different state. The patient's past medical history is significant for lymphoma when she was 5 years old. As part of her treatment, she received chemotherapy and radiation therapy involving her neck and chest. According to the patient and her mother, the patient has been doing well in school and has no complaints. On physical exam, a mass is palpated in her neck; the rest of her physical exam is completely normal. The patient and family state that they are unaware of the mass. What diagnostic test would you now order for this patient?

Ultrasound-guided fine needle biopsy of the mass - In a patient with a history of previous malignancy and irradiation to the neck and chest area, a neck mass is highly suspicious for an underlying thyroid carcinoma. - Although thyroid carcinomas are rare in the pediatric population, there is a possibility of this diagnosis in patients that have a history of radiation therapy to the head and neck area. - Papillary thyroid carcinoma is the most common form of thyroid malignancy in the pediatric population. - Ultrasound-guided fine needle biopsy is the best diagnostic test to determine if the patient truly has an underlying malignancy or a benign condition. Other tests to consider should include, but are not limited to, TSH, free T4 levels, and a complete blood count.

18 mo old transported to ED following removal from his home d/t medical neglect. He appears cyanotic and several malnourished. No petechiae or purpura. RR 38. HR 140. Gallop rhythm noted on cardiac exam. CXR reveals cardiomegaly, especially right side of heart. What vitamin deficiency is most likely cause of his symptoms?

Vitamin B1 / thiamin - important in release of energy from carbs - important for synthesis of acetylcholine - deficiency = cardiac sequelae --> cardiomegaly: heart failure + generalized edema

A presumptive diagnosis of iron deficiency anemia (IDA) is made by the combination of positive risk assessment and laboratory findings of a microcytic anemia, which may be mild. The diagnosis is confirmed if

a trial of oral iron supplementation (3 mg/kg elemental iron per day) produces a hemoglobin (Hgb) rise of >1 g/dL within four weeks for children with mild anemia or within two weeks for those with severe anemia.

Patient experienced menarche <2 years ago, is not obese, and has no signs or symptoms of a chronic medical illness, making anovulatory bleeding the likely cause of her abnormal uterine bleeding. Anovulatory cycles are d/t immature hypothalamic-pituitary-ovarian axis and characterized by

absence of normal midcycle surge of LH / absence of functional corpus luteum

What cell characteristic would support a dx of abetalipoproteinemia

acanthocytes on peripheral blood smear = erythrocytes with spiny membrane projections - AR disorder - mutation in gene for microsomal glyceride transfer protein - absence of beta lipoproteins - steatorrhea, ataxia, dev'tal delay

Purpose of high-frequency oscillator ventilator in congenital diaphragmatic hernia ...

achieves acceptable oxygen at lower pressures --> reduces barotrauma to the lungs + better synchronizes with infant's own respiratory efforts - usually reserved for infants with persistent hypoxia and hypercarbia

Major reasons for delay in transplantation...

active infections uncontrolled malignancy ABO incompatibility - infections must be treated before transplantation

A 6-year-old boy with Down syndrome is brought to the emergency department after a drowning accident in a lake. Submersion time is unknown, but he was missing for some time. He was resuscitated and intubated by emergency medical technicians during transport. On arrival, his heart rate is 76 beats/min, BP 104/72 mm Hg, and rectal temperature is 81°F (27°C). He remains comatose, exhibiting non-purposeful flexion withdrawal to pain. His pupils are equal and reactive. What is the best way to treat this patient's drowning-related hypothermia?

active internal warming - gold standard for warming patients who have cardiac arrest or ongoing cardiovascular instability due to hypothermia, and it is most commonly achieved with cardiopulmonary bypass or extracorporeal life support - severe hypothermia with core temp ≤90°F is best treated with active internal warming. Intravenous fluids can be heated to approximately 104-113°F (40-45°C). Infusing warmed intravenous fluid also offers an additional advantage of improved absorption of administered drugs. Active internal warming by arteriovenous circulation, esophageal rewarming tubes, and body cavity lavage are also methods that have been described as effective.

Epididymitis usually presents with

acute or subacute (>24 hours) scrotal pain along with dysuria, urinary frequency, urethral discharge, and fever - On physical examination, the scrotum may be red, swollen, and tender. - Elevation of the scrotum may alleviate the pain (Prehn sign). The testis is in a normal vertical position, and a cremasteric reflex is present. - Urinalysis may show pyuria; a urine culture should be obtained. - Nucleic acid amplification testing for C trachomatis and N gonorrhoeae in a urine specimen is recommended for sexually active postpubertal boys. - Epididymitis is usually a clinical diagnosis; however, when the diagnosis is unclear, Doppler ultrasonography may be performed to confirm increased flow to the epididymis. - Treatment of epididymitis includes analgesics, scrotal support, and rest. Antibiotics are indicated for those who are sexually active and those with pyuria, a positive urine culture, or an underlying risk factor for urinary tract infection.

A 2-week-old male infant presents with a 1-day history of loose stools, vomiting, and refusal to feed. He is passing less urine than he did in the morning. Birth weight was 2.8 kg. Physical examination reveals that the infant is lethargic with signs of dehydration. Anterior fontanelle is depressed; heart rate is 180/min; BP is 54/34 mm Hg; respiratory rate is 76/min. Capillary refilling time is 5 seconds. Abdominal examination is normal. Blood examination shows Hemoglobin 16 gm/dL, total leukocyte count is 12,400/mm3, polymorphs 62%, lymphocytes 34%, eosinophils 2%, monocytes 2%, and CRP-negative. Serum sodium 120 mEq/L, potassium 6.8 mEq/L, blood urea 3.5 mg/dL, creatinine 0.8 mg/dL, bicarbonate 15 mEq/L, and platelet count 200,000/mm3. Urinalysis shows brownish granular casts, urinary osmolality of 295 mOsmol/kg, and urinary sodium of 65 mEq/L. What is the most likely diagnosis?

acute tubular necrosis - Urinalysis shows brownish granular casts, urinary sodium of 65 mEq/L (normal 10-40 mEq/L), and urinary osmolality of 295 mOsmol/kg (normal is 300 mOsmol/kg). - Considering the clinical features and biochemical investigations, the above infant has tachycardia, tachypnea (acidotic breathing), poor perfusion (impending shock), hyponatremia, hyperkalemia, low bicarbonate level, and raised urea and creatinine levels (showing features of acute renal failure). - The most likely diagnosis is acute tubular necrosis (ATN) following an episode of acute diarrhea, vomiting, dehydration, and shock. The presence of brownish granular casts in the urine with increased urinary sodium and lower urinary osmolality further support the diagnosis. - A significant increase in the serum creatinine suggests renal insufficiency. ATN due to dehydration and volume depletion is the most common cause of acute renal failure in an infant. Impaired tubular function in ATN results in impairment of reabsorption of water and sodium, leading to increased sodium excretion and low urinary osmolality. - There may be a significant increase of blood urea nitrogen (BUN) with little or no change in serum creatinine with volume depletion without intrinsic renal damage. - ATN may also occur in seriously sick children and in those exposed to nephrotoxic agents like aminoglycosides or to ischemic insults.

Boy with a history of prior sore throat, cola-colored urine, hematuria, proteinuria, hypertension, azotemia, elevated level of antistreptolysin O, and a low complement 3 level—has acute poststreptococcal glomerulonephritis (PSGN). The presence of edema and hypertension reveal that he has fluid retention. The most appropriate treatment is to

administer intravenous furosemide - Poststreptococcal glomerulonephritis is a self-limiting condition with a good prognosis - Fluid overload usually resolves in 10 days; gross hematuria in 1 to 3 weeks; and hypertension, renal dysfunction, and proteinuria in 3 to 4 weeks - Serum complement levels return to normal values in 8 to 12 weeks - Microscopic hematuria can take more than 1 year to resolve completely

Iron supplementation guidelines for preterm infants ...

all preterm infants need 2 mg/kg elemental iron per day for past 12 mos of life - can be supplied through formula - start by 1 mo in breastfed infants

Child has a congenital defect of the cribriform plate, which can predispose him to recurrent meningitis. Recurrent bacterial meningitis can be associated with a congenital or acquired disruption of skull integrity resulting in a cerebrospinal fluid leak. Congenital abnormalities of the anterior fossa, temporal bone, and spinal defects, as well as fractures or a neurosurgical procedure involving the nasal or otic bones place children at risk for recurrent bacterial meningitis, particularly from Streptococcus pneumoniae. Children at high risk for invasive pneumococcal infection benefit from

administration of pneumococcal polysaccharide vaccine 23 valent, 8 weeks after their last conjugate pneumococcal 13 vaccine - Children with meningitis should be started on empiric vancomycin and a third-generation cephalosporin (eg, cefotaxime, ceftriaxone) until susceptibility data are available. - Vancomycin should be discontinued if the species identified is susceptible to β-lactams (eg, penicillins or cephalosporins). - Noninvasive infection (eg, acute otitis media, sinusitis, or uncomplicated community-acquired pneumonia) can be treated with oral amoxicillin.

Children at high risk for invasive pneumococcal infection benefit from

administration of pneumococcal polysaccharide vaccine 23 valent, 8 weeks after their last conjugate pneumococcal 13 vaccine - congenital or acquired defect of the skull resulting in a cerebrospinal fluid leak predisposes a child to recurrent pneumococcal meningitis - children at risk for invasive pneumococcal disease should receive pneumococcal polysaccharide vaccine in addition to the routine conjugate pneumococcal vaccination series. - streptococcus pneumoniae is responsible for infections ranging from noninvasive infections (eg, acute otitis media, sinusitis, conjunctivitis, uncomplicated community-acquired pneumonia) to invasive infections (eg, meningitis, orbital cellulitis, bacteremia, complicated pneumonia [empyema, necrotizing])

Relying on family history to detect cardiovascular risk factors and determine who should be screened for dyslipidemia has not been demonstrated to be very effective. Therefore, universal screening is recommended by the AAP between 9 to 11 years of age and again after age 16 years. Lipoprotein levels fluctuate with age. They reach a steady state around age 10 years, prior to decreasing at puberty. Those with risk factors for cardiovascular disease, including a positive family history, should undergo screening starting at

age 2 years with a fasting lipid profile These risk factors include: - Obesity - Hypertension - Diabetes - Conditions or medications associated with secondary dyslipidemia - Family history of dyslipidemia (total cholesterol >240 mg/dL [6.22 mmol/L]) - Family history of myocardial infarction; angina; coronary artery bypass, graft, or stent; angioplasty; or sudden cardiac death in a parent, grandparent, aunt, or uncle at <55 years for males or <65 years for females

Girl has right facial swelling arising from a right upper molar dental abscess, a type of soft tissue odontogenic infection. Signs and symptoms supporting the diagnosis of dental abscess include fever, hot and cold sensitivity, right upper maxillary edema, the collection of purulent fluid on the right upper gingiva, right submandibular lymphadenopathy, and lack of upper respiratory symptoms or abnormal findings on otologic and pharyngeal examination. Furthermore, girl is well appearing and tolerating oral intake well. For her soft tissue odontogenic infection, the treatment of choice is

amoxicillin-clavulanate - Ultimately, the girl will require evaluation by a dentist and possible drainage of the abscess or extraction of the tooth, but empiric oral antibiotic therapy should be started immediately with amoxicillin-clavulanate. Azithromycin may be an acceptable alternative for the penicillin-allergic child, but it is not the treatment of choice; clindamycin may be a better alternative in the child with a penicillin allergty

Child's history and physical examination findings are consistent with complicated rhinosinusitis with periorbital cellulitis. He should be treated with an oral antibiotic that covers Streptococcus pneumoniae and Haemophilus influenzae. Best med to administer is oral...

amoxicillin-clavulanic acid

Initial empiric tx for suspected bacterial meningitis in neonates under 7 years of age

amp + cefotaxime

Recognize listeria meningitis of neonate, in which tx is

ampicillin

Patients who have acute lymph node enlargement suspicious for infection (eg, tender to palpation, fluctuant, erythematous), but without another source of infection, may be given

an antibiotic trial to cover Staphylococcus aureus, group A Streptococcus, and possibly Bartonella - If the enlargement does not resolve or is chronic, a complete blood cell count, serologies for specific infectious organisms, inflammatory markers, and markers for cell turnover may be recommended - If any results are concerning, or the cause of lymph node enlargement is still elusive, patients should be referred for specialist consultation for further evaluation

The initial empiric antimicrobial therapy in cases of septic arthritis beyond the neonatal period should include

an antistaphylococcal agent, either a β-lactamase-resistant penicillin (eg, nafcillin, oxacillin), a first-generation cephalosporin (eg, cefazolin), or clindamycin or vancomycin - In children aged 5 years or younger, in addition to S aureus, empiric coverage for K kingae with ampicillin-sulbactam or second-generation or third-generation cephalosporin must be considered - Empiric coverage for H influenzae type b with ceftriaxone is also warranted for unimmunized patients. - In sexually active adolescents, empiric coverage should include agents active against N gonorrhoeae (such as ceftriaxone).

Treatment of pityriasis alba is with

an emollient or a short course (7 days) of a low-potency topical corticosteroid

When is rotateq preferred over rotarix?

anaphylactic allergy to latex

A 16-year-old adolescent girl is having a health supervision evaluation. She has bipolar disorder, and at the time of her last visit, was being treated with aripiprazole by her psychiatrist. She discontinued this medication 6 months ago, because she did not like the adverse effects (insomnia and weight gain). Instead, she has been smoking marijuana 3 times per day to manage her symptoms. Her mother is aware of her marijuana use and is supportive of her daughter using a "natural" plant-based drug to control her mood, as opposed to a psychiatric medication. The patient was recently caught with marijuana at school and expelled. She is currently enrolled in an alternative school, but has not been attending regularly because of her marijuana smoking schedule. She plans to start homeschooling and online courses. The counselor at her former school filed a report with child protective services, and the patient and her mother have been interviewed by a caseworker. The case is currently open, but the family expects it will soon be closed. The mother agrees that her daughter's marijuana use is having a negative impact on her education, but is not sure how to help her. This patient's practice of self medication with marijuana is a concern for the primary care physician. Of the following, the BEST next step in this adolescent's management is to

assess the mother's readiness for change, including the possibility of signing her daughter into a drug rehabilitation program without her assent - This girl is self-treating her bipolar disorder and has signs of cannabis use disorder based on the frequency of daily use, failure to attend school, and continued use despite the negative effects it has had on her life. She would benefit from a drug rehabilitation program to address her substance use, but may need parental intervention if she is not willing to go on her own and believes she does not have a problem.

A 3-month-old male infant is presented in the hospital clinic for a bulge in the scrotum. The parents say that the bulge has appeared and spontaneously disappeared 7-8 times over 1 month. They believe its appearance may be associated with crying. Since yesterday, the bulge has remained constant, and the patient appeared to be in discomfort for the first time. Examination reveals a firm tender bulge in the right inguinal region and upper scrotum; the bulge is slightly red and edematous. Transillumination is negative. On auscultation, bowel sounds can be heard in the right scrotum. The abdomen is benign. The left testis is palpable in the scrotum, but examination of the right hemiscrotum is unsatisfactory due to discomfort. What is the most appropriate next step in management?

attempt to reduce the mass - incarcerated inguinal hernia with bowel is present in the scrotal sac - to avoid bowel ischemia, there must be an immediate attempt to reduce the mass: an attempt to return the bowel to the abdominal cavity - oral sedation may facilitate the procedure. - most pediatric inguinal hernias are indirect, caused by the protrusion of intra-abdominal contents (eg, bowel, omentum) through a patent processus vaginalis (the embryonic structure through which the intra-abdominal testis passes in its descent to the scrotum). If the processus vaginalis fails to obliterate completely, an indirect inguinal hernia may result. - If the patient is not toxic, incarceration must be treated emergently with attempts to reduce the hernia. Since they frequently occur, definitive surgery must be scheduled promptly.

Hearing screen recommended in 4 mo old ...

automated auditory brainstem response - <6 mos old = ABR or OAE

A 17-year-old adolescent boy in foster care is seen for a health supervision visit. He has attention-deficit/hyperactivity disorder, depression, and a learning disability. The boy's symptoms have been under poor control. He does not participate during sessions with his therapist and does not take his prescribed medication regularly. Foster care ends in the state at age 18 years, and there is concern that he will not be successful in addressing his mental health needs or living independently when he is no longer in foster care. He states that he can take care of himself. Of the following, the core ethical principle MOST applicable to this situation is

autonomy

Inheritance of von willebrand disease

autosomal dominant - type I = most common - type 2 and 3 = autosomal recessive

Inheritance pattern of NF1

autosomal dominant manner with 100% penetrance - demonstrates both inter- and intrafamilial variability in expression The clinical diagnosis of NF1 is made when at least 2 of the following features are present: - Café au lait macules (CALs) (at least 6; larger than 5 mm prepubertal, larger than 15 mm postpubertal) - Axillary and/or inguinal freckling - Cutaneous neurofibromas (at least 2) - Iris Lisch nodules (at least 2) - Plexiform neurofibroma (at least 1) - Optic glioma - Osseous lesion (sphenoid dysplasia or tibial pseudoarthrosis) - First-degree relative with NF1 as defined by the above criteria

Inheritance pattern of cystinosis

autosomal recessive - hereditary metabolic dz - belongs to family of lysosomal storage disorders - accumulation of cystine within lysosomes - CTNS gene encodes damaged or absent cystinosin protein

Screen time recommendation for children under 18 months old ...

avoid all screen time/media for children under 18 mos, except video chatting

A 14-year-old boy is brought to the office by his mother for behavioral concerns. He has refused to go to school for the past week. Before that, he was frequently tardy, telling his mother that he was not feeling well or that he did not have clean clothes to wear. For a short time, she could get him to school without conflict if they arrived well in advance of the other students. The adolescent states that he feels like he is being "judged" by the other students when he arrives on campus. His mother states that he does not want to go on family outings and that he appears self-conscious and nervous when in public. The boy admits to feeling anxious and distressed in social situations and has been having a difficult time falling asleep due to excessive worries. He recently asked his mother if he could be homeschooled. What is a risk factor for this condition?

behavioral inhibition - social anxiety disorder - neurotransmitters γ-aminobutyric acid (GABA), serotonin, and norepinephrine have been associated with the development of anxiety disorders. γ-Aminobutyric acid acts through inhibition of neurons in the amygdala. Norepinephrine and serotonin regulate brain circuits affecting mood and response to stress or fear - evidence-based treatments for anxiety disorders include cognitive behavioral therapy (CBT) and medication. The first-line treatment recommendation is CBT - when CBT is not sufficient to treat the symptoms, is inaccessible, or when symptoms are severe, medications can be considered. While some selective serotonin reuptake inhibitors (SSRIs) are approved by the United States Food and Drug Administration for treating depression and OCD in children, they are not yet approved for treating anxiety disorders in children

pathergy test is useful in dx of

behcet disease

A 4-month-old infant is seen for a health supervision visit. She does not track voices or respond to loud noises. She failed both her newborn hearing screen and a follow-up audiology evaluation in both ears. An otolaryngologist and audiologist confirmed bilateral sensorineural hearing loss. Her father asks about devices that could help improve her hearing. Of the following, the MOST appropriate device to consider for this infant is/are

behind-the-ear hearing aids - may be provided for infants as young as age 2 months and are the first-line intervention for infants with hearing loss - bone-anchored hearing aids are appropriate for children at least 5 years old with conductive hearing loss

A 6-day-old female neonate is brought to the emergency department for evaluation of abnormal movements. She was born at 39 weeks' gestation to a gravida 2 para 2 mother with an uncomplicated pregnancy via normal spontaneous vaginal delivery. Apgar scores were 9 and 9 at 1 and 5 minutes, respectively. Her neonatal course was unremarkable, and she was discharged home on the second day after birth. On the day of presentation, her parents noted abnormal movements that were described as asymmetric twitching of her legs with accompanying lip smacking and perioral cyanosis with periods where she appeared to hold her breath. These periods would last up to 2 minutes before self-resolving with return to her typical feeding and activity. Family history is significant for her mother and a maternal uncle with seizures around the time they were born, which subsequently resolved. The neonate's vital signs are within normal limits. She appears alert and has normal neurological examination findings. During examination, she has a typical event where her eyes open and she stares straight ahead with clonic movements of her legs and lip smacking with apnea. Intravenous phenobarbital (20 mg/kg) is administered, and the movements stop. Results of noncontrast computed tomography of the head are normal. Results of a complete blood cell count, comprehensive metabolic panel, urinalysis, ammonia level, C-reactive protein, lactate, serum and urine toxicology screen, and capillary blood glucose are within normal limits. Lumbar puncture yields clear cerebrospinal fluid. Laboratory data from cerebrospinal fluid analysis are shown: Laboratory testResult - White blood cell count 4/μL - Lymphocytes 75% - Red blood cell count 0/μL - Protein 120 mg/dL - Glucose 100 mg/dL (5.6 mmol/L) Cultures are pending. Of the following, the MOST likely diagnosis is

benign familial neonatal convulsions - 1 of 2 recognized benign neonatal seizure syndromes = affected neonate will present with focal clonic seizures responsive to antiepileptic medication - seizures typically resolve spontaneously in the first few days to week after birth - positive family history of neonatal seizures distinguishes benign familial neonatal convulsions from benign neonatal seizures, or "fifth-day fits" - key features supportive of the diagnosis of benign familial neonatal convulsions include a positive family history and lack of clinical, radiologic, or laboratory risk factors for seizure in a neonate with normal neurological examination findings and normal interictal electroencephalography (EEG) background - prognosis is excellent with a benign clinical course - antiepileptic medication can be withdrawn after a few weeks to months of therapy

A 7-year-old boy is brought to the emergency department for evaluation of a seizure. His mother heard a thud in his room at 6 AM and found him lying on the floor next to his bed. She noted that he had generalized shaking for 3 minutes, after which he was very tired and confused. In the emergency department, he is groggy but arousable. His neurologic examination findings are normal. A computed tomography scan of the head as well as results from a complete blood cell count, comprehensive metabolic panel, and serum and urine toxicology are all normal. On further questioning, the boy recalls several self-resolving episodes of waking in the middle of the night unable to talk, with associated drooling and facial twitching. Of the following, the boy's MOST likely diagnosis is

benign rolandic epilepsy - also known as benign childhood epilepsy with centrotemporal spikes - common childhood focal motor epilepsy syndrome, typically presenting between the ages of 5 and 10 years - infrequent brief focal motor seizures that occur predominantly in sleep - neuroanatomic location of the rolandic region near the central sulcus, the clinical presentation of seizure is typically described as facial and arm twitching, speech arrest with accompanying oropharyngeal guttural sounds, and hypersalivation with preservation of consciousness - spontaneously remit in adolescence, usually by 16 years of age - diagnosis of BRE is confirmed by electroencephalography demonstrating biphasic independently bilateral centrotemporal spikes on a normal background, often identified in light sleep - anti-epileptic medication is offered when seizures are frequent; initiation requires shared decision-making with the family regarding potential adverse effects of medication balanced against seizure frequency, the impact on the child, and parental concern - common antiepileptic medications options include oxcarbazepine and levetiracetam

A 9-month-old infant presents with history of progressively increasing pallor, easy fatiguability, shortness of breath, irritability, and a lack of appetite for the last 3 months. Baby was born at full term and was delivered by normal vaginal route. Birth weight was 3 Kg. Length was 49cm. And no congenital malformations were detected. Baby was accepting feeds well until about 5-6 months. Mother has been giving some multivitamin and iron preparations to the baby for the last 1 month but without a satisfactory response. Physical examination reveals that the infant has moderate pallor, is cranky, and resents examination. Grade II ejection systolic murmur is heard in the pulmonary and mitral area. Abdominal examination reveals liver 3 cm below costal margin and spleen 2.5 cm below costal margin. Respiratory system is normal. Blood examination shows Hemoglobin 6 gms%, Total leukocyte count 8,100/mm³, Polymorphs.60%, Lymphocytes 35%, Eosinophils .3%, and Monocytes.2%. Reticulocyte count 5%, peripheral smear shows microcytic hypochromic picture with anisopoikilocytosis. Platelet count 210,000/mm³ and Serum iron 200 micrograms/dL. What is the most likely diagnosis?

beta thalassemia - moderate anemia with hepatosplenomegaly - microcytic hypochromic blood picture - serum iron level is increased in the above infant, which supports the diagnosis of thalassemia - mutation of beta globin chain results in beta thalassemia and mutation of alpha globin chains leads to alpha thalasemia - serum iron is raised due to break down of endogenous or transfused red cells. This excess of iron is deposited in the liver, spleen, heart, and endocrine glands (hemosiderosis) leading to damage and impairment of function of these organs. - typical thalassemia facies with frontal and parietal prominence, depressed bridge of the nose, and hypertrophy of maxilla appears after 1 year of age. There is growth retardation. - peripheral smear shows microcytic hypochromic anemia with fragmented red cells, and nuclear fragments like Howell Jolly bodies and Heinz bodies may be seen. A large number of normoblasts are seen in the peripheral smear. - Bone marrow is hypercellular with erythroid hyperplasia. - Treatment consists of repeated blood transfusions to maintain hemoglobin levels between 10-12gm/dL and iron chelation therapy. - Splenectomy may be indicated when the packed cell transfusion requirement is more than 250ml/Kg/year. - Bone marrow transplantation from an HLA matched sibling can result in cure. - Beta thalassemia can be diagnosed prenatally by chorionic villus sampling between 10-12 weeks of gestation.

The average age toilet training begins in the United States is when?

between 2 and 3 years of age

The first dose in the rotavirus series may be given when ?

between 6 weeks and 14 weeks and 6 days of age - If an infant has not received the first dose by 15 weeks and 0 days, the infant should not begin the series - At least a 4-week interval is required between doses, and the vaccine may not be administered after 8 months and 0 days - Typically, RV5 is given at 2, 4, and 6 months of age, or RV1 is given at 2 and 4 months of age - Importantly, the rotavirus vaccine should not be administered while an infant is still in the neonatal intensive care unit due to the risks associated with viral shedding in the stool after the vaccination

A 16-month-old boy is brought to the emergency department for trouble breathing. His mother states that he was well until approximately 48 hours ago when nasal congestion and wet cough began. On physical examination, the boy has a heart rate of 160 beats/min, respiratory rate of 60 breaths/min, oxygen saturation of 88% on room air, and blood pressure of 98/55 mm Hg. He is in moderate respiratory distress with intercostal and subcostal retractions. Lung examination reveals coarse rhonchi and scant wheezing with good aeration throughout. Although somnolent, the boy interacts with his mother appropriately and cries when a blood specimen is obtained via arterial puncture. The remainder of his physical examination findings are unremarkable. Arterial blood gas reveals a pH of 7.30, PCO2 of 55 mm Hg, and PO2 of 60 mm Hg. Of the following, the BEST next management step for this boy is to initiate

bilevel positive airway pressure ventilation - Noninvasive positive-pressure ventilation (NIPPV) for acute respiratory failure in children is associated with rapid clinical improvement and a decreased need for intubation. Thus, a trial of NIPPV with bilevel positive airway pressure (BiPAP) would be the next best step in management. - A nasal or full face mask is used to administer BiPAP and allows for independent adjustment of inspiratory positive airway pressure (iPAP) and expiratory positive airway pressure (ePAP). - Inspiratory positive airway pressure promotes lung inflation during inspiration, leading to alveolar recruitment, and ePAP prevents end-expiratory alveolar collapse with the application of continuous end-expiratory pressure.

A 15-year-old adolescent girl is evaluated in the office for a 2-month history of chronic nonproductive cough associated with fatigue, 5- to 10-lb weight loss, low-grade fever, and mildly painful nodules on her arms and legs. She has not traveled out of the state or had any known sick exposures. On pulse oximetry, her oxygen saturation is 98% on room air. On physical examination, the girl has no respiratory distress, and no crackles or wheezes are heard. She has several 1- to 3-cm diameter, tender, nonerythematous, nonexcoriated nodules on the extensor surfaces of both arms and legs. Chest radiography suggests hilar adenopathy. Computed tomography additionally demonstrates interstitial changes in the pulmonary parenchyma. Pulmonary function testing shows a mild restrictive pattern with a decrease in forced vital capacity and total lung capacity. Carbon monoxide diffusion is slightly decreased for age and hemoglobin concentration. A tuberculin skin test has a negative result 60 hours after placement. Of the following, the BEST next step in the evaluation of this adolescent is

biopsy of skin nodule - The girl in the vignette likely has sarcoidosis, with both pulmonary and nonpulmonary manifestations. - The presence of cough and fatigue with hilar adenopathy and restrictive lung disease may be nonspecific, but for this adolescent with tender peripheral nodules, sarcoidosis is the most likely diagnosis. - Most appropriate next step in evaluation is biopsy of one of the nodules to demonstrate noncaseating granulomas. - Sarcoidosis is a multisystem disease that usually presents between 20 and 60 years of age. - Sarcoidosis is 4 times more common in black than white populations, and more common in women than men. - Black women are most commonly affected; they may develop disease up to 10 years earlier than white patients. It is estimated that the lifetime risk for sarcoidosis in blacks in the United States is 2.4% compared with a lifetime risk of 0.85% in whites. - The most specific diagnostic test for sarcoidosis is biopsy of an affected organ; for the adolescent in the vignette, biopsy of a skin nodule is the least invasive approach to obtaining a tissue diagnosis. - After the lungs, the skin, eyes, and lymph nodes are the most commonly involved organs. - The liver, spleen, parotid glands, and bone marrow may also be sites of noncaseating granulomas characteristic of sarcoidosis.

Signs and symptoms of colitis include

bloody diarrhea nocturnal stooling urgency tenesmus

The girl in the vignette has a mild restrictive pulmonary process with decreased forced vital capacity (FVC) but normal forced expiratory volume in 1 second (FEV1) and FEV1/FVC ratio. Measurement of total lung capacity (TLC) will confirm a restrictive process. Lung volumes can be measured by:

body plethysmography (body box) or nitrogen or helium dilution techniques - Decreased forced vital capacity may represent restrictive lung disease, which can be corroborated by measurement of total lung capacity. - When lung volumes are normally distributed, forced vital capacity is a reasonable marker for total lung capacity. - Forced expiratory volume in 1 second (FEV1) and ratio of FEV1 to forced vital capacity are measures of airflow and when reduced indicate obstruction.

Adolescent presents with symptomatic anemia (exercise intolerance), leukopenia with very severe neutropenia (absolute neutrophil count < 200/µL), and thrombocytopenia. A decrease in more than 1 blood cell line (leukocytes, erythrocytes, or thrombocytes) is referred to as pancytopenia. This patient has been symptomatic for a long time (> 6 months), she does not have splenomegaly, she is mildly macrocytic (an estimate of an appropriate mean corpuscular volume is 72 fL plus age in years), her reticulocyte count is inappropriately low, and there are no leukemia blasts noted on her peripheral blood smear. This combination of symptoms, signs, and laboratory findings is most consistent with a diagnosis of aplastic anemia. The diagnostic test of choice for aplastic anemia is

bone marrow biopsy with an assessment of percent cellularity - While acute leukemia most often presents with a hypercellular marrow, aplastic anemia presents with a markedly hypocellular marrow - Flow cytometry is the diagnostic test of choice for leukemia because it can distinguish cells with aberrant surface marker expression

15 yo boy presents with knee pain that continues to interfere with athletic activities. Left knee has bothered him "off and on" for several mos. Pain increases with activity and when he "stretches his legs during warm-ups." His knee will also "sometimes lock up," making it impossible for him to continue activity. On exam of left knee, there is soft tissue swelling and joint line tenderness. During internal rotation of tibia with knee flexed to 90 degrees, he complains of increased pain as the knee is slowly extended. Full extension of knee is limited. What findings is likely to be identified on plain radiograph during further eval?

bony fragment in medial condyle of femur - osteochondritis dissicans (OCD) - focal area of subchondral bone undergoes necrosis - medial femoral condyle = most commonly affected - stiffness after periods of nonuse - Wilson sign = internally rotating tibia with knee flexed to 90 degrees, followed by careful extension - is conservative management fails, referral to arthroscopy warranted

A 14-year-old adolescent girl is brought to the emergency department 30 minutes after she collapsed while walking with a friend. She is awake and able to follow commands but is not speaking. Her pupils are equal, round, and reactive to light; extraocular movements are intact, and she can close her eyes completely. Her smile is asymmetric. She is moving her left arm and leg vigorously; there is minimal movement in the right arm and leg. She is unable to ambulate. A head computed tomography scan is normal. The girl is admitted to the pediatric intensive care unit for observation with a diagnosis of seizure with a post-ictal paralysis. Over the next 12 hours, the girl's hemiparesis remains unchanged. Her friend reports that the girl collapsed while walking; she did not lose consciousness, had no abnormal movements, and was unable to get up or speak. Of the following, the BEST next diagnostic study to order in the management of this child is

brain MRI - The preferred study is magnetic resonance imaging (MRI) of the brain and magnetic resonance angiography (MRA) of the head and neck, which can show ischemic changes within minutes of the event. A computed tomography (CT) scan of the head can be performed when urgent MRI is unavailable; however, CT results can be normal in the setting of acute stroke, necessitating further imaging. - Clinical symptoms include hemiparesis, facial weakness, disturbances of language and speech, ataxia, or vision changes. - Children with an acute stroke are more likely than adults to have nonlocalizing symptoms and signs such as altered mental status and seizures. - Stroke risk factors include sickle cell disease, moyamoya and other vasculitides, congenital heart disease, prothrombotic conditions, and infection (especially of the head and neck).

A 16-year-old adolescent boy is being seen for his annual health supervision visit. His mother is concerned that he has recently quit playing on both his school and club soccer teams. He has always been an A student but is failing two classes at school, and he has started having outbursts at home and speaks rudely to his parents and younger sister. The mother expresses her frustration and inquires whether her son needs a psychiatric evaluation. During the confidential interview, he reports that his mother is exaggerating and that he can get his grades up in the classes he is failing. He quit playing soccer because it no longer interests him. He discloses that over the last 6 months he has smoked cigarettes once or twice per week, smoked marijiuana daily to help him sleep at night, and has tried ecstasy once. He reports no other drug use and does not drink alcohol. He does not feel that he has a problem and says he knows he can stop using any of these substances at any time. When asked if it would be all right to discuss his substance use with his mother, he becomes agitated and does not want anything he discussed in private to be disclosed. Of the following, the BEST next step in treatment is to

break his confidentiality and discuss the substance use concerns with his mother because it is unlikely he will be able to stop using on his own - The best plan to ensure that this adolescent remains safe and receives appropriate services would be to break confidentiality and involve his mother in the plan of care - In situations of substance abuse in which patient well-being is at risk, it is appropriate to break confidentiality to allow care to occur

A 15-year-old girl with a history of mild asthma has had worsening episodes of cough, wheezing, and increasing bloody sputum over the past 5 months. She denies weight loss, decreased appetite, lethargy, or travel. She has increased her bronchodilator use, but she had not sought further care. Her mother has noted facial flushing with sweating that sometimes appears when she feels stressed—brief at first but lasting longer now. On exam, her respiratory rate is 32 breaths/min, temperature is 98.6°F, heart rate 84 bpm, BP 114/76 mm Hg, oxygen saturation is 94%. Her throat is clear, RRR without murmur; on auscultation, breath sounds over the left hemithorax are diminished without retractions or wheezes; there are few fine crackles at the base. Right side is clear. Remainder of the exam is normal. Chest X-ray reveals a round area of increased opacification near the right hilar region. CBC shows normal white count and differential. What diagnosis would most easily explain the patient's symptoms?

bronchial carcinoid tumor - most common primary malignant lung tumor in children - tend to arise in the perihilar region - one of the hormones, serotonin, is thought to cause carcinoid syndrome, which consists of episodic flushing, wheezing, and diarrhea - patients can present with recurrent pneumonia, cough, hemoptysis, wheezing, asthma, or chest pain - resection is the preferred treatment for localized tumors; survival rates, when localized, are >90%=

Kiddo with CF. Has hx of recurrent sinusitis and pna, barrel chest, nasal polyps. What could you see on CXR?

bronchiectasis

A 3-year-old girl presents with a 4-day history of cough, fever, and dyspnea. Chest X-ray showed right lower lobe consolidation. She showed some improvement with antibiotics and supportive therapy. After 6 weeks, she again presents with similar issues, and the X-ray showed increased consolidation of the right lower lobe. What investigation will most likely provide new information to guide treatment?

bronchoscopy - Examination of the bronchoalveolar lavage (BAL) fluid will help in isolation of the causative organisms for appropriate management of the case. It can also detect a foreign body in the tracheobronchial tree, which can be successfully extracted through the bronchoscope. - Recurrence of pneumonia in the same localized area is suggests aspiration of a radiolucent foreign body not visualized on chest X-ray. There may not be a positive history of aspiration of a foreign body, but choking, episodes of coughing, and wheezing highly suggest it. - In non-resolving pneumonia or recurrent pneumonia, the BAL fluid is subjected to microbiological evaluation and cytopathology for isolation of the causative organisms. - Bronchoscopy is helpful in diagnosis of obstruction due to any endobronchial pathology or any congenital malformation in the trachea and bronchi, which may cause recurrent pneumonia.

7 yo girl complains of 5 day hx of pain in her right arm, which first began after playing baseball with her older brothers. Eval includes plain radiograph of her right arm, which reveals a lucent lesion with homogenous matrix resembling "ground glass" appearance d/t loss of normal trabecular pattern in shaft of humerus. Finding are associated with ...

cafe au lait spots + precocious puberty - evidence of fibrous dysplasia - McCune Albright syndrome

Calves in duchenne muscular dystrophy

calf enlargement

6 mo old + dev'tal delay + regression + poor fixation + no tracking + hypotonia + MR: diffuse symmetric white matter changes with iv't of U fibers + MR spectroscopy with increased peak of N-acetylaspartic acid. Dx?

canavan dz - AR - def. of aspartoacylase --> accum of N-acetylaspartic acid in brain - jews - 17p13 alteration - onset = 10 wks - 4 mos - megancephaly - elevated urinary N-acetylaspartic acid level - no specific tx

2 day old infant + 2 day hx of bilateral serosang drainage from eyes + baby born at home + received prophylactic erythromycin ointment. Most likely cause of infant's symptoms?

chemical conjunctivitis - rxn to erythromycin ppx - lack of purulent eye discharge

A 6-year-old boy with history of acute lymphoblastic leukemia undergoing chemotherapy presented with fever 1 week ago. During his recent maintenance regimen therapy, he has had a prolonged neutropenia closely monitored with twice-weekly blood counts. He was continued on trimethoprim-sulfamethoxazole 3 times a week for prophylaxis against pneumocystis before the development of this febrile illness. Initial chest X-ray showed bilateral infiltrates, and he was started on broad-spectrum antibiotic therapy. Despite 5 days of therapy, his fever persisted, and his chest X-ray showed worsening of infiltrates. He underwent a diagnostic bronchoscopy with bronchoalveolar lavage; silver staining reported oval budding organisms 2-6 μm in diameter with pseudo-hyphae. What is the most likely causative organism in this patient?

candida - most common of the fungal pathogens and are a frequent cause of nosocomial infections - Neutropenic patients, low birth weight infants, or premature neonates whose mothers have Candida chorioamnionitis are at risk for pulmonary dissemination. - More than 95% of strains of C. albicans are sensitive to fluconazole, but amphotericin B is the preferred initial therapy for most cases.

Respiratory alkalosis + hyperammonemia + low urine orotic acid =

carbamoyl phosphate synthetase deficiency

Who inflicts the majority of child maltreatment?

caregivers related to the child

What to do if there is snuffbox tenderness, but x-ray is negative...

cast them in thumb spica and recheck in 2 weeks --> initial x-rays can miss it - immobilizing can help it heal and prevent surgery

For children who are not toilet trained, urine collection via...

catheterization or by suprapubic aspiration - Because a clean-catch specimen cannot be collected from a child who is not toilet trained, a diagnosis of UTI should not be made based on urine culture results from a bagged-urine specimen - According to the guideline, any bacterial growth in a suprapubic aspirate or 50,000 or more CFU of a single organism in a catheterized urine sample is confirmatory of UTI - In a voided urine sample (bag or clean-catch), more than 100,000 CFU is considered significant; however, growth of more than one organism on the urine culture suggests contamination

Boy stepped on dirty nail. OTC med doesn't work. Soaked. Now infected foot for 4 days that worsened after getting in hot tub. Need for hospitalization for abx. Septic. Appropriate monotherapy?

ceftazidime - good gram positive and gram negative coverage - antipseudomonal coverage - pseudomonal infection via hot tub - maybe early osteo

In an otherwise well-appearing patient with sickle cell disease and fever, what would provide adequate coverage?

ceftriaxone

A 10-year-old girl was seen in the ED last night and diagnosed with probable meningococcal meningitis. She presented with a 2-day history of headache, vomiting, and fever. She was seen by her primary physician, who diagnosed a viral illness and instructed her parents on symptomatic care and signs to watch for worsening. Later that night, her fever spiked to 103°F, and she began having a purplish non-blanching rash on her extremities. Her parents brought her into the ED, and she was quickly evaluated and admitted for treatment. She has been actively involved at school, participating in sports, dance, and band activities. What antibiotic should be given to this patient for treatment and to eradicate possible carriage?

ceftriaxone - Effective treatment and eradication of nasopharyngeal carriage is accomplished with cefotaxime or ceftriaxone (100 mg/kg per day in 1-2 doses) for 7 days. - Once treatment is completed and the children recover, they may then return to school or childcare.

A 10-year-old girl with type 1 diabetes is seen for decreased appetite and fatigue over the past month. She eats three meals per day, but she consumes small portions because of nausea. She has not had abdominal pain, vomiting, diarrhea, constipation, or recent illness. Her diabetes is treated with rapid-acting insulin via an insulin pump. She has had a 2.3-kg weight loss since her last health supervision visit 4 months ago. Her vital signs and the remainder of her physical examination findings are normal. Her most recent hemoglobin A1c level was 7.9% 2 months ago. Today, her hemoglobin level is 10 g/dL (100 g/L) with a mean corpuscular volume of 72 fL. Of the following, the MOST likely underlying diagnosis causing this girl's symptoms is

celiac disease - decreased appetite, fatigue, nausea, weight loss, and microcytic anemia - occurring in up to 16% of those with type 1 diabetes, celiac disease is the second most common associated autoimmune condition after autoimmune thyroid disease - guidelines recommend screening children with type 1 diabetes for celiac disease around the time of their diabetes diagnosis, with repeat screening within 2 years of diagnosis, then after 5 years - a tissue transglutaminase IgA antibody level is the recommended screening test

Pt brought in for worsening pain in both legs. Describes pain as "burning" and worsens with physical activity and hot or cold temps. Doing well in school. Vitals normal. PE = several 1-2 mm reddish and bluish spots in periumbilical area. Hepatomegaly noted. What molecule accumulates in muscle and cardiac myocytes in this condition?

ceramide trihexose / Gb3 - Fabry disease - X-linked - cutaneous = angiokeratomas - peripheral neuropathy

Most deaths during diabetic ketoacidosis (DKA) in children are due to ...

cerebral edema - HA = concerning - usually presenting 4-12 hours after starting treatment. - occurs in 0.5-1% of DKA episodes and has a mortality rate of 21-24%, with 15-26% of survivors left with permanent neurological injury - exact cause in unknown, but theories include cerebral ischemia and hypoxia, fluid shifts between extravascular and intravascular intracranial compartments, increased cerebral blood flow, and altered membrane ion transport - Risk factors include young age, undiagnosed diabetes, pH <7.2, lower serum bicarbonate, higher serum glucose, higher serum blood urea nitrogen, hypernatremia and hypocapnia, lack of sodium change with rehydration, and treatment with bicarbonate - Signs and symptoms are severe headache, sudden deterioration in mental status, bradycardia, hypertension, cranial nerve dysfunction, and incontinence - Immediate treatment with intravenous mannitol should be started if suspected.

Perforated nasal septum associated with hx of chronic congestion and nasal discharge is indicative of cocaine use. Cocaine is a potent vasoconstrictor, which can lead to...

cerebral hemorrhage seizures stroke MI

A 10-year-old boy who had visited a tropical country for 2 months and had returned 2 weeks ago is brought to the hospital with high fever followed by convulsions and unconsciousness. He has pallor and a palpable spleen 2.5 cm below costal margin. There is no significant lymphadenopathy or any skin rash.There are no signs of meningeal irritation. Fundus examination shows retinal hemorrhages, but there is no papilledema.Blood examination shows hypoglycemia.CSF examination is essentially normal. What is the most likely clinical diagnosis?

cerebral malaria - child has presented with high fever, convulsions, and unconsciousness with splenomegaly - hypoglycemia is suggestive of severe and complicated malaria. It is believed to be partly due to large glucose requirement by the malarial parasite and may also be precipitated by quinine-induced insulin secretion. It is associated with increased mortality and neurologic sequelae. - The merozoite stage of malarial parasite multiplies in the erythrocytes, and later these erythrocytes burst to liberate large number of daughter merozoites. This leads to production of cytokines, which are responsible for suppression of hepatic gluconeogenesis, leading to hypoglycemia, and suppression of erythropoesis, causing anemia. - Blood glucose should be frequently monitored in sick children with severe and complicated malaria. - Quinine stimulated hypoglycemia can be prevented by giving quinine infusion slowly over 4 hours in 5% or 10% dextrose.

3 yo boy, recently removed from his home for medical neglect, presents with hx of "draining ear" for indeterminant time. After cleaning external ear canal of purulent material, spherical whitish-gray, cystic-like mass is identified behind left tympanic membrane along edge of posterior-superior quadrant of drum. Dx?

cholesteatoma - progressively enlarging cystic lesion composed of dead keratin debris - usually arise within focal retraction of tympanic membrane - if not removed, may invade and damage both ossicles and temporal bone Indications to refer to ENT include - any whitish-gray mass behind intact tympanic membrane - continued drainage from ear >2 weeks despite appropriate treatment - new-onset hearing loss in previously operated-on ear

A 42-year-old primipara mother at 11 weeks gestation is concerned about the well-being of the unborn child. She has learned that female patients over age 35 have increased chances of having a baby with Down syndrome or other chromosomal defects. She wants to know if the baby will be born with Down syndrome. What test is most appropriate?

chorionic villus sampling - done at 10-12 weeks gestation - can detect chromosomal defects, including the defect that causes Down syndrome - CVS can detect hemoglobinopathies, hemophilia, Duchenne muscular dystrophy, and Tay-Sachs disease - cannot detect neural tube defects - risk of miscarriage is 0.5-1% higher than amniocentesis, but CVS can be performed at an earlier gestation than amniocentesis.

Boy has dysmorphic features and behaviors suspicious for Williams syndrome, which is caused by a deletion at 7q11.23 and would be detected on

chromosomal microarray - Alternatively, if the clinical features of Williams syndrome are recognized, fluorescence in situ hybridization (FISH) using a probe targeted to 7q11.23 can be ordered instead of microarray to detect this deletion

Pt presenting with alopecia areata. Pt is female and adolescent, both more typical for dx of

chronic autoimmune thyroiditis/ Hashimoto

Has been shown to decrease risk of initial strokes in high-risk children

chronic exchange transfusion therapy

A 14-year-old adolescent boy is brought to the office by his mother for evaluation after he underwent computerized neuropsychologic testing at his school as a baseline for potential postconcussion comparison. He performed very poorly on this test, and was advised by the school's athletic trainer that he needs additional evaluation before he will be allowed to participate on his school's freshman lacrosse team. The boy had a concussion last year with complete resolution of symptoms and returned to his previous level of academic performance. A subsequent preparticipation examination was performed, and he was cleared for unrestricted sports participation. He reports no additional injuries. His mother reports no change in personality or behavior and his teachers have not reported any classroom difficulties. He has been involved in high-intensity cardiovascular conditioning and plays for a travel lacrosse team without difficulty. He has attention-deficit/hyperactivity disorder (ADHD) that has been well controlled with atomoxetine 60 mg daily. His neurologic examination findings are normal. Of the following, the BEST next management step for this boy is to

clear him for unrestricted sports participation - Preinjury assessment of neurocognitive function is essential for detecting postconcussion deficits. - Neurocognitive function is best assessed in the medical home during health supervision visits and/or preparticipation examinations. - Clearance for return to participation in contact sports after concussion requires: full symptom resolution, no symptom recurrence with high-intensity physical activity, and return to baseline neurocognitive function and academic performance. - Computerized neuropsychological test results should not be used to diagnose concussion or as the sole criterion for return to sport.

A 14-year-old adolescent boy is brought to the office by his mother for evaluation after he underwent computerized neuropsychologic testing at his school as a baseline for potential postconcussion comparison. He performed very poorly on this test, and was advised by the school's athletic trainer that he needs additional evaluation before he will be allowed to participate on his school's freshman lacrosse team. The boy had a concussion last year with complete resolution of symptoms and returned to his previous level of academic performance. A subsequent preparticipation examination was performed, and he was cleared for unrestricted sports participation. He reports no additional injuries. His mother reports no change in personality or behavior and his teachers have not reported any classroom difficulties. He has been involved in high-intensity cardiovascular conditioning and plays for a travel lacrosse team without difficulty. He has attention-deficit/hyperactivity disorder (ADHD) that has been well controlled with atomoxetine 60 mg daily. His neurologic examination findings are normal. Of the following, the BEST next management step for this boy is to

clear him for unrestricted sports participation Clearance for return to participation in contact sports after concussion requires: - Full symptom resolution - No symptom recurrence with high-intensity physical activity - Return to baseline neurocognitive function and academic performance

A 7-year-old boy was bitten on the hand by a stray dog in his neighborhood yesterday. His parents immediately washed the hand with soap and water. Today the area is red, swollen, and painful with range of motion. He has not had a fever. The dog was captured by animal control officers; it is acting normally and remains in their custody. The child is up to date with his immunizations and has an allergy to penicillin. Of the following, the MOST appropriate therapy at this time is

clindamycin and trimethoprim-sulfamethoxazole orally - Wound infections after an animal bite such as this are usually polymicrobial. The most common pathogens in dog, cat, or mammal bites are Pasteurella multocida, Staphylococcus, Streptococcus, Capnocytophaga, Moraxella, Fusobacterium, and Bacteroides species. - The oral drug of choice for treatment of animal bites is amoxicillin-clavulanate. However, this boy has an allergy to penicillin; the most appropriate alternative treatment for infection caused by a dog bite is clindamycin plus trimethoprim-sulfamethoxazole. - A broad-spectrum cephalosporin plus clindamycin is another acceptable oral alternative.

During health maintenance visit, 10 yo girl noted to have hard, fixed, painless, irregular 1.5-cm x 2-cm mass along lateral aspect of right distal tibia. Although she denies any trauma or associated local or systemic symptoms, she reports that she first noticed swelling 3-4 mos earlier. Radiograph reveals cauliflower-like outgrowth of cortical and medullary bone arising from right distal tibia, which is associated with areas of increased density scattered throughout lesion. Most appropriate next step in lesion?

clinical monitoring with repeat radiograph in 6 mos - osteochondroma = most common type of benign tumor in children

3 yo + bruises over legs, torso, forehead. Had URI 2 weeks ago and has had epistaxis daily since. CBC is normal except that his platelet count is 3000. Exam is otherwise normal and there is currently no active bleeding. What tx is most likely to be helpful>

close observation ITP - no to mild bleeding = observation - severe bleeding = steroids, IVIG, anti-Rh (D) immunoglobulin

A 14-year-old girl presents with a 12-hour history of right eye pain after being struck by a ball while playing outside. Severe eye pain with a foreign body sensation and lacrimation of the right eye began minutes later. Vital signs are normal except for a pulse of 122/min. The sclera of the right eye is injected. Visual acuity is 20/30 on the left, but it could not be tested on the right. The pupil was 2 mm on the left but pinpoint on the right. What is the most appropriate way to confirm the suspected diagnosis?

cobalt blue light exam - Corneal abrasions make the eye red and painful, often with a foreign body sensation. - Meiosis is often present secondary to pain. - The most effective way to confirm the diagnosis of corneal abrasion is fluorescein dye and inspection under cobalt blue light (preferably during slit-lamp examination), under which a corneal abrasion will appear green (yellow under natural light). - Treatment is with ophthalmic antibiotics, anesthetics, and patching.

Study follows children of 9/ll victims over 10 years to assess dev't of PTSD. What type of study design is this?

cohort study - longitudinal study - examines particular outcome in subjects according to presence or absence of exposure

A 2-year-old boy is brought to the emergency department for evaluation of rectal bleeding. He has a 1-month history of intermittent rectal prolapse, which occurs every 2 to 3 days and is easily reducible at home, with no rectal bleeding noted. His first episode of prolapse was easily reduced by a physician in the emergency department. He is an otherwise healthy boy with normal growth, who is not yet toilet trained. He has 1 to 2 stools daily which his parents describe as soft blobs. He has never had a history of infrequent or hard stools. Because of his history of prolapse, the boy has been taking polyethylene glycol 3350 daily for the last month. Physical examination reveals a hemodynamically stable child. Digital rectal examination finds dark red blood and a palpable rectal mass; the remainder of his examination findings are normal. Laboratory evaluation reveals a normal hemoglobin level for age. Of the following, the test MOST likely to confirm the etiology of this boy's condition is

colonoscopy - recurrent rectal prolapse without a history of constipation or cystic fibrosis - viven the finding of a palpable rectal mass, the most likely cause of his recurrent rectal prolapse and rectal bleeding would be a rectal polyp. This could be confirmed on colonoscopy. - rectal prolapse occurs most commonly in children younger than 4 years because of normal anatomic variants in this age group. Children older than 4 years are more likely to have a pathologic condition contributing to prolapse - constipation is the most common cause of rectal prolapse in children, as a result of increased intra-abdominal pressure caused by prolonged sitting and straining during attempts to pass stools - In the acute setting, if the rectal prolapse does not resolve spontaneously, manual reduction should occur. If this is unsuccessful, an urgent surgical consultation is needed - In cases of constipation, the most common cause of rectal prolapse, laxative therapy should begin, as well as limiting toilet sitting to 5 to 10 minutes, using a child insert on the toilet seat, and using a 2-step stool to raise the feet to promote complete stool evacuation

Symptom or deficit in voluntary motor function in pts without anatomic or physiologic basis

conversion disorder

The most common cardiac conduction abnormality in infants with NSLE is =

complete congenital heart block (CHB) or third-degree atrioventricular block - Postnatally, a full 12-lead electrocardiogram should be performed on infants born to mothers with SSA and SSB antibodies to evaluate for CHB. - While most infants with complete CHB in the setting of NSLE are asymptomatic, a subset may exhibit or develop signs or symptoms such as gallops, murmurs, variable first heart sound intensity, and congestive heart failure (CHF) due to cardiomyopathy. - Asymptomatic infants require close follow-up. - Those with signs and symptoms of cardiomyopathy and CHF (eg, fatigue, diaphoresis, rales on lung examination, peripheral edema, hepatomegaly, etc) may require pacemaker implantation.

The most common cardiac conduction abnormality associated with neonatal systemic lupus erythematosus presenting with bradycardia is

complete congenital heart block, or third-degree atrioventricular block - A full 12-lead electrocardiogram should be performed on neonates born to mothers with SSA and SSB antibodies to evaluate for congenital heart block

A 2-year-old boy's parents are concerned about significant speech delay. He exhibits unusual dysmorphology, including malar and zygomatic hypoplasia with downslanting palpebral fissures; prominent nose; significant micrognathia and retrognathia; small, posteriorly rotated, and malformed ears; and lower eyelid colobomas with absent eyelashes. His father has similar dysmorphology. Of the following, the MOST likely reason for this boy's speech delay is

conductive hearing loss Treacher Collins syndrome (TCS) - conductive hearing loss because of underlying hypoplasia of the middle ear cavities and malformation of the ossicles - inner ear anatomy is typically normal so sensorineural hearing loss is not usually seen - typically an autosomal dominant disorder caused by gene mutations in TCOF1 (78%-93%) and POLR1C or POLR1D (8%) - intelligence is usually normal - fertility is normal

A study is conducted in a tertiary care center to examine the relationship between morbidity in children with cystic fibrosis and annual household income. Results showed that children of families with incomes below the poverty line were 5 times as likely to have cystic fibrosis-related morbidities compared with children of families whose incomes were above the poverty line. An attempt was then made to increase access to health care for children living in households with incomes below the poverty line. A social worker was assigned to each family and provided gas vouchers and mileage and taxi reimbursement to the parents of patients whose incomes were below the poverty line. Of the following, the type of bias that MOST accurately describes what was corrected in the study is

confounding bias - when extraneous factors or variables are present in the study that can distort the association between exposure and outcome - can be addressed in a study design by using techniques such as restriction, matching, and randomization

After delivery, neonate had respiratory distress and abnormal lung examination and chest radiography findings. He was evaluated for cardiac and pulmonary parenchymal sources of his chest opacification and was found to have only an exudative effusion. His likely diagnosis is

congenital chylothorax - most common cause of neonatal pleural effusion - decreased pulmonary lymphatic drainage - results from interruption of pleural lymphatic drainage and is characterized by an exudative effusion with high levels of chylomicrons, triglycerides, and lymphocytes (>80% of the total white blood cell [WBC] count in the pleural fluid) - chylous effusions typically appear thin and milky, as opposed to empyemas, which appear cloud - triglycerides and chylomicrons in the fluid are the source of the characteristic milky appearance - chylous effusions may be caused by thoracic duct trauma (eg, during surgery), thoracic duct obstruction, or lymphangiectasia, or may be idiopathic. Most cases of congenital chylothorax are idiopathic.

Neonate has conjugated hyperbilirubinemia and microcephaly. The most likely cause of these findings is

congenital cytomegalovirus (CMV) infection - Conjugated hyperbilirubinemia is defined by a conjugated serum bilirubin greater than 20% of the total serum bilirubin level. - The differential diagnosis of conjugated hyperbilirubinemia is broad and, in addition to CMV infection, includes hypothyroidism, hepatitis, Escherichia coli infection, Alagille syndrome, biliary atresia, galactosemia, choledochal cyst, tyrosinemia, and α1-antitrypsin deficiency. - Because biliary atresia should be treated with the Kasai procedure within the first 8 weeks after birth, the initial evaluation of conjugated hyperbilirubinemia must involve abdominal ultrasonography to confirm the presence of bile ducts. - Symptomatic infection with CMV affects 0.4% of neonates in the United States annually. - Cytomegalovirus is the leading cause of sensorineural hearing loss (SNHL) among children in the United States. Of note, half of children with hearing loss related to congenital CMV infection pass their neonatal hearing screening and exhibit hearing loss in infancy or early childhood. - Children with symptomatic CMV infection should be treated with oral valganciclovir for a total of 6 months to decrease the risk of SNHL. Infants who are critically ill or do not tolerate the oral formulation may require intravenous ganciclovir. Because of the risk for neutropenia, absolute neutrophil counts should be monitored weekly for the first 6 weeks, at 8 weeks, and then monthly until the completion of treatment.

The neonate described in the vignette has clinical manifestations of microcephaly, cataracts, sensorineural hearing impairment, radiolucent osseous lesions, and possible cardiac disease, all of which are highly suggestive of

congenital rubella syndrome (CRS) - diagnosis confirmed by detection of rubella-specific serum IgM antibody (usually within the first 6 months after birth) or detection of virus in culture or polymerase chain reaction (ie, throat, nose, urine) - documentation of stable or rubella-specific rising IgG titers over the first 7 to 11 months after birth is also suggestive of congenital infection - ophthalmologic evaluation may detect cataracts or retinopathy, whereas computed tomographic scan of the brain may detect periventricular calcifications

Adolescent has aseptic meningitis and parotitis due to mumps. Mumps is an acute, communicable, vaccine-preventable infection in children caused by the mumps virus. Mumps is characterized by unilateral or bilateral parotid gland swelling; common complications of mumps include aseptic meningitis and orchitis. Mumps is spread via

contact with infected respiratory droplet secretions and saliva = droplet precautions - Individuals with mumps are contagious from 1 to 2 days before the onset of parotitis to several days after. Therefore, droplet precaution is the recommended choice for isolation for this adolescent.

During a health supervision visit, a 14-year-old adolescent boy with known primary hypertension is found to have a blood pressure of 146/90 mm Hg. The cuff size was confirmed as appropriate. His body mass index is at the 85th percentile. His history and physical examination findings are otherwise unremarkable, and previous evaluations for end-organ damage have been negative. Bilateral radial and femoral pulses are normal and without delay. The adolescent has been participating in high school football and weight training without complaint. Of the following, the BEST recommendation for this adolescent regarding football participation is to

continue aerobic and low-intensity conditioning only - He merits restriction from competitive sports activities but he should continue aerobic and low-intensity conditioning only. - Although participation in exercise is generally associated with a decrease in blood pressure over time, the acute blood pressure response during exercise varies depending on the type of activity - The American Heart Association, American College of Cardiology, and the AAP's Council on Sports Medicine and Fitness more specifically recommend that athletes with stage 2 hypertension should be restricted from high static activity until blood pressure is under control. Although football is considered a "moderate static" activity, weight training is considered "high static" and this boy should not participate until his blood pressure is below his stage 2 threshold (ie, <140/90 mm Hg).

The mother of a 4-month-old boy was recently diagnosed with shingles on her lower abdomen. The boy was born at term and has been growing and developing normally with no intercurrent illnesses. His mother is wondering if she can continue to breastfeed. Of the following, the MOST accurate response is that she should

continue breastfeeding without interruption - Because the mother can and should cover the lesions to prevent her breastfeeding infant from coming into contact with them, she may continue to breastfeed without interruption. - At 4 months of age, this infant is too young to receive the varicella vaccine. Active shingles is not an indication for a mother to stop breastfeeding or pump and discard her milk. - It is important to note that maternal infection with hepatitis B, hepatitis C, or cytomegalovirus are not contraindications to breastfeeding. - Mothers with herpes simplex virus infection may continue to breastfeed unless the lesions are on the nipple or breast; in this situation, a mother should be advised to cover the lesions and feed temporarily only from the unaffected breast.

A 35-week-gestation neonate is being evaluated in the nursery. She was born via cesarean delivery because of severe maternal preeclampsia and breech presentation. Her mother has a history of gestational diabetes and a negative group B Streptococcus screening result. In the delivery room, the neonate required positive pressure ventilation for 30 seconds because of decreased respiratory effort. She voided in the delivery room. In the nursery, her vitals signs include a temperature of 36.5°C, a heart rate of 156 beats/min, a respiratory rate of 45 breaths/min, a blood pressure of 52/22 mm Hg, with a mean arterial blood pressure of 32 mm Hg, and oxygen saturation on pulse oximetry of 95% in room air. Physical examination reveals a comfortable neonate with normal work of breathing and +2 peripheral pulses. Of the following, the BEST next management step for this neonate is to

continue routine care - The optimal blood pressure for a neonate is difficult to define. - Providers must consider clinical factors such as perfusion, end-organ perfusion, and clinical stability before deciding whether to treat a low blood pressure. - A neonate with low blood pressure and evidence of end-organ hypoperfusion should receive volume resuscitation. If hypotension persists, vasopressor or corticosteroid therapy may be required. - The neonate in the vignette does not have evidence of hypotension with end-organ hypoperfusion so a normal saline bolus is not indicated.

A 2-month-old boy is brought in for immunizations and a well-baby check. He is the product of a term delivery to a 26-year-old G1P1 mother who had good prenatal care; Apgars were 7 and 9. He was a little slow to feed at first but seemed to improve with advice from lactation care specialists before discharge. His mother is now concerned about his seeming lack of weight gain. She says he sleeps too much and is hard to arouse, and when he does nurse, it is only for a few minutes before he falls asleep again. She has tried formula, which he mostly spits out, and his bowel movements are dark and runny. She has also noted that sometimes his lips look a little dark. Otherwise, he has not been sick. On exam, he is alert but quiet and thin-looking; heart rate is 160 beats/min, and there is a grade 2/6 holosystolic murmur at the lower left sternal border. His weight is up 10 g from birth weight, and height and head circumference are following the 10th percentile. What is the most appropriate feeding management for this patient?

continuous NG feedings - This baby is showing signs of congenital heart disease (CHD). - Both cyanotic and acyanotic congenital heart disease patients have increased energy requirements to keep up with elevated total energy expenditure from increased cardiac work, increased work of breathing, and increased autonomic sympathetic stimulation. Generally, 110-120 kcal/kg per day is needed in these patients. - Despite normal birth weights, most infants with CHD tend to become malnourished due to the increased total energy expenditure and reduced oral intake from anorexia, early satiety, and frequent complicating illnesses. - Malnourished infants with CHD experience better growth with continuous nasogastric drip feedings that can be accomplished at nighttime, so as to not impede on the mobility of infants and their families. - The nutritional status is important, as it can influence the timing, type, and recovery from cardiac surgery.

First step in eval of suspected neonatal intestinal obstruction ...

conventional abdominal radiograph

Mucopolysaccharidoses are lysosomal storage disorders characterized by accumulation of glycosaminoglycans in various tissues and organs. Mucopolysaccharidosis type I (Hurler syndrome) is inherited in an autosomal recessive manner; mucopolysaccharidosis type II (Hunter syndrome) is inherited in an X-linked recessive manner. Hurler and Hunter syndromes have similar clinical features. Children with Hunter syndrome do not have

corneal clouding - and have a better cognitive outcome

Unlike adults, children with sinusitis usually present with ...

cough, nasal discharge, and halitosis

6 yo (at 50th %ile for height) suddenly stops growing and by 10 yo is off thew growth curve (<5th %ile). He essentially has not grown since 7 yo. Most likely etiology?

craniopharyngioma - often associated with hypothalamic and pituitary destruction - may present as growth failure in children or adolescence - sudden arrest of growth should raise suspicion for this tumor

A 12-year-old male is being seen for a sports physical. He has been the star of his middle school wrestling team for the last year. Though doing relatively well and offering no complaints, on your review of systems, he does admit to fairly regular stomach aches, occasional diarrhea, and mild nausea. He denies any drug or substance abuse, though he does take some over-the-counter vitamins and nutritional supplements that were recommended by his coach and teammates. His vital signs and physical exam are normal, and he has gained 5 lb since his last check up 2 years ago. You're concerned about possible performance-enhancing drug use. Based on these symptoms, which ergogenic drug is most likely being consumed?

creatinine - formed from glycine, arginine, and methionine by the liver, kidneys, and pancreas then transported to muscle, heart, and brain - also naturally present in dietary meat and fish - 95% remains in muscle where it exists in balanced equilibrium with phosphocreatine that provides energy to muscle by its dephosphorylation, donating a phosphate to adenosine diphosphate (ADP) and producing adenosine triphosphate (ATP) - oral supplemental creatine can cause a 20% increase in muscle phosphorylation stores, quicken phosphocreatine replenishment, and buffer lactic acid, potentially delaying fatigue onset - athletes taking creatine commonly report early weight gain, minor gastrointestinal discomfort, and muscle cramps - remains a legal nutritional supplement, though the American College of Sports Medicine has recommended that is not to be used by anyone younger than 18 years.

Pt has a supracondylar fracture. Most common long-term complication?

cubitus varus - elbow deformity with a decreased carrying angle - deviation of the forearm toward the midline of the body - most common complication of a supracondylar fracture of the elbow, usually caused by poor reduction of the fracture with medial displacement, internal rotation, and extension of the distal fragment - produces a cosmetic deformity, but it is usually of little functional significance

6 yo boy with hx of acute onset of left arm pain after falling from playground monkey bars. On PE, he refuses to move his left arm at elbow, which is swollen and tender. Most likely fracture in this scenario, if substantially displaced, is well recognized to cause what complication?

cubitus varus - supracondylar fracture of distal humerus - ages 5-10 usually after falling on outstretched hand with elbow fully extended potential complications - radial or median nerve injury - brachial artery injury - comp't syndrome - volkmann contracture = claw like deformity of wrist and fingers - improper reduction --> cubitus varus / gunstock deformity of elbow

Shwachman-Diamond syndrome is an AR syndrome of ...

exocrine pancreatic insufficiency + bone marrow dysfuncion

Chronic cough in children ≤ 14 years usually defined as

daily cough lasting four or more weeks

First-line therapy for functional constipation is

daily polyethylene glycol - should continue for at least 2 months and should not stop unless symptoms have resolved for at least 1 month

In tet of fallot, tx must increase blood flow towards lungs and decrease blood flow to body to raise oxygen back to baseline. Movement of pt's legs to chest, and admin of O2, sucrose, morphine, fluid bolus, phenylephrine are intended to ...

decrease pulmonary vascular resistance increase systemic vascular resistance

Brains of individuals with autism have ..

decreased formation of Purkinje cells in cerebellum

langerhans cell histiocytosis has what urine findings ...

decreased urine osmolarity - via pituitary dysfunction associated with DI

Changes in neurobiological factors in the brain contribute to aggressive behaviors. These changes may include

decreased: serotonin, oxytocin, and γ-aminobutyric acid (GABA) levels increased: vasopressin and dopamine levels

simple distal hypospadias with otherwise normal exam. management...

defer circumcision if everything else is ok (like penile length)

results from abnormal mechanical or structural external forces, rather than an intrinsic abnormality of the fetus

deformation sequence

Severity of intellectual disability is based on ...

degree of adaptive impairment

Side effects of creatinine used in athletes ...

dehydration, muscle cramps, GI distress

A 3-year-old boy is brought in by his parents for an evaluation of his stuttering. They state he has word and phrase repetitions, with only occasional sound repetitions. He does not appear to be aware of his problem, and he shows no adverse behavioral reactions when his parents attempt to help him finish his sentences. He has demonstrated normal development prior to this problem. He is on no medications. No family history of stuttering is elicited. Physical exam is entirely within normal limits. What is the most likely cause of the patient's condition?

dev'tal dysfluency - characterized by a lack of awareness of the speech pattern, onset at age 2-3, spontaneous resolution, and word and phrase repetitions much more common than sound and syllable repetitions

A 10-year-old boy presents for an evaluation due to his inability to fall asleep before 1 AM despite his parents trying different methods to make him fall asleep earlier. Waking him up in time for the school bus has become an "everyday battle." He is an active 10-year-old who does not drink too many caffeinated drinks, but his school performance has recently fallen below average. On weekends, if allowed to sleep, he will typically sleep until 2 PM. More recently, there have been reports from his teachers of his dozing off in class and not paying attention. Analysis of his sleep diary (which the parents were asked to maintain 2 weeks prior to this visit) reveals a consistent pattern throughout the weekdays as described above. Based on this information, you suspect a circadian rhythm sleep disorder (CRSD). What subtype of CRSD does this patient most likely have?

delayed sleep phase cycle - most common among the CRSDs The diagnostic criteria for DSPT set forth by the ICSD-3 are: - A delay in the phase of the major sleep period relative to the desired sleep time and wake-up time is present. - When allowed to set their schedule, patients will exhibit normal sleep quality and duration for age and maintain a delayed but stable phase of entrainment to the 24-hour sleep-wake pattern. - Sleep log or actigraphy monitoring for at least 7 days demonstrates a stable delay in the timing of the habitual sleep period.

Williams syndrome is caused by what genetic defect?

deletion on chromosome 7

12 yo M + pallor and dark urine + splenomegaly. Labs = total bili 4.8 and increased alk phos. CBC with hgb 10, hct 30%, MCV 85, mean cell hemoglobin concentration 38%, retic count 4%. Blood sample tested for hemolysis in buffered salt solutions at varying concentrations. As salt solution increases, hemolysis decreases more in pt's sample than in the control. What is expected on the pt's smear?

dense red blood cells with absent central pallor - hereditary spherocytosis - most common form of hemolytic anemia caused by defects in cell membrane - nml MCV - increased MCHC

Researcher is conducting study in which renal tubular fluid is sampled at different sites along nephron in high and low ADH states. At what sites would tubular fluid have highest osmolarity in low ADH state?

descending loop of henle - tubular fluid becomes maximally concentrated as it moves down the descending loop of Henle

A bleeding workup is warranted when a child has either repeated and/ or unexplained bleeding. Screening for a bleeding disorder warrants

detailed history and physical examination --> will help guide the initial laboratory evaluation = complete blood count with a peripheral blood smear review, prothrombin time (PT) with mixing studies, activated partial thromboplastin time (aPTT) with mixing studies, thrombin time, fibrinogen level, and von Willebrand antigen and activity levels - factor levels may be obtained after determining which is prolonged, the PT or aPTT (PT and aPTT may be normal in factor XIII deficiency, though the child can experience severe bleeding)

Clinical presentation and cerebrospinal fluid (CSF) findings of adolescent are highly suggestive of aseptic meningitis or meningoencephalitis. Given his recent mosquito bites while hiking in New York, the most likely etiology of his illness is West Nile virus (WNV). The diagnosis is generally made by

detection of WNV IgM antibody in the CSF or blood - Following initial detection in New York City in 1999, cases of West Nile neuroinvasive disease (WNND) have been reported in most US states, with the highest numbers seen in California, North Dakota, South Dakota, Oklahoma, Colorado, and Nebraska. - The incubation period for WNV illness is 2 to 6 days. Most individuals with WNV infection are asymptomatic. Symptomatic illness is characterized by fever, headache, eye pain, gastrointestinal symptoms, and fatigue. The duration of acute illness is 3 to 5 days. During convalescence, individuals may develop a rash, which starts on the trunk and may extend to the extremities and face. - Symptoms and signs may include abrupt onset of fever, headache, photophobia, altered mental status, and seizures; physical examination findings may include neck stiffness, cranial nerve palsies, and focal neurologic deficits. - Cerebrospinal fluid analysis shows pleocytosis with a predominance of lymphocytes; a neutrophilic pleocytosis may be present early in the illness. - Magnetic resonance imaging of the brain is often normal but may reveal signal abnormalities in the brainstem, basal ganglia, thalamus, or spinal cord.

A 26-month-old boy is being evaluated for concerns about speech and language. His mother says that he speaks using single words and has a total vocabulary of about 20 words. She is also concerned about his behaviors. She states that he does not look at her or looks away, even when she holds his face in her hands. He has prolonged tantrums, particularly when he does not get what he wants. The boy is particular about what he eats and gets upset with loud and sudden sounds. His favorite activity is to line up his blocks and cars. The boy's father is not as concerned about the boy's language development as he has a personal history of "not talking until preschool." The father is concerned that the boy tantrums when told "no" and that this has made it difficult when they are out in public. Of the following, the BEST next management step is referral to a(n)

dev'tal psychologist - Any child who is seen with significant language delay should receive an audiological evaluation. However, given the accompanying behaviors, a developmental evaluation is more likely to identify ASD as the source of his language delay and behaviors. - A behavior therapist, particularly one trained in applied behavioral analysis therapy, would be an appropriate professional to assist this child and family once the child's diagnosis and developmental status have been established.

An 8-year-old boy is seen for concerns about learning and development. He was recently diagnosed via psychoeducational evaluation as having moderate intellectual disability. A chromosomal microarray did not identify any genetic abnormalities, and the fragile X test result was negative. His parents are concerned about the possibility of a condition that was present at birth and ask if there are any other laboratory tests that could be conducted. Testing for an inborn error of metabolism is considered. Of the following, the sign or symptom MOST consistent with this type of condition is

dev'tal regression - The more severe the intellectual disability, the higher is the likelihood of identifying a cause. However, only 0% to 5% of children with intellectual disabilities are found to have an inborn error of metabolism. Results are more likely to be positive in patients with developmental regression; atypical odor; failure to thrive; or neurologic signs or symptoms such as abnormal tone, ataxia, or seizures. - Metabolic testing can include plasma amino acids, acylcarnitine profile, and total homocysteine; and urine organic acids, oligosaccharides, and glycosaminoglycans.

Reverse differential cyanosis, in which the postductal oxygen saturation level is higher than the pre-ductal level in neonates, is seen in

dextro-transposition of the great arteries - with either pulmonary hypertension or coarctation of the aorta - Dextro-transposition of the great arteries refers to the aorta arising anteriorly from the right ventricle and the pulmonary artery arising posteriorly from the left ventricle. Surgical correction via an arterial switch procedure is warranted.

Parents of 10 month old concerns that their son has "yellow jaundice." Report that he is otherwise well with the exception that his stools are a little more frequent. On PE, his skin has a yellowish hue, most prominent on the palms, soles, cheeks, and tip of his nose. Conjunctivae are clear. What is most likely to provide explanation for these findings?

dietary history - likely yellow-orange vegetables: carrots, squash, sweet potatoes = high levels of carotenoids = yellowing of skin except for sclera

15 yo girl presents for evaluation of amenorrhea. She also has hx of bicuspid aortic valve, horseshoe kidney, and sensorineural hearing loss. She takes not daily meds and has no known allergies. Height at 5th %ile. Wt at 35%ile. Breast dev't and pubic hair distribution are both at sexual maturity rating (Tanner) stage 2. Most likely to be identified during initial evaluation?

diffusely enlarged nodular thyroid - Turner syndrome --> associated with chronic autoimmune thyroiditis

4 yo boy + recurrent hx of pna and skin infections. Infected axillary lymph node, which needed to be drained. Culture was positive for aspergillus. What lab tests would most likely lead you to correct diagnosis of underlying disease?

dihydrorhodamine test - CGD - recurrent and often severe bacterial and fungal infections + granuloma formation - aspergillus - defects in NADPH - NBT = simple screening test

The snowman sign, a rounded, figure of eight, like cardiac contour with supracardiac shadow in superior mediastinum, is produced by...

dilated vertical left SVC, the innominate vein, and the right SVC = supracardiac anomalous pulmonary vein return - all or some of the pulmonary veins fail to drain into left atrium

The mother of a 13-year-old adolescent boy is concerned about a rumor that some students at his middle school have been using alcohol. She has discussed the rumor with her son, and he assured her that he has never used alcohol, tobacco, marijuana, or any other drug, and he does not have friends who use drugs. The mother has discussed with her son the use of drugs in their neighborhood and used the rumor as an opportunity to re-emphasize her anti-substance abuse stance. She is particularly worried because the boy's father has a history of problems associated with alcohol use. The boy's parents divorced when he was 18 months old and the father has subsequently not been involved in his son's life. The boy has no siblings. The mother is employed as a secretary for a construction firm and has no history of substance use. The boy has had no behavioral problems, gets good grades, and attends an after-school program associated with his school. He enjoys playing on a community soccer team, and his mother enjoys accompanying him to weekly practices and games. The importance of the mother's consistent and clear anti-substance use message is affirmed, and continued involvement in her son's extracurricular activities is encouraged. Of the following, the BEST additional advice for this mother is

discuss rules in the context of safety and responsibility - Authoritative (balanced) parenting style is associated with better behavioral outcomes including lower risk of adolescent substance use/abuse. - Rules for adolescents should be established in the context of safety, responsibility, and peer pressure. - Key protective factors against substance abuse include authoritative parenting style, individual attributes (eg, high self-esteem, good social/problem-solving skills, spirituality), school connectedness, communities with low tolerance for and limited access to drugs, and effective prevention programs.

A 12-year-old boy is seen in the office for bilateral knee pain. He reports that the pain began several weeks after starting basketball practice, and he has had no specific injuries. The pain is located just below his patella. It initially occurred primarily after activity, but now occurs throughout the day. Ibuprofen provides temporary relief. He has not experienced any swelling or redness. The boy is otherwise healthy with normal growth and development. On physical examination, he has focal tenderness over the inferior pole of both patellae and patellar tendons, but none over the tibial tuberosities. Mild pain is elicited with lateral patellar movement, without guarding or apprehension. He has pain with resisted knee extension. He has full lower extremity strength and range of motion. No knee ligamentous laxity is noted. His gait is unremarkable. The remainder of the boy's physical examination findings are normal. Of the following, the MOST likely diagnosis for this boy is

distal patellar apophysitis - also known as Sinding-Larsen-Johansson syndrome (SLJS) - a traction apophysitis of the patellar tendon on the growth plate at the inferior pole of the patella - atraumatic pain over the inferior pole of the patella in a skeletally immature child or adolescent is consistent with SLJS - Sinding-Larsen-Johansson syndrome is an overuse injury caused by repeated high-intensity activation of the extensor mechanism of the knee, such as running or jumping - pain associated with SLJS is typically gradual in onset, located over the inferior aspect of the patella, and often occurs after an increase in running and/or jumping activity - physical examination findings may include pain, tenderness, and focal swelling over the inferior patellar apophysis - a true knee effusion indicates intra-articular pathology and warrants additional evaluation -= the pain may be reproduced with active or resisted knee extension - gait should be evaluated for a limp or malalignment that may predispose a child to SLJS Treatment for anterior knee pain such as that caused by SLJS and Osgood-Schlatter disease includes: - Minimizing discomfort with local application of ice for 10 to 15 minutes per session 3 to 4 times per day, especially after activity, and over-the-counter analgesics. - Relative rest and avoidance of pain-generating activities (eg, running, jumping, hiking/prolonged walking, especially on hills). Activities that do not create pain during or after participation should be encouraged or continued. Although bicycling is generally well-tolerated, cycling up hills or against heavy resistance can provoke symptoms. - Use of a counterforce brace in those with SLJS, Osgood-Schlatter, or patellar tendinitis, if there is symptom relief. - Use of crutches and/or a knee immobilizer for several days for those with a significant limp or pain with daily activity. Children requiring these devices should be monitored closely and undergo imaging studies if they do not show rapid improvement. - Physical therapy or a therapeutic exercise regimen. Compliance with a good home exercise program is important for rapid recovery and to minimize recurrence risk

A 9-month-old boy is seen for a routine health supervision visit. His parents report no concerns. He is eating well, has appropriate weight gain, and is meeting appropriate developmental milestones. His past medical history is significant for premature birth at 34 weeks' gestation and a ventricular septal defect, status post repair. He has no residual cardiopulmonary disease. He required only short-term nutritional support and did not require vascular access in the neonatal intensive care unit. His family history is negative for early myocardial infarction, hypertension, or renovascular disease. His physical examination findings are normal. The office does not routinely measure blood pressure in very young children. The physician reviews the discharge summary for recommendations regarding blood pressure monitoring for this child.' Of the following, the BEST recommendation for this child is that he

does require blood pressure measurement because of his congenital heart disease The American Academy of Pediatrics Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents was last updated in 2017 The guideline recommends that children should have a blood pressure measurement at each annual health supervision visit beginning at 3 years of age. Routine blood pressure measurement should begin at an earlier age in patient populations with specific conditions or circumstances including: - Obesity - Renal disease - Diabetes - Aortic arch obstruction - Those taking medications known to cause hypertension - History of prematurity <32 weeks, small for gestational age, very low birth weight, neonatal complication requiring intensive care or umbilical artery line - Congenital heart disease (repaired or unrepaired) - Recurrent urinary tract infections, hematuria, or proteinuria - Renal disease or urologic malformations - Family history of congenital renal disease - Solid-organ transplant - Malignancy or bone marrow transplant - Treatment with drugs known to raise blood pressure - Other systemic illnesses associated with hypertension (eg, neurofibromatosis, tuberous sclerosis, sickle cell disease) - Evidence of increased intracranial pressure

A 13-year-old adolescent boy is brought to the emergency department 20 minutes after he fell off his skateboard, knocking out his front tooth. He has no other injuries. His friend picked up the tooth and immediately placed it in a container of milk. In the emergency department, the tooth is cleaned and returned to place. Upon discharge to home he is prescribed an antibiotic after it is determined that he has no medication allergies. Of the following, the MOST appropriate antibiotic choice for this patient is

doxycycline - Immediately after the injury, if the tooth can be found, it should be cleaned briefly (less than 10 seconds) with clean water. - Ideally, prior to transporting the child for emergency care, the tooth should be replaced into the socket or held in the mouth between the molars and the cheek (if the child is old enough that there is no risk of choking). If this measure is not possible or safe, the tooth can be transported in an alkaline liquid such as milk. - Transporting a tooth dry or in water should be avoided; a tooth exposed to air for 60 minutes or transported in a non-alkaline medium is not viable. - Upon arrival to the emergency department, if the tooth was already replaced into the socket it should be left in place. The area can be flushed with saline and lacerations repaired. If the tooth has not been replaced, the tooth and socket should be gently cleaned with a saline wash via syringe. Then, with gentle pressure, the tooth can be replaced into the socket. - After the tooth is replaced, the child or parent should perform twice daily chlorhexidine (0.1%) solution rinses for 1 week. Prophylactic antibiotics are recommended. Doxycycline twice daily for 7 days is the recommended antibiotic for children. Amoxicillin or penicillin are alternative antibiotics that may be used.

A 17-year-old male college student is evaluated in the emergency department for a 1-day history of fever, headache, and vomiting. He was well until 3 days ago when he developed malaise, loss of appetite, and painful swelling of his left jaw and cheek area. His immunizations are up to date. Two of his college roommates recently developed a similar illness, one of whom also developed orchitis. On physical examination, he is febrile (38°C); his other vital signs are normal. He is alert and interactive, but has neck stiffness and pain with neck flexion. He has left parotid swelling that is mildly tender to palpation. The overlying skin is not warm or red. The remainder of his physical examination findings are unremarkable. Cerebrospinal fluid analysis shows 460 white blood cells (35% neutrophils, 60% lymphocytes, and 5% monocytes), a glucose level of 48 mg/dL (2.7 mmol/L), and a protein level of 65 mg/dL (0.65 g/L). Gram stain of the cerebrospinal fluid shows no organisms. He is admitted to the hospital. Of the following, the MOST appropriate isolation precaution for this adolescent is

droplet - aseptic meningitis and parotitis due to mumps - acute, communicable, vaccine-preventable infection in children caused by the mumps virus - unilateral or bilateral parotid gland swelling; common complications of mumps include aseptic meningitis and orchitis - spread via contact with infected respiratory droplet secretions and saliva - individuals with mumps are contagious from 1 to 2 days before the onset of parotitis to several days after - droplet precaution is the recommended choice for isolation for this adolescent

A 16-year-old boy presents after being involved in a motor vehicle accident as an unbelted front seat passenger; the car was was not equipped with airbags. The patient was unconscious for approximately 1 minute at the scene; blood was noted on the passenger-side dashboard. He states he has a headache, pain in the right forehead, and a strange, salty taste at the back of his throat. Past medical history and family history are unremarkable. Growth and development have been normal and immunizations are current. Vital signs are normal; Glasgow Coma Scale rating is 15. There is swelling and tenderness of the right forehead, with a step-off palpable 1 cm superior to the right brow (underlying a laceration). The nose is deviated to the left, with clear rhinorrhea present on the right. The remainder of the examination is normal, without evidence of extracranial trauma. A right frontal sinus fracture is suspected. What complication is most likely present in this patient?

dural tear - salty taste and clear rhinorrhea indicate the presence of a cerebrospinal fluid (CSF) leak likely due to a dural tear - frontal sinus fractures are high impact injuries, most commonly due to motor vehicle accidents and less commonly due to assault - CSF leaks occur in approximately 1/3 of cases of frontal sinus fractures. A CSF leak is indicative of a dural tear or an accompanying nasoorbitoethmoid fracture. - Some CSF leaks resolve spontaneously, but this is more likely with a nondisplaced fracture; the step-off palpated in this case makes a displaced frontal sinus fracture more likely. - Surgical exploration will likely be required. Untreated CSF leaks can lead to meningitis.

Boys with duchenne muscular dystrophy are at increased risk of developing specific learning disabilities:

dyslexia dysgraphia dyscalculia

Isotreinoin use associated with ...

ear abnormalities / microtia eye abnormalities (microphthalmia) cleft palate cerebellar malformations hydrocephalus microcephaly prematurity

Central precocious puberty caused by ...

early activation of pulsatile secretion of GnRH

Hydrocephalic 10 week old infant with large, segmental, unilateral facial hemangioma found on axial CT scan of head to have large posterior fossa cyst and dilated ventricles. In light of these findings, what additional studies do you perform in this patient?

echo - dandy-walker cyst in posterior fossa on CT + facial hematoma = most likely PHACES syndrome = more common in females - PHACES = posterior fossa malformations, hemangiomas, arterial anomalies, cardiac anomalies (coarct, PDA, ASD, VSD), eye abnormalities , sternal clefting/supraumbilical raphe Screening - large facial hemangiomas --> MR of brain, echo, neuro eval, complete ophtho eval - other evals for PHACES = thorough physical exam, MRI/MRA of brain and neck, ophtho eval

A mother brings her 6-year-old daughter to you for both nighttime bedwetting and daytime incontinence. The mother has noticed that the child's underwear remains wet all the time, but she voids normally. The child has not been dry since infancy. She has achieved her other developmental milestones normally. There is no history of fecal soiling of her underwear. She is intelligent and is doing well in school. Cardiac, pulmonary, abdominal, and neurological examinations are normal. What is the most likely clinical diagnosis?

ectopic ureter - when the ureter drains outside the bladder - drains the upper pole of the duplex kidney, and the normal ureter drains the lower part of the collecting system - the ectopic ureter may enter the urethra at the bladder neck; it may also enter the urethrovaginal septum

For patients with acute cluster headache, we recommend initial treatment with

either oxygen or triptans - Oxygen should be tried first if available since it is without side effects - Otherwise, subcutaneous sumatriptan 6 mg can be used as initial therapy for patients with no contraindications - For patients who have a suboptimal response to inhaled oxygen and are unable to tolerate subcutaneous sumatriptan, alternatives include intranasal sumatriptan or intranasal zolmitriptan

What type of toothbrushes have been shown to be more effective at reducing plaque and decreasing gingivitis in older teens and adults?

electric toothbrushes

For an infant with cyclical vomiting, because of the increased likelihood of inborn errors of metabolism and adrenal insufficiency, laboratory and imaging evaluation should be performed when symptomatic (and before intravenous fluids) and should include:

electrolytes glucose urine ketones lactate ammonia serum amino acids urine organic acids upper gastrointestinal series abdominal ultrasonography

The diagnosis of dermatomyositis is made clinically based on examination findings of proximal muscle weakness and dermatologic findings. Laboratory evaluation includes

elevated levels of creatinine kinase, lactate dehydrogenase, and aldolase - Results of a complete blood cell count and basic metabolic panel are typically within normal limits - A lack of myositis-specific antibodies does not exclude the diagnosis, especially in children - Magnetic resonance imaging of the proximal muscles can be useful for diagnosing and monitoring the illness - Proximal muscle weakness associated with a rash is consistent with dermatomyositis

Recognize pityriasis alba, in which the management is

emollient pityriasis alba - hypopigmented scaling macules on his face - borders of the lesions are not sharply demarcated; rather, there is a gradual transition from normal to abnormal pigmentation - some clinicians treat with a low-potency topical corticosteroid (eg, hydrocortisone 1% or 2.5%) for 7 days, but using a mid-potency preparation like triamcinolone is not advisable because of the potential for skin atrophy - form of postinflammatory hypopigmentation that often occurs in children who have a history of atopic dermatitis - lesions commonly involve the face but may occur on the trunk and extremities - often becomes apparent after sun exposure because normal skin tans but affected areas do not - patient and family should be counseled that the return of normal pigmentation takes months - in addition, they should be instructed to treat new lesions (ie, pink, scaling macules) with a low-potency topical corticosteroid to prevent new areas of hypopigmentation

Physical examination findings are notable for prominent scales on the extensor surfaces of the legs and hyperlinear palms. These findings suggest a diagnosis of ichthyosis vulgaris (IV), the most common form of the disease. Accordingly, treatment via what?

emollient containing an α-hydroxy acid (eg, ammonium lactate) - Ichthyosis vulgaris usually improves with age, during warm-weather months, and in warm, humid environments. - A clue to the diagnosis of IV is thickening of the skin on the palms and the presence of prominent creases (ie, hyperlinear palms).

A 5-year-old boy is seen in the office for a health supervision visit. His past medical history is significant for an atrioventricular septal defect that was repaired at 6 months of age; there is residual left atrioventricular valve regurgitation that is being monitored by his pediatric cardiologist. His activity and diet have been normal. His mother has no concerns. The boy's physical examination findings are normal aside from his murmur, which is unchanged from prior visits. At the most recent cardiologist visit, his heart function was depressed and the boy was started on a new medication; the mother cannot remember its name. Of the following, the MOST likely medication prescribed for this child is

enalapril - Boy has evidence of heart failure. - The boy has repaired congenital heart disease and has developed ventricular systolic dysfunction. - Children with heart failure may have nausea, vomiting, abdominal pain, decreased oral intake, fatigue, irritability, and increased work of breathing. - Physical examination findings may include tachycardia, tachypnea, diaphoresis, increased work of breathing, and hepatomegaly. - Testing that may help guide the diagnosis and management of heart failure include electrocardiography, echocardiography, chest radiography, and a brain natriuretic peptide level. - The boy in the vignette, who is asymptomatic with decreased systolic function, would be classified as being in stage B. - Clinical guidelines recommend starting an angiotensin-converting enzyme (ACE) inhibitor, such as enalapril, for patients in stage B.

A 5-year-old boy is seen in the office for a health supervision visit. His past medical history is significant for an atrioventricular septal defect that was repaired at 6 months of age; there is residual left atrioventricular valve regurgitation that is being monitored by his pediatric cardiologist. His activity and diet have been normal. His mother has no concerns. The boy's physical examination findings are normal aside from his murmur, which is unchanged from prior visits. At the most recent cardiologist visit, his heart function was depressed and the boy was started on a new medication; the mother cannot remember its name. Of the following, the MOST likely medication prescribed for this child is

enalapril - heart failure - repaired congenital heart disease and has developed ventricular systolic dysfunction. - children with heart failure may have nausea, vomiting, abdominal pain, decreased oral intake, fatigue, irritability, and increased work of breathing - physical examination findings may include tachycardia, tachypnea, diaphoresis, increased work of breathing, and hepatomegaly

A 17-year-old patient is seen for a health supervision visit. The patient seems withdrawn during the visit and does not contribute much to the conversation. During the confidential psychosocial interview, the patient discloses that 6 weeks ago she went to a party during a freshman orientation program and was given some punch that she thinks was altered. She knows that she was sexually assaulted but cannot recall what happened or who harmed her. She has not disclosed this information to anyone and fears having to go back to campus. She declines to contact the school and let them know what has occurred. She reports no abdominal pain, vaginal discharge, or dysuria. Her last menstrual period was 1 week ago. The pediatrician explains that the patient will be screened for sexually transmitted infections and that as a mandated reporter, the physician is required to file a report. The pediatrician also shares concerns about the patient's mental health after this traumatic event. Of the following, the BEST next step in management is to

encourage the patient to discuss the incident with her mother and recommend therapy to address the traumatic event - was sexually assaulted 6 weeks before disclosing the event to her pediatrician; she is showing signs of being withdrawn and now fears going to college --> encouraging her to disclose the event to her mother is the first step in ensuring she has a support system and an advocate to assist with scheduling her for therapy, as well as to make sure she is compliant with both medical and mental health follow up - too much time has passed to perform a sexual forensic examination. - Medical providers must be aware of reporting laws for sexual assault for their state(s) of practice, and the adolescent should be informed if a report will be filed. - The pediatrician should obtain a detailed history and, if the event occurred less than 72 hours before the time of disclosure, should encourage the adolescent to consent to undergo a medical and sexual forensic examination. - A forensic examination is usually performed in an emergency department or by a sexual assault team to try to collect DNA samples from the perpetrator. If the adolescent declines a forensic examination, a medical examination would still be warranted and should include a pregnancy test in women, screening for sexually transmitted infections, drug screening, and evaluation for injuries. - Female patients should be given emergency contraception within 120 hours of an assault and human immunodeficiency virus prophylaxis within 72 hours of an assault. The Centers for Disease Control and Prevention also recommends empirical treatment for Chlamydia infection, gonorrhea, and trichomoniasis. - If the assault is reported more than 72 hours but less than 7 days after the event, collection of samples is dependent on protocols followed by local authorities. - It is difficult to collect evidence a week after the event.

Syncope is common during adolescence. The vast majority of these are vasovagal events that do not warrant intervention beyond reassurance of the patient and caregivers. The timing of collapse is a crucial distinction. Collapse after high levels of exertion, particularly at the finish line of running events, most commonly occurs because of blood pooling in the legs. This can be prevented by

encouraging athletes to perform an active cool-down --> treatment involves placing athletes supine, with their legs elevated above the heart - Episodes of syncope that occur during physical exertion need a full evaluation to rule out cardiac pathology, because vagal tone is reduced during exercise

16 mo old girl swallowed lye. Evidence of upper airway and oropharynx being affected. Next step?

endotracheal intubation - airway swelling can happen rapidly

Over 90% of cases of childhood obesity are d/t ...

energy imbalance

A 6-month-old boy is seen in the emergency department with fever of up to 38.8°C, rhinorrhea, cough, tachypnea, and wheezing. He was born at term after an uncomplicated pregnancy and has been exclusively breastfed. He started daycare 2 weeks ago when his mother returned to work as a school teacher. The mother reports the onset of symptoms 3 days ago, with worsening of cough and breathing difficulty today. He is still able to nurse, but not as vigorously as she expects. He continues to have the expected number of wet diapers. On physical examination, he is alert but clings to his mother. He appears adequately hydrated, with tears in his eyes, capillary refill is 2 to 3 seconds, and skin turgor is normal. His heart rate is 130 beats/min; respiratory rate is 40 breaths/min; room air oxygen saturation is 94% on pulse oximetry. He has mild intercostal retractions but no suprasternal retraction or nasal flaring. There is diffuse expiratory wheezing and mildly prolonged expiration. Of the following, the MOST appropriate management approach for this child is

ensuring adequate hydration, counseling family on hand hygiene, providing follow-up instructions - acute bronchiolitis, characterized by fever and acute respiratory symptoms including cough and wheezing in a child younger than 2 years - this child, who was not premature, is more than 12 weeks old, has no underlying heart or respiratory disease to put him at risk for poor outcome, represents the most common presentation of bronchiolitis, and is at low risk for complications - oxygen saturation greater than 90% when awake, respiratory rate less than 50 breaths/min, mild but not severe retractions, adequate hydration, and ability to feed by mouth all indicate that it is safe at this time to treat him as an outpatient if the family is reliable and close follow-up is possible to monitor respiratory distress and hydration status

A 3-year-old boy presents with "hallucinations". On exam, the child is found to be hypertensive and tachycardic. The mother reveals to you that the child has had severe nasal congestion for 1 month; symptoms include excessive sneezing, watery eyes, and an itchy nose. She admits to giving the child a medication that she received at an "alternative therapy" clinic. What is most likely to produce the signs and symptoms found in this child?

ephedra - This patient is showing signs of ephedra toxicity. - Ephedra, also known as Ma Huang, has the active ingredient ephedrine. It is used for nasal congestion, asthma, appetite suppression, and as a "natural high". - The side effects include hypertension, tachycardia, anxiety, palpitations, restlessness, headache, insomnia, and dizziness. - There are reports of teenagers who have died after taking ephedra. The general consensus is that ephedra is not safe for use by children.

You are evaluating a 16-year-old boy at a health maintenance visit. He had been doing well academically and socially in his junior year in high school until approximately 4 months ago, when he secured a coveted spot on his school's long distance cycling team. Since that time, his academic performance has declined. Friends and family have noted that he has become irritable and easily agitated. He notes that he has had difficulty sleeping. There have been no recent changes in family status or social relationships. Past medical and family histories are unremarkable. Growth and development are normal and immunizations are current. Vital signs are normal except for a pulse of 120 at rest. Height and weight are at the 75th and 50th percentiles, respectively. He appears generally anxious and has a mild tremor of the hands at rest. Body habitus is unchanged from previous examinations. Upon further questioning, he admits to using a substance given to him by a teammate to reduce fatigue and improve his cycling times. What performance enhancing drug has this patient most likely been taking?

ephedrine - Stimulants, such as ephedrine and pseudoephedrine, are used as performance enhancing agents to reduce fatigue, increase endurance, and improve times. - There is no reliable evidence of their efficacy, however. - Adverse effects include agitation, anxiety, irritability, insomnia, tremor, and palpitations, with occasional reports of myocardial infarction, cerebrovascular accident, psychosis, and seizures. - Major athletic governing bodies, including the International Olympic Committee (IOC) and the National College Athletic Association (NCAA), have banned the use of such agents.

Appearance of rash of rheumatic fever ...

erythematous, serpiginous, macular lesions with pale centers on trunks and extremities = erythema marginatum

Part of your practice involves managing pediatric patients in a long term care facility for developmentally disabled children. One of the patients is a 7-year-old near drowning victim who was found at the bottom of a pool 2 years ago. She has been a resident at the facility for the past year when her family was no longer able to care for her at home. She suffered anoxic brain damage, is developmentally disabled, and has a tracheostomy and gastrostomy tube, though she has been able to take limited oral feedings. She was started on a proton pump inhibitor for reflux some time ago and was doing well until 1-2 weeks ago when she began having increasing difficulty swallowing with more back and neck posturing during feedings and increased irritability. Which diagnostic study would best assess the adequacy of dosage of medication for gastroesophageal reflux disease (GERD) in this child?

esophageal pH monitoring - Esophageal pH monitoring measures the frequency and duration of acid reflux episodes by transnasal placement of acid sensing microelectrodes. This is useful in determining the presence of a temporal association between acid reflux and frequently occurring symptoms. - It is also useful for assessing the adequacy of dosage of histamine-2 receptor antagonists or proton pump inhibitors in children whose GERD symptoms are not improving.

16-month-old girl is evaluated in the office for a 9-month history of worsening vomiting. She has nonbilious, nonbloody emesis that occurs after nearly every meal, although she tends to vomit less often when given liquids (she prefers whole milk or water) or dissolvable foods like butter crackers and puffs. Noodles, bites of meats, and fruits (like bananas and strawberries) almost always result in emesis. The girl was born at term. She was breastfed, with intermittent regurgitation noted prior to the introduction of solids. She has been growing and developing normally. Her physical examination findings are normal. For her age, her weight is at the 12th percentile, length is at the 15th percentile, and weight-for-length is at the 25th percentile. Of the following, the BEST next step in management is

esophagography - Late-onset (older than 6 months of age) vomiting with persistent symptoms. - Her difficulty associated with eating solids is suggestive of dysphagia, and therefore causes of esophageal dysfunction, including congenital esophageal stricture and vascular ring, should be considered. Esophagography is the test of choice to delineate esophageal anatomy. - This patient's esophagography identified a congenital esophageal stricture

A 14-year-old boy presents for a health maintenance visit. He has no complaints and is doing well in school. Past history is unremarkable except for occasional minor upper respiratory infections and an episode of viral pneumonia at age 3 years. Family history is positive for hypertension and coronary artery disease in the father. Growth and development are normal and immunizations are current. Height and weight are at the 75th percentile. Temperature, pulse, and respiratory rate are normal, with blood pressure at 135/92 mm Hg (above the 95th percentile, but below the 99th percentile plus 5mm Hg for age, height, and gender). He is at Tanner Stage III. The remainder of the examination is normal. What is the most likely cause of this boy's hypertension?

essential hypertension - In adolescents, any average BP values above 120/80 mm Hg are classified as prehypertensive even if below the 90th percentile. - If there is a discrepancy as to percentile between systolic and diastolic BP values, the higher is used. Uncontrolled hypertension can lead to multiple adverse outcomes, including cardiovascular, cerebrovascular, renal, and ocular disease.

A 15-year-old adolescent boy is brought to the office for a health supervision visit. He has a history of mild intermittent asthma. He is not receiving any medications. The boy's mother has a history of high blood pressure and diabetes. The boy's weight is 100 kg (>95th percentile), height is 170 cm (50th percentile), and body mass index is 34.6 kg/m2 (>95th percentile). His heart rate is 80 beats/min, respiratory rate is 15 breaths/min, and blood pressure is 140/86 mm Hg using an oscillometric device. A repeat blood pressure with the auscultatory method is 136/82 mm Hg. Physical examination shows dark, velvety skin discoloration on the back of his neck and axilla. He has no abdominal bruit. Neurologic examination findings are unremarkable. Laboratory results reveal normal serum electrolytes, renal function, and thyroid function. Hemoglobin A1C is 6%. Results of urinalysis with microscopy are normal. Of the following, this boy's MOST likely diagnosis is

essential hypertension - features supporting the diagnosis of essential hypertension are obesity (body mass index >95th percentile), family history of hypertension, presence of acanthosis nigricans, elevated hemoglobin A1C, and an unremarkable laboratory evaluation for secondary causes of hypertension - Pharmacologic intervention is indicated for stage 2 hypertension, symptomatic children, presence of end-organ damage (eg, left ventricular hypertrophy), and stage 1 hypertension not responsive to lifestyle modification. The initial pharmacologic intervention options for primary hypertension include an angiotensin-converting enzyme inhibitor, angiotensin receptor blocker, calcium channel blocker, or thiazide diuretic. The choice of antihypertensive in secondary hypertension will vary depending on the underlying cause. The goal is to reduce the systolic and diastolic blood pressure below 120/80 mm Hg for adolescents and below the 90th percentile blood pressure for children younger than 13 years.

Children with PRS can have conductive and/or sensorineural hearing loss, which presents in the neonatal period or can develop over time. In addition to the newborn hearing screen, an audiologic evaluation should be performed how often?

every 6 months through 5 years of age, and annually thereafter - A genetics workup is strongly recommended for neonates with PRS. - At minimum, a high-resolution karyotype and microarray should be performed. - A detailed ophthalmologic assessment should take place at birth and 6 months of age. - A targeted family history should be obtained regarding the presence of clinical features suggestive of Stickler syndrome, such as osteoarthritis, myopia, and short stature. - Stickler syndrome, a connective tissue disorder, is associated with midfacial hypoplasia, cleft palate, Pierre Robin sequence, hearing loss, and eye abnormalities including high-grade myopia, cataracts, and increased risk for retinal detachment.

3 yo M + nml left testicle but right testicle not palpable. Next step ...

exam under anesthesia - with inguinal orchiopexy or laparoscopy as indicated

Amoxicillin-clavulanate is the drug of choice for treatment of dog, cat, or other mammal bites. For children with penicillin allergy, oral alternatives are

extended-spectrum cephalosporin plus clindamycin or trimethoprim-sulfamethoxazole plus clindamycin

Choosing the right formula can be a bit of trial and error. For children who have food allergies and eczema, trying what formulas is a good place to start?

extensively hydrolyzed formula such as Nutramigen or Alimentum - If allergy symptoms are poorly controlled, then trying an amino acid based formula such as EleCare is the way to go - Just remember to check that the formula is fortified with iron. Low-iron formulas are nutritionally inadequate - Nutramigen is made from cow's milk. Elecare is made from synthesized amino acids and sometimes plant sugars. Nutramigen is an extensively hydrolyzed formula which means that its protein source is derived from cow's milk and broken down. Elecare is an elemental formula which means that the formula is made of amino acids. Both are hypoallergenic but Elecare is is given to babies who are allergic to extensively hydrolyzed formulas like Nutramigen.

7 mo old infant, born at term, presents for evaluation of possible dev'tal delay. Her delivery was complicated by nuchal cord, perinatal asphyxia, and neonatal seizures. What primitive reflexes may still be considered a normal finding among infants in this age group?

extensor plantar response / babinski reflex - usually disappears between 9-12 mos - can be seen as late as 24 mos

A 4-week-old Chinese American girl had jaundice shortly after birth that was treated with phototherapy and appeared resolve at 1 week of life; it reappeared at 2 weeks with steady progression. She has had slight difficulty feeding both by breast and bottle, but she appears to have gained weight. Urine is dark and stools are light. History is remarkable only for physiologic jaundice. Birth weight at term was 7 lb, 6 oz. Today's weight is 8 lb, 4 oz (50th percentile). Skin and sclera are markedly icteric. Liver is firm and palpable 2.5 cm below the right costal margin. The spleen is palpable 1 cm below the left costal margin. Total bilirubin is 23 mg/dL with a direct fraction of 18.2 mg/dL. Alpha-1-antitrypsin testing reveals a PIMM phenotype. Studies for hepatitis A, B, and C are negative. DISIDA (hepatobiliary scintigraphy) scan shows non-excretion with good hepatic uptake. No evidence of extrinsic biliary duct compression. What is the most likely diagnosis?

extrahepatic biliary atresia - usually presents in the first month of life with largely direct hyperbilirubinemia (often after physiologic jaundice has subsided), dark urine, and acholic stools. While various etiologies have been proposed, the endpoint is obliteration of all or part of the extrahepatic biliary tree. - Hepatobiliary scintigraphy shows non-excretion. - The alpha-1-antitrypsin PIMM phenotype is normal.

More likely with hepatitis b infection than hepatitis A infection ...

extrahepatic manifestations, like - polyarthralgia or arthritis - macular or urticarial rashes - thrombocytopenia

Stridor is a hallmark of ...

extrathoracic obstruction

Consider bariatric surgery only in mature adolescent dedicated to losing weight and able to give informed consent with supportive and committed family. Additional criteria include ...

failure to lose weight after >/= 6 mos of organized attempts at weight loss physiologic mature per tanner staging BMI > 120%ile severe comorbidity OR >140%ile without severe comorbidity

The introduction of an air bubble in a capillary glass tube allows the oxygen in the bubble to diffuse into the capillary blood. The partial pressure of carbon dioxide present in the air bubble is much lower than that in blood, setting up a gradient and allowing CO2 to diffuse from the blood sample into the air bubble. This will falsely result in ...

falsely increased paO2 falsely decreased pCO2

Red flags for organic gastrointestinal diseases

family history of autoimmune disease weight loss/failure to thrive poor linear growth hematochezia or hematemesis nocturnal stooling tenesmus - further testing to evaluate these conditions should occur

3 yo + difficulty walking. Few months ago, she began having difficulty walking that are of sudden onset and last for several minutes. Family states she "seems to be drunk" and has to lie down on the ground. During these episodes, she is pale and refuses to move but is responsive throughout the episode and immediately returns to baseline after the event. Episodes have recurred several times. Parents show you video of her abnormal eye movements when she tries to get up during an event. She has normal neuro exam. What family hx is consistent with this diagnosis?

family hx of migraines - Paroxysmal vertigo of childhood = 1-4 yo = sudden onset, recurrent vertigo = ataxia, nystagmus, pallor, emesis - unlike with seizure, they have retained consciousness with no postictal state - this is a precursor to migraines

Reye syndrome liver biopsy =

fatty infiltration - and cerebral edema

What may contribute to tinea versicolor?

favorable cutaneous lipid pattern hereditary factors (a positive family history is common) excess skin asparagine alterations in cell-mediated immunity

The most common test used to diagnose pancreatic insufficiency is

fecal elastase-1 level - Elastase-1, produced by the pancreas, is not degraded by intestinal proteases and is not impacted by the use of PERT - A low (<100 μg/g) elastase-1 level correlates with EPI; elastase levels from 100-200 μg/g are indeterminate; levels >200 μg/g are associated with pancreatic sufficiency *Watery stools may result in a falsely low elastase-1 level

Risk factors for anorexia nervosa ...

female gender (3x more common) family hx higher socioeconomic status family distress sexual abuse hx of dieting certain athletic endeavors

A 14-year-old girl who plays softball for school has noticed that she is taller than many boys her age. She first noticed breast development 3 years ago and experienced menarche 4 months ago. Her father is 6'2", and her mother is 5'5". Growth and development have been normal and immunizations are current. Vital signs are normal, with a weight of 120 pounds (50th percentile) and a height of 5'6" (75th percentile). Areolae are present as secondary mounds above the breast contour (breast stage IV) and copious long dark curled pubic hairs not extending to the medial thighs (pubic hair stage IV). Why is she taller than boys her age?

female sex - Girls begin linear growth acceleration earlier than boys. - The longer period of prepubertal growth in boys and greater peak height velocity accounts for a higher average adult height in men. - This patient will not experience much further linear growth.

A 5-year-old girl is undergoing an evaluation for possible leukemia. Before her bone marrow aspiration procedure, she is given an anxiolytic and analgesic. She continues to be very apprehensive and uncooperative, causing her dosage to be increased. Just before the completion of the procedure, she reports not being able to breathe well and pain around her chest. What medication is most likely causing this side effect?

fentanyl

A 5-year-old girl is undergoing an evaluation for possible leukemia. Before her bone marrow aspiration procedure, she is given an anxiolytic and analgesic. She continues to be very apprehensive and uncooperative, causing her dosage to be increased. Just before the completion of the procedure, she reports not being able to breathe well and pain around her chest. What medication is most likely causing this side effect?

fentanyl - High doses of fentanyl are associated with chest wall stiffness and rigidity, especially with rapid titration - It is a potent opioid analgesic used for painful procedures that may be combined with midazolam to reduce anxiety

Fetal exposure to warfarin during 6-9 weeks gestation can cause...

fetal warfarin syndrome with nasal hypoplasia and stippled epiphysis

Absolute contraindication to sports participations

fever acutely

Bronchiolitis is diagnosed clinically in children younger than 2 years without underlying predispositions who exhibit the typical constellation of signs and symptoms including

fever, rhinorrhea, cough, wheezing, and tachypnea

First permanent teeth to erupt usually include ...

first molars or lower central incisors = "6-year-molars" - usually 6-7 yo - most children have 28 permanent teeth by 13 yo (erupt at 17 - 21 yo)

Gender studies in non-transgender participants have found that children are aware of their gender by what age?

five or six - and often earlier

17 yo girl complains of decreased exercise tolerance over preceding several months. She is especially concerned because she has always been an outstanding athlete. She has no hx of any chronic medical problems and takes no daily medications. On exam, you note a finding consistent with a congenital heart lesion that may be asymptomatic until the 2nd decade of life or later, depending on the severity of the defect. What auscultatory findings do you likely hear?

fixed split 2nd heart sound - ASD = wide fixed splitting of 2nd heart sound - exercise intolerance = often the first symptom to arise as a result of an ASD ~ most often presents in 2nd and 3rd decade of life - sx closure = complete resolution

Tx for pts with Hirschsprung-associated enterocolitis (HAEC) ...

fluids resc + nasogastric decompression + broad-spectrum abx + rectal irrigation

Infant has hyponatremic dehydration because of an excessive loss of sodium from gastroenteritis and attempted rehydration with diluted formula. The result is low serum osmolality, which in turn leads to

fluids shifting from the extracellular fluid (ECF) compartment to the intracellular fluid (ICF) compartment - To balance the osmoles, water moves from ECF to ICF in hyponatremic conditions - Treatment of hyponatremia should be gradual, and the target rise in sodium should not be more than 0.5 mmol/L/h or 10 to 15 mmol/L in 24 hours - A rapid correction of hyponatremia leads to osmotic shift of water from the brain cells into the ECF causing central pontine myelinolysis

Six-year-olds begin to develop an awareness of their peers and make meaningful relationships with peers and adults. Other skills include

following simple directions counting to 100 demonstrating basic writing skills demonstrating emerging reading skills such as recognizing words on sight or reading simple books - Participation in sports begins, but the focus is on participation rather than competition

parachute reflex protects child in event of a fall. infant will extend arms forward as if to break a fall. reflex develops around 6-9 months and normally persists how long?

for life

Hydrocephalus develops in approximately 90% of patients with meningomyelocele. Ventriculomegaly is present at birth in about 20% of cases and develops after closure of the defect in about 70% of cases. Leakage of CSF from the sac may be related to absence of ventriculomegaly at birth, but it is not protective after closure. It is usually associated with a Chiari II malformation, which consists of downward displacement of the lower brainstem through the foramen magnum. In this case, obstruction occurred at the level of

foramina of Luschka and Magendie - causing dilatation of the lateral, 3rd, and 4th ventricles

Biphasic stridor is associated with fixed central airway obstruction such as external compression, like ...

foreign body or subglottic stenosis

Polycystic ovary syndrome is the most common cause of chronic anovulation and infertility in young women. The diagnosis of polycystic ovary syndrome in adolescent girls is often based on evidence of hyperandrogenemia and ovulatory dysfunction. The most sensitive test for diagnosis in women is

free and total testosterone level

Can increase sickling in acute pain crisises

furosemide/lasix/diuretics

A 2-day-old newborn, a vaginal delivery of a 29-year-old G2P1Ab0 Caucasian woman, presents regarding sex assignment. The newborn was born at term and weighs 2.8 kg. Prenatal, birth, and family histories are unremarkable. It cannot be determined if the phallus is an enlarged clitoris or a slightly small penis. The urethral meatus arises at the base of the phallus. What appears to be a scrotum is present, without palpable gonads. Chromosome analysis revealed a 46,XX pattern. What is the true nature of the apparent scrotum?

fused labia - The human embryo is sexually undifferentiated until 8 weeks gestation. It possesses both Wolffian structures, which in an ultimately determined male embryo differentiate into the vas deferens, epididymis, and seminal vesicles; and Müllerian structures, which in an ultimately determined female embryo differentiate into Fallopian tubes, the uterus, and the upper 1/3 of the vagina. - In a genetic female, excess testosterone (e.g., in cases of congenital adrenal hyperplasia or maternal testosterone exposure) can lead to labial fusion, which is what is described in this clinical scenario; labial fusion can look like a scrotum.

Symptoms of serum sickness-like reaction

generalized itchy urticarial wheels generalized malaise irritability headache diarrhea increased temperature lymphadenopathy facial edema arthralgias

Current recommendations are that all index patients with established or possible hypertrophic cardiomyopathy and all at-risk family members first undergo ...

genetic testing

For the child diagnosed with ASD, the pediatrician should conduct a medical workup that includes

genetic testing (eg, chromosomal microarray analysis, fragile X analysis) and/or referral to genetics - The pediatrician should also ensure that the child with ASD is referred for and/or receiving appropriate therapy and services. - Those younger than 3 years should be referred to Early Intervention, whereas those aged 3 years and older should be referred to their local school district (special education).

During a healthcare maintenance visit, a 4 yo boy is noted to have a murmur. His BP is 80/55. His HR is 76 and regular. You recognize the murmur as benign and not indicative of a structural cardiac defect. What best describes this heart murmur?

grade 2/6 continuous murmur, heard best throughout systole and diastole, that is heard best in the 1st and 2nd right intercostal spaces and varies in intensity just by changes in the neck = venous hum - turbulent flow via jugular venous system

Murmur of ASD ...

grade 2/6 systolic ejection murmur, best heard at left upper sternal border, with normal S1 and widely split S2 that does not vary with respiration - left to right shunting --> large volume overload - fixed, split S2

More severe coarctations are associated with ...

greater reduction of flow and *increased afterload*

Development in children is sequential and orderly and occurs in what direction ...

gross motor = cephalocaudal and proximodistal fine motor = midline to lateral

Most pts with intellectual disability have preserved ...

gross motor function

9 yo boy with hx of atopic dermatitis with 2 day hx of painful swelling at tips of several fingers. Fluid-filled, tense blister with surrounding erythema located over volar fat pad on distal portion of both 3rd and 4th fingers of right hand. Most common cause of these clinical findings?

group A beta hemolytic strep = blistering distal dactylitis - bullae initially tense --> center may eventually erode - bullae may require incision and drainage + abx

Milder forms of autism may not be diagnosed until later in life when adolescents have difficulty with social interactions. Best tx?

group-based therapy

A 6-year-old boy was diagnosed with type 1 diabetes last year and presents for his first follow-up. His single mother had to cancel his last two appointments. He lives with his mother and three siblings. His mother works full-time; he and his siblings are cared for by his aunt, with three of her own young children. His mother is tired and has forgotten to bring his blood sugar recordings. She thinks they sometimes range high, but she is not sure how to adjust his insulin dose, and this usually happens while she is at work. He has remained on the same dose of insulin since his hospital discharge. She says her sister sometimes forgets to check his blood sugar because two of her own children have special needs that take up much of her time. She says his appetite seems normal. What long-term complication would be most likely in this patient?

growth disturbance - Chronic undertreatment with insulin will negatively affect linear growth, resulting in decreased growth velocity and delayed skeletal and sexual maturation: downward crossing of height and weight percentiles and eventual short stature.

In children immunized against h influenzae type B, most common cause of epiglottitis is are ...

h influenzae serotypes A, F, or non-typeable strains strep pneumo strep pyogenes staph aureus

10 yo boy with skin rash. Itchy, rough, red. Appears after sun exposure. PE = ataxia and bilateral nystagmus. Cause?

hartnup disorder - AR

Sports participation in athletes with implantable cardiac defibrillators (ICDs) may be considered if the athlete

has not received any ICD shocks from episodes of ventricular flutter or fibrillation for at least 3 months

A previously healthy, 3-week-old girl, born at term, is admitted to the pediatric ward for fever of 38.9°C. The neonate has been sleepy and not feeding well since the day prior to admission. Her temperature is 37.7°C (after 1 dose of acetaminophen), heart rate is 180 beats/min, respiratory rate is 40 breaths/min, and oxygen saturation by pulse oximetry is 97% in room air. Her anterior fontanelle is bulging, capillary refill time is 4 seconds, and she is sleepy but arousable. Cardiac and respiratory examination findings are normal. A complete evaluation for sepsis is initiated, and appropriate antibiotic therapy is administered. Results of a complete blood count and basic metabolic panel are normal. Bacterial culture from the cerebrospinal fluid is positive the following day for Streptococcus pneumoniae. After 72 hours, blood and urine culture results are negative. Of the following, this child should MOST likely be evaluated for

hearing loss - All children with bacterial meningitis should undergo evaluation for hearing loss at the time of diagnosis with repeat evaluation at 4 to 8 weeks, 6 months, and 12 months after discharge. - Children with mild to severe hearing loss can be fitted for hearing amplification devices beginning as young as 3 months of age. - For the best functional outcome, hearing loss should be identified in all infants by age 3 months, according to the Universal Hearing Screening Program guidelines. Children with hearing loss often coo and babble like other infants initially, but then lose these skills after 6 to 9 months of age. They do not continue to develop speech, such as saying "mama" or "dada" at the typical age of 12 to 15 months. - Based on this and other evidence, the Universal Hearing Screening Program recommends that interventions for infants with hearing loss should begin by 6 months of age, and the child should be established in a medical home.

Spurious hyperkalemia most likely caused by...

hemolyzed specimen thrombocytosis >450, platelets leukocytosis >100,000

Postcircumcision bleeding is a common presentation of ...

hemophilia - get PT/PTT/INR

A 4-year-old boy recently adopted from an orphanage in Myanmar is being seen for a routine health supervision visit. The parents do not report any complaints. He arrived in the United States approximately 2 weeks earlier. One week earlier, he was evaluated at the local health department with complaints of low-grade fever of 3 days' duration with associated malaise, loss of appetite, and emesis. Hepatitis serologic testing performed at the local health department is positive for hepatitis A virus (HAV)-specific total antibody (IgG plus IgM) with a positive serum IgM antibody to HAV. He has not received immunization against HAV. The boy lives with his adoptive parents and 12-month-old sister, all of whom are asymptomatic. The sister has not received her vaccination against HAV. On physical examination, the boy is afebrile and well appearing with normal vital signs. No scleral icterus is noted. His abdomen is soft and nontender, with no hepatosplenomegaly. The rest of the physical examination findings are normal. Laboratory evaluation is notable for elevation of liver enzymes but normal level of serum bilirubin. Supportive care is recommended for the child with viral hepatitis. Of the following, the BEST next management step for the postexposure prophylaxis of the 12-month-old sister is administration of

hepatitis A vaccine - The hepatitis A vaccine is licensed for use in people aged 12 months through 40 years and is recommended for postexposure immunoprophylaxis of HAV among unimmunized individuals within this age range. - Administration of immune globulin (Ig) is recommended for postexposure immunoprophylaxis of HAV for exposed infants younger than 12 months. - Prevention measures against HAV include routine immunization of all children 12 to 23 months of age with the hepatitis A vaccine.

An 18-month-old boy is brought to the office for a health supervision visit. His mother is concerned about his resistance to going to sleep and picky eating. The boy's medical history is not significant. His growth, development, and physical examination findings are within normal limits for his age. His mother is pregnant, and worried about how she is going to manage her family's needs when her second child is born. Neither parent is employed, and the family is having a difficult time making sure they have a stable place to live and food to eat. Of the following, the MOST appropriate referral to address this family's needs is

home visiting program - The family in the vignette, consisting of expectant parents and a young child, is struggling with multiple needs and could benefit from help with their parenting skills, food insecurity, and housing insecurity. A home visiting program can provide an array of services such as family support, counseling, case management, and parenting training, to address the challenges faced by this at-risk family. - Families can call the national 211 helpline for information on resources in their community that address food, housing, employment, and health care needs.

A neonate, born prematurely to a 35-year-old primigravida mother due to maternal preeclampsia, is seen in the neonatal intensive care unit. The mother recently emigrated from Southeast Asia and did not receive prenatal care in the United States. The results of maternal hepatitis B virus screening are pending. The mother's laboratory findings include an elevated alanine aminotransferase level of 1,500 U/L and an aspartate aminotransferase level of 900 U/L. The neonate's weight is 1,500 g (3 lb, 5 oz); physical examination findings are normal with no hepatomegaly. Of the following, the BEST next step in the management of this neonate is to administer

hepatitis B vaccine now and immunoglobulin within 12 hours of birth - Neonates born to mothers with positive hepatitis B surface antigen or unknown status should receive hepatitis B vaccination and immunoglobulin within 12 hours when birth weight is <2,000 g or within 7 days for those weighing ≥ 2,000 g. - The mother's hepatitis B virus (HBV) infection status is unknown. She recently emigrated from Southeast Asia, an endemic region for HBV infection, her liver enzyme levels are elevated, and the neonate's birth weight is 1,500 g ( <2,000 g). This infant should continue the vaccine series beginning at 1 to 2 months of age based on mother's hepatitis B surface antigen (HBsAg) result (ie, administer 3 additional hepatitis B vaccine doses with single-antigen vaccine at ages 1, 2 to 3, and 6 months or a hepatitis B-containing combination vaccine at ages 2, 4, and 6 months). - Hepatitis B immunoglobulin prophylaxis is recommended for neonates born to mothers with a positive HBsAg or unknown HBV status. In the absence of post-exposure HBV prophylaxis, the risk of acquiring HBV from an infected mother is 70% to 90% for infants born to mothers who are HBsAg and HBeAg positive. HBV acquisition risk is 5% to 20% for infants born to HBsAg-positive but HBeAg-negative mothers. - HBV immunoprophylaxis is most effective if administered within 12 hours of birth. - Breastfeeding should not be delayed after the infant is immunized. - HBsAg and anti-HBs should be checked in infants born to HBsAg-positive mothers 1 to 2 months after the last vaccine dose (test ≥9 months of age).

Name this tumor: - increased risk for preterm infants - Beckwith-Wiedemann = increased risk - AFP is high - most common primary malignancy of liver for children under 3 yo

hepatoblastoma

A 6-year-old child presents with an inability to stand and walk without support; his speech is slurred. On examination, he is alert; his physical examination detects a lack of tendon reflexes, nystagmus, and marked scoliosis. His sitting posture is unsteady and there is no resistance to neck flexion. What disorder is most likely present?

hereditary degenerative disorder - Friedreich's ataxia - becomes apparent between 2 and 16 years - characterized by kyphosis, absence of tendon reflexes indicating degeneration of the spinal cord, and dysarthria (almost universally present) - autosomal recessive inheritance - progression is rapid, but shows no sign of ophthalmoplegia or dementia - nystagmus is a frequent part of the clinical picture; nystagmus is an oscillating eye movement with slow movement in 1 direction and a subsequent saccadic jerk back to the midline of gaze. The direction of nystagmus refers to the fast component. - Various biochemical defects have been implicated in Friedreich's ataxia.

A neonate is born via spontaneous vaginal delivery to a woman with painful genital ulcers. Maternal membranes were ruptured for 18 hours, and fetal heart tracings were reassuring. A genital lesion swab is sent for herpes simplex virus (HSV) polymerase chain reaction testing, along with maternal serology (IgG and IgM) for HSV-1 and HSV-2. The neonate is well appearing. His temperature is 36.5°C, respiratory rate is 45 breaths/min, heart rate is 110 beats/min, and oxygen saturation is 99% on room air. He weighs 5 lb, 6 oz. The remainder of his physical examination findings are unremarkable. Of the following, the BEST next management step for this neonate is to obtain

herpes simplex virus surface culture at 24 hours after birth - Beonatal surface HSV cultures and a blood specimen for HSV DNA polymerase chain reaction (PCR) testing should be obtained at 24 hours after birth. - Detection of HSV at 24 hours after birth most likely reflects neonatal viral replication rather than contamination from maternal lesions. - Signs of neonatal infection may not be apparent until the second to fourth week after birth.

A 20-month-old boy presents with a 1-week history of fever up to 101°F and irritability. His mother noted sores in his mouth 4 days ago; she states that she has noticed him drooling and that his appetite is quite diminished. His past medical history is unremarkable. He has no medical allergies and his only current medication is acetaminophen. He is current on his immunizations. His physical exam reveals normal vital signs except for a temperature of 100.5°F. On examination of his oral cavity, you note swollen erythematous gingiva with ulcerations present mostly on the left of his mouth. The ulcerations appear yellowish-white and friable. White-gray lesions approximately 3 mm in diameter are seen on the anterior tongue. The tonsils appear erythematous without exudates. His lips are slightly cracked, and his mucous membranes are slightly tacky. Neck examination reveals bilateral anterior cervical adenopathy. He has no skin lesions. The remainder of his exam is normal. His strep test is negative. What is the most likely cause of this patient's condition?

herpetic gingivostomatitis - caused by herpes simplex virus type 1 - is the most common cause of stomatitis in children 6 months to 5 years old - symptoms may appear abruptly, with fever, drooling, fetid breath, and refusal to eat - fever may precede the oral lesions by 4 days and presage to a more insidious onset of the disease - tongue, cheeks, and gingiva are most commonly affected, but the entire oral cavity may be involved - these areas can present with ulcers that are yellowish-gray in color with a red halo surrounding the lesions, and the gingiva may be quite friable - drooling may be present secondary to the pain associated with chewing and swallowing, and dehydration is a real concern in the management of the patient - cervical and submaxillary adenitis is common - acute phase may last 1-3 weeks - oral acyclovir may be an option if caught within 72-96 hours and the child is having trouble with hydration due to painful lesions

The mother of a 10-year-old boy with a history of leukemia calls your office very concerned that she just found out that her son was closely exposed to a friend 4 days ago who had a mild rash that has now been diagnosed as chicken pox. So far her son seems fine but she has many questions including what to expect and what they should do. Which of the following manifestations of varicella is most likely to occur in this patient?

high fevers - Immunocompromised patients who have leukemia and other malignancies are at high risk for severe disseminated varicella disease. This may also be increased by concomitant use of immunosuppressive agents. These patients are more likely to have high fevers, extensive and prolonged rashes that are diffuse involving the palms and soles, and prolonged communicability. - They have trouble clearing the viremia so that the virus may spread to other organs, especially the lungs, liver, and central nervous system resulting in viral pneumonia, hepatitis, and meningoencephalitis despite antiviral therapy. - Children with leukemia have a 7% mortality rate. - Passive immunization with varicella-zoster immune globulin, now available as VariZIG from Canada with central review board approval, can prevent symptomatic infection although it cannot clear an established infection. IV immune globulin is an alternative if not available. Treatment of infection with IV acyclovir is also recommended.

16 yo girl comes to office with complaints of frequent sore throat, chest pain while lying flat, occasional vomiting. Notices she constantly needs breath mints due to "nasty" taste in her mouth. Denies cough, congestion respiratory difficulty, recent trauma, illicit drug use. Takes no meds. Vitals stable, and PE unremarkable. What symptom is most likely found in teens with this medical condition?

hoarseness - via reflux-induced laryngitis - GER

Boy has gram-negative bacteremia after travel abroad, which is most concerning for typhoid fever. Typhoid fever is endemic in many resource-limited countries. Most cases in the United States occur after travel to Pakistan, India, Mexico, and Bangladesh. Most likely source of infection is:

humans = Humans are the source of enteric fever infections, including infections caused by Salmonella enterica serovars Typhi, Paratyphi A, and Paratyphi B. - Typhoid fever must be considered in the differential diagnosis of a febrile illness in any traveler returning from a resource-limited country. - Nontyphoidal Salmonella infections typically manifest as gastroenteritis. Typhoidal Salmonella infections are characterized by fever, constitutional symptoms, and abdominal pain. - Ceftriaxone is usually the first-line parenteral antibiotic used in the treatment of invasive Salmonella infections.

A 14-year-old adolescent boy is being evaluated for persistent symptoms after he sustained a concussion while playing football. He was tackled during a game, hitting his head against the ground with loss of consciousness for an estimated 5 seconds. He immediately complained of headache and vertigo and was removed from play. Head computed tomography performed at that time was normal. Over the next 3 months, he complained of persistent dull headaches, intermittent vertigo, insomnia, and moodiness. He had trouble focusing and his grades were falling, which he attributed to feeling constantly in a fog. Before his concussion, the boy had episodic migraine headaches, which occurred infrequently and responded to ibuprofen. There is a family history of depression, and the patient had seen a counselor a few years ago for anxiety symptoms. At this visit, he is wearing sunglasses. He is alert and answering questions appropriately but appears disengaged. Notable neurologic findings include difficulty with recall of 3 objects after 5 minutes, performance of serial 7s, and tandem gait testing. Of the following, the element of his history that PREDISPOSES him to this presentation is

hx of anxiety Postconcussive syndrome - a neurocognitive disorder affecting approximately 10% to 15% of those with concussion - symptom onset is within 4 weeks after a concussion and lasts a minimum of 3 months Risk factors for the development of postconcussive syndrome include: - pre-existing psychiatric disorders such as anxiety, depression, or poor coping skills - multiple prior concussions - history of migraine headaches - learning disorder - factors related to the concussion itself (severe initial symptom burden or delayed symptom onset, loss of consciousness for >1 minute, vestibular symptoms)

Term infant delivered with tight nuchal cord leading to initial respiratory depression responsive to PPV. Apgars were 5 and 8 at 1 and 5 minutes. He remained with his mother for normal newborn care and was discharged at 48 hours of age. During routine f/u care with his pediatrician at 12 days of age, he is noted to have several indurated, reddish nodules and plaques on on lower back and buttocks. These clinical findings place the patient at potential risk for for what metabolic disorders?

hypercalcemia - subcutaneous fat necrosis - typically self-limiting with resolution by 6-8 weeks of age - f/u for dev't of hypercalcemia in first 6 mos of life is recommended

What do the hands look like in icthyosis vulgaris ...

hyperlinear palms with accentuated skin markings

18 yo M + several weeks of fatigue and weight loss. CBC = WBC 1600, hgb 5.4, plt 37,000. HIV +. Bone marrow biopsy shows ...

hypocellular bone marrow with fatty filtration - aplastic anemia - pancytopenia - radiation, viruses, idiopathic, meds

Fanconi anemia bone marrow aspiration would show ...

hypocellular marrow with fatty infiltration - inherited bone marrow failure syndrome - often initially presents with thrombocytopenia - cytopenias + dysmorphic facies - tx = stem cell transplant

Child with cystic fibrosis (CF) who has excessive loss of electrolytes with sweating would have what electrolyte imbalances: - Cl - Na - Bicarb - K

hypochloremia hyponatremic dehydration metabolic alkalosis (low bicarb) hypokalemia - he defect in the cystic fibrosis transmembrane regulator gene leads to increased sodium chloride losses from the sweat gland. - The chloride depletion leads to extracellular volume contraction and metabolic alkalosis. Hypokalemia occurs as a result of losses with increased strenuous activity and heat. - Sodium chloride supplementation prevents the risk of electrolyte disturbances in the setting of fever, vomiting, diarrhea, strenuous activity, or exposure to heat.

Children with cystic fibrosis are at risk of experiencing what electrolyte imbalances?

hypochloremia, hyponatremic dehydration, hypokalemia, and metabolic alkalosis - because of loss of electrolytes with sweating - sodium chloride supplementation prevents the risk of electrolyte disturbances in the setting of fever, vomiting, diarrhea, strenuous activity, or exposure to heat

Kids with CF at risk of what in hot weather?

hyponatremia

7 yo boy + 48 hours of fevers, fatigue, anorexia, diffuse myalgias, headache. Upon awakening on day of presentation, parents noticed a rash that has continued to worsen. Temp is 102.6 F. He appears ill and has an erythematous, maculopapular, and petechiael rash that includes the trunk, and most prominently, the wrists, ankles, palms, and soles. Lab findings most likely to be found in this pt?

hyponatremia - RMSF - via Rickettsia rickettsii - labs = hyponatremia, thrombocytopenia, elevated transaminases, leukopenia, anemia - tx = doxycycline

Low calcium and high phosphorus levels are consistent with

hypoparathyroidism - low PTH level results in hypocalcemia and hyperphosphatemia - treatment of hypoparathyroidism = oral calcium and calcitriol (1,25-dihydroxyvitamin D), the activated form of vitamin D

A 1-day-old neonate is admitted to the neonatal intensive care unit for persistent hypoglycemia. He was born at 40 weeks' gestation by cesarean delivery because of failure of labor progression. The pregnancy was otherwise uncomplicated. His birth weight was 3400 g. Jitteriness noted at 8 hours after birth prompted measurement of a plasma glucose level, which was 28 mg/dL (1.6 mmol/L). Despite treatment with oral feedings of standard infant formula, the neonate was unable to maintain a plasma glucose level above 50 mg/dL (2.8 mmol/L). He is requiring a glucose infusion rate of 8 mg/kg/min to maintain normal glucose levels. He is afebrile and has normal vital signs for age. Physical examination findings are normal except for the genital examination. His right testicle is not palpable, and his left testicle is palpable high in the scrotum. The stretched phallic length is 1.6 cm. Of the following, the MOST likely etiology of this neonate's hypoglycemia is

hypopituitarism - His small penis size and undescended testicle with the second testicle found high in the scrotum are manifestations of hypogonadism due to gonadotropin deficiency - Hypogonadism associated with persistent hypoglycemia is consistent with hypopituitarism - His hypoglycemia is due to other anterior pituitary hormone deficiencies (adrenocorticotropic hormone, growth hormone) - Failure of labor progression is another feature of hypopituitarism

In DeGeorge syndrome, thymic aplasia results in T-cell def and ...

hypoplasa of parafollicular cortex of lymph nodes

Initiating event in Pierre-Robin sequence ...

hypoplasia of mandible / micrognathia - develops before 9 weeks gestation

A 6-week-old female infant presents with failure to gain weight since birth. She does not accept feeds well and remains sluggish. She was born full-term, birth weight 2.9 kg. Apgar scores were 8 (1 minute) and 9 (5 minutes). Physical examination reveals lethargy and hypothermia. Temperature is 35°C, and skin has mottled appearance. She has icterus with an indirect serum bilirubin of 18 mg/dL. What is the most likely diagnosis?

hypothyroidism - poor feeding, failure to gain weight since birth, sluggishness, and hypothermia - prolongation of physiological jaundice due to delayed maturation of the glucuronide conjugation - macrocytic anemia is often present

Risk factors for SCFE ...

hypothyroidism and GH deficiency

Most common cause of seizures in newborn

hypoxia

A 16-year-old adolescent boy is seen for concerns of abdominal pain and loose stools. His symptoms began several years ago but have worsened over the last year. He now has pain several times a week, accompanied by loose bowel movements. Defecation seems to improve his abdominal pain. He reports fecal urgency and nocturnal stooling, although he has not seen blood in his stool. He has missed 22 days of school in the last year because of these symptoms. He has a history of exercise-induced asthma. His father has psoriasis. He does not take any prescribed or over-the-counter medications. His weight is greater than the 99th percentile for age, his height is at the 62nd percentile for age, and his body mass index is greater than the 99th percentile for age. He is in no apparent distress. His abdominal and rectal examination findings are normal. His stool is negative for occult blood. Laboratory and endoscopic evaluation (upper intestinal endoscopy and colonoscopy) are normal. Irritable bowel syndrome is suspected. Of the following, the component of his history that BEST meets the diagnostic criteria for the suspected diagnosis is

improvement in abdominal pain with defection - The adolescent boy in this vignette has suspected irritable bowel syndrome (IBS), a common pediatric functional gastrointestinal disorder defined as abdominal pain associated with a change in defecation. - He describes abdominal pain associated with loose stools with improvement in the pain after defecation. This finding, in the absence of an organic cause, is consistent with IBS The diagnostic criteria (Rome IV) developed by the Rome Foundation for pediatric IBS include - Abdominal pain more than 4 times monthly associated with defecation, change in frequency of stool, and/or change in stool appearance - Continued abdominal pain despite treatment of constipation (when constipation is present) - After appropriate evaluation, the symptoms cannot be fully explained by another medical condition

Treatment of an abscess is primarily through

incision and drainage (I&D) - Smaller abscesses (<5 mm) may resolve spontaneously with the application of warm compresses and antibiotic therapy - Larger abscesses will require I&D as a result of an increase in collection of pus, inflammation, and formation of the abscess cavity, which lessens the success of conservative measures Equipment - Universal precaution materials (gown, gloves, protective eyewear) - Sterile draping towels and sterile gloves - Local anesthetic (1% or 2% lidocaine with or without epinephrine) - 10-cc syringe and 25- to 30-gauge needle - Skin prep material (chlorhexidine [Hibiclens] or iodine swabs) - No. 11 or 15 blade and scalpel - Curved hemostats - Scissors - Packing (plain or iodoform) ribbon gauze - Dressing (4- × 4-inch gauze pads and tape) - Prep the surface of the abscess and surrounding skin with povidone-iodine or chlorhexidine solution and drape the abscess with sterile towels. Perform a field block by infiltrating local anesthetic around and under the tissue surrounding abscess. - Make a linear incision with a no. 11 or 15 blade into the abscess. - Allow purulent material from the abscess to drain. Gently probe the abscess with the curved hemostats to break up loculations. Attempt to manually express purulent material from the abscess. - Insert packing material into the abscess with hemostats or forceps. Dress the wound with sterile gauze and tape.

Rapid recognition and treatment is critical in the care of a patient with bacterial meningitis. Ongoing serial examinations and monitoring is crucial for rapid detection and treatment of complications. Common acute complications of bacterial meningitis include

increased intracranial pressure hydrocephalus seizures stroke hemorrhage subdural empyemas

2 yo with hx of recurrent sinusitis and pna is hospitalized for evaluation of prolonged fever and cough associated with dehydration. He is subsequently found to have pna caused by PCP. There is not hx of eczema or easy bruising. Most likely to be identified during additional evaluation?

increased levels of serum IgM - hyperIgM syndrome - caused by mutations in gene that encodes for CD40 ligand normally expressed on activated T-helper cells --> highly susceptible to PCP

Initial symptoms of tylenol toxicity develop within 6-24 hours and include anorexia, nausea, vomiting, malaise, diophoresis. Second stage develops within 24-72 hours and is characterized by ...

increased liver enzymes liver enlargement RUQ pain

hypertrophic cardiomyopathy murmur when standing ...

increases

The differential diagnosis of bloody stool includes

infectious causes (eg, Salmonella, Shigella, Campylobacter, and Yersinia species; Clostridioides difficile (formerly known as Clostridium difficile, Escherichia coli 0157:H7, and Entamoeba histolytica) inflammatory bowel disease (IBD) vascular abnormalities Meckel diverticulum colonic polyps hemorrhoids/fissures

17 yo boy is transported to ED after found in bathroom at high school screaming and terrified, claiming the door was too small for him to exit. During transport, he said ambulance attendants look like midgets, his hands were shrinking, and the sun was red and on fire. Urine drug screen negative. What disorder may include perceptual distortions as presenting feature?

infectious mononucleosis - "Alice in Wonderland" syndrome

A previously healthy 17-year-old girl is at the office for a routine health supervision evaluation. She has not had a medical visit in 2 years. Her mother is concerned that her daughter has not had a period in 4 months and has been losing weight. The girl has eaten a vegetarian diet for the past 6 months, and is very active, participating on both her school and club soccer teams. In private, the girl discloses that she intentionally started losing weight because one of her soccer coaches told her it would improve her performance. She has been limiting her intake to 1,000 calories per day, and exercises 2 to 3 hours per day with soccer practice. She is aware that she has become thin, but would like to lose 5 more pounds. She denies the use of diet pills, laxatives, diuretics, or supplements. She also denies the use of tobacco, illicit drugs, or alcohol. She has not had any suicidal thoughts or attempts. She has never been sexually active. The girl has lost 35 pounds since her last office visit. On physical examination, her heart rate is 42 beats/min and blood pressure is 88/50 mm Hg. There is palpable stool in the left lower quadrant, and acrocyanosis of her hands and feet. It is recommended that her mother take her to the emergency department for evaluation and admission because of the concern for an eating disorder. She is told that the hospitalization may last for several weeks while the girl is medically stabilized and demonstrates some weight gain. Upon hearing this suggestion, the girl becomes defiant, stating that she is fine and refusing admission to the hospital, because she has an important soccer tournament coming up that she does not want to miss. Of the following, the BEST next management step for this patient is to

inform the mother that she can consent to having her daughter admitted for care because the girl is a minor - The best next management step for the adolescent girl in the vignette would be to inform her mother that she can consent to having the daughter admitted for care because the girl is a minor. - The girl's signs and symptoms suggest that she is becoming medically compromised because of malnutrition from an eating disorder. Because she is intentionally trying to lose weight, restricting her intake, and does not feel she has a problem, it is unlikely that she will follow suggestions to improve her nutrition/health on her own. - Because this girl is not yet 18 years of age, and is not an emancipated minor, her mother can still make medical decisions for her and consent to have her daughter admitted for care.

A 16-year-old adolescent girl has a 2-week history of polyuria, polydipsia, fatigue, and a 5-lb weight loss. Her medical history is not significant. Both her mother and maternal grandmother have type 2 diabetes. Physical examination reveals a temperature of 36.7°C, blood pressure of 136/90 mm Hg, heart rate of 102 beats/min, and respiratory rate of 18 breaths/min. Her weight is 101 kg (>97%) and body mass index is 35 kg/m2 (>97%, 120% of the 95th percentile). She has acanthosis nigricans around her neck and mild hirsutism over her upper lip, chin, and lower abdomen. The remainder of her physical examination findings are unremarkable. Laboratory evaluation shows the following: - Glucose 320 mg/dL (18 mmol/L) - Bicarbonate 28 mg/dL (928 mmol/L) - Hemoglobin A1C 10.2% - Urinalysis Large glucose, negative ketones Of the following, the BEST next step in management for this patient's condition is

insulin subQ - The American Diabetes Association recommends that insulin should be initiated at diagnosis for youth with type 2 diabetes if the blood glucose is greater than or equal to 250 mg/dL (13.9 mmol/L) or hemoglobin A1c is greater than or equal to 8.5%. - Other indications for insulin therapy include ketosis, and if the distinction between type 1 and type 2 diabetes is not clear. - Once metabolic control is established, metformin may be added to her treatment regimen and the insulin dose lowered. The patient in the vignette meets the following diagnostic criteria for diabetes mellitus: - a random plasma glucose ≥200 mg/dL (11.1 mmol/L) with classic symptoms of hyperglycemia (polyuria, polydipsia) - hemoglobin A1c ≥6.5% - Other diagnostic criteria for DM include a fasting plasma glucose greater than or equal to 126 mg/dL (7 mmol/L) or a 2-hour plasma glucose greater than or equal to 200 mg/dL (11.1 mmol/L) during a 75-g oral glucose tolerance test.

A 4-year-old, previously healthy boy who is fully immunized (including with the bacillus Calmette-Guérin vaccine) is seen for a routine health supervision visit. He was born in India and emigrated to the United States at 1 year of age. His mother states that the boy's grandfather, who is visiting from India, was recently diagnosed with reactivation tuberculosis. The grandfather arrived 2 months ago and has since had a cough and weight loss. The boy has not had fever or cough and has been gaining weight appropriately. His physical examination findings are normal. Anteroposterior and lateral chest radiographs obtained on the day of the visit are normal. Of the following, the BEST next management step for this boy is to order a/an

interferon-γ release assay, and start isoniazid immediately - Because the boy has received the bacillus Calmette-Guérin (BCG) vaccine and his age is younger than 5 years, the best next step in management is to order an interferon-γ release assay (IGRA) and start isoniazid immediately. - Receiving the BCG vaccination soon after birth can result in a false-positive TST up to 10 years of age, with reactivity of less than 10 mm. The IGRA result is not affected by BCG vaccination. - Children younger than 5 years and those who are immunocompromised are at high risk of progression from tuberculosis infection to disease. Children who are at high risk, when exposed to tuberculosis, should receive prophylaxis, even if the result of the initial tuberculosis test (TST or IGRA) is negative. A repeat TST or IGRA should be performed 8 to 10 weeks after the last exposure to the infected person. If the result of the repeat test is negative, prophylaxis can be discontinued. - Options for prophylaxis include isoniazid for 9 months, rifampin for 4 months, or isoniazid-rifapentine weekly treatment for 3 months. - Interferon-γ release assays to diagnose tuberculosis can be used in children as young as 2 years with a history of bacillus Calmette-Guérin vaccination.

Treatment options for nasal polyps include

intranasal corticosteroids - such as fluticasone nasal spray nasal saline rinses systemic corticosteroids - If symptoms persist despite medical treatment, polyps can be surgically removed

metatarsus adductus is caused by

intrauterine crowding - typically resolves spontaneously within 1 year of life

Child has a new, symptomatic complete heart block. Additionally, he reports recent travel to New England. While not diagnostic, this raises suspicion for Lyme carditis. He should be admitted to the hospital for cardiac monitoring, given his symptomatic cardiac condition. While undergoing evaluation for the cause of his heart block, the boy should be started on

intravenous (IV) therapy for possible Lyme disease --> Red Book® recommends IV ceftriaxone for those admitted to the hospital - Child has complete heart block = no electrical connection between the P waves and the QRS complexes + rhythm is too slow

ketosis without acidosis is characteristic of what kind of poisoning?

isopropanolol poisoning - CNS depression, inebriation

A 4-year-old boy is seen in the clinic for evaluation of cough and eye redness. He has had nasal congestion, mild cough, and bilateral itchy, red eyes for the past week. He has been afebrile and otherwise well, with a normal appetite and energy. He has normal vital signs. He has mild bilateral periorbital edema and erythema. His bulbar and palpebral conjunctiva are erythematous but without discharge. His tympanic membranes are normal, and he has normal findings from cardiac and respiratory examinations. Of the following, the MOST appropriate treatment for the boy's ocular symptoms is

ketotifen eye drops - allergic conjunctivitis - ketotifen eye drops = H-1 antihistamine and mast cell stabilizer - presence of pruritus and the absence of physical examination findings consistent with upper respiratory tract infection make allergic conjunctivitis more likely - in addition to removal of the allergen, treatment for allergic conjunctivitis can be with supportive measures (eg, eye lubricant, cool compresses), a short course of vasoconstrictor/antihistamine combination drops (eg, naphazoline and pheniramine), antihistamine drops (eg, olopatadine, ketotifen), or mast cell-stabilizing drops (eg, cromolyn sodium)

A known seizure patient comes in for follow up after being started on a new anticonvulsant by her neurologist for a poorly controlled complex partial seizure disorder. She was initially on carbamazepine and topiramate was started when her seizures did not decrease. The seizures have slowed considerably. In your evaluation for known primary side effects, which would most likely be expected with the use of this new anticonvulsant?

kidney stones - Topiramate is one of the newer antiepileptic drugs used to treat complex partial seizures (CPS). - Its primary adverse effects include cognitive impairment, weight loss, and kidney stones.

Delayed puberty is defined as

lack of breast development in girls by age 13 years lack of testicular growth to 4 mL in volume (2.5 cm in length) by age 14 years in boys - Delayed puberty in girls can also manifest as primary amenorrhea, the lack of menses by age 15 years

The first sign of intellectual disability in a young child can be delay in

language development, particularly receptive language, as this correlates with cognitive function

What fish should pregnant ppl avoid?

large fish = king mackerel, tilefish, shark, swordfish - accumulate mercury by eating smaller fish

Teenage boy who spends hours each week playing tennis is most likely to suffer from ...

lateral epicondylitis / tennis elbow

A 16-year-old girl presents with right knee pain. She came to a sudden stop while her right foot was planted playing basketball. She has previously had occasional pain in both knees but never to the current extent. On exam, she can bear moderate weight on her right leg. There is minimal effusion around her anterior knee, with mild tenderness over the anterior tibial tubercle area. She cannot completely extend the knee. What diagnostic test would be most helpful?

lateral x-rays - possible type I tibial tubercle fracture versus Osgood-Schlatter disease (OSD), which is more chronic and associated with less severe symptoms due to forces exerted on the tibial tubercle by the patellar tendon in rapidly growing adolescents during sports that involve jumping and squatting - tibial tubercle fractures type II and III can easily be diagnosed with plain lateral radiographs of the knee and show partial elevation of the tubercle - lateral knee radiographs would also show soft-tissue swelling and fragmentation of the tubercle in OSD, which is self-limiting with conservative treatment

Children with unexplained persistent fever, severe persistent pain (often of the back or extremities), pallor, fatigue, petechiae, or bruising, should be evaluated for

leukemia - Pathologic fractures may warrant investigation for leukemia. - Children with trisomy 21, Klinefelter syndrome, Fanconi anemia, and Bloom syndrome have an increased risk of developing leukemia.

Transient neonatal pustular melanosis is a common neonatal rash seen predominantly in African-American infants. It is characterized by superficial vesicles that rupture, leaving a collarette of scale, and they develop a hyperpigmented macule. The condition may persist for a few months; it is benign and requires no treatment. Smears of the lesion reveal numerous

leukocytes and debris

21 mo old girl referred to ophthalmologist for evaluation of nystagmus, first noticed by PCP several days prior to presentation. On PE, periods of intermittent, rapid pendular nystagmus noted in associated with head nodding and torticollis. Pt appears alert both during and after episodes of nystagmus. Normal brain MRI and sleep-deprived EEG. Prognosis?

likely resolution of all symptoms within mos to years - spasmus nutans

A 5-year-old child presents with a 6-day history of high fever with chills, cough, difficulty in breathing, and pain over the right side of the chest. Physical examination shows temperature of 104°F. Toxemia is present. Pulse 112/min, regular good in volume. BP 100/70 mmHg. Trachea is central. Chest movements during respiration are diminished on the right side. Percussion note is impaired over the right upper and mid zones. Bronchial breathing heard over the right upper and mid zone. Vocal fremitus and vocal resonance are increased over the same area. X-ray chest has confirmed the diagnosis of consolidation of the right upper and middle lobes. Child has been treated initially with ampicillin and later with ceftriaxone without a satisfactory response. Blood culture has grown multidrug-resistant pneumococci. What is the antibiotic of choice for the treatment of this child?

linezolid - Oxazolodinone - drug of choice and is reserved for treatment of invasive pneumococcal disease due to multidrug-resistant isolate - available in both oral and parenteral formulations - licensed for use for highly resistant Gram-positive infections and approved for use in children - indicated for penicillin-resistant peumococci, MRSA, coagulase-negative staphylococci, and vancomycin-resistant enterococci (VRE). - can cause anemia and thrombocytopenia and is a monoamine oxidase inhibitor

2 yo M + abnormality in left leg + region of moderate subcutaneous atrophy of lateral side of left thigh + appears as if underlying adipose tissue has been "scooped out" + overlying skin without erythema or swelling + hyperpigmentation at lesion site. Most like cause?

localized scleroderma - subcutaneous morphea - autoimmune attack - UEs and LEs mostly

Adolescent has polyarthritis, carditis, and a red rash with central clearing after a recent sore throat. She has a new murmur that is consistent with aortic regurgitation. Her history and physical examination findings are consistent with acute rheumatic fever (ARF) for which she should receive

long-term treatment with intramuscular penicillin G monthly - Typically, monthly injections of penicillin G benzathine are required for 5 to 10 years depending on the presence or absence of carditis and residual cardiac disease - The revised Jones Criteria lists the major and minor criteria required for the diagnosis of acute rheumatic fever from populations at low or moderate to high risk. Diagnosis is made with either 2 major, 1 major and 2 minor, or 3 minor criteria. - Evidence of prior group A Streptococcus infection is required for diagnosis of acute rheumatic fever, unless chorea is present. Upon presentation, a child with suspected ARF should undergo laboratory evaluation including: - Rapid strep antigen - Throat culture - Anti-DNAse B titer - Antistreptolysin O titer - Inflammatory markers (erythrocyte sedimentation rate and/or C-reactive protein level) - Echocardiography - Electrocardiography (to assess PR and other conduction intervals)

Boy has acute poststreptococcal glomerulonephritis (PSGN). His prior history of a skin infection, gross hematuria, hypertension, azotemia, edema, and proteinuria favor this diagnosis. What lab findings support the diagnosis of PSGN?

low complement C3 normal C4 - Poststreptococcal glomerulonephritis is the most common childhood glomerulonephritis, typically presenting between 4 and 12 years of age. - The symptoms and signs of acute glomerulonephritis (hematuria, hypertension, azotemia, and edema) usually present 1 to 2 weeks after a streptococcal sore throat or 2 to 6 weeks after a streptococcal skin infection. - Children with PSGN may have minimal microscopic hematuria or a rapidly progressive glomerulonephritis. - Severe acute-onset hypertension leading to headache, visual disturbances, and encephalopathy can sometimes be seen. - Salt and water retention in PSGN leads to generalized edema, and in severe cases, can cause pulmonary edema and congestive heart failure.

Thyroxine levels in celiac disease

low free thyroxine levels - tTG antibodies found in celiac disease may contribute to thyroid dysfunction by binding to thyroid cells. - If you have celiac disease along with a mild form of hypothyroidism called subclinical hypothyroidism, following a strict gluten-free diet for a year may bring your thyroid hormone levels back to normal

Lab findings of CVID ...

low serum IgA, IgM, IgG

In prepubertal male with Fragile X, more prominent signs are large HC, hypotonia, and dev'tal delays in speech and motor skills. Motor delays are d/t combo of

low tone and joint hypermobility

6 yo F + frequent wheezing + last 6 mos wakes up at night 3 times per mo with wheezing and coughing + mild limitations with exertion and uses albuterol 1x/week during day. Next step?

low-dose steroid = mild persistent asthma

A 3.5-month-old male patient presents with a 2-day history of elevated temperature, maximum 103.8°F. He was irritable and screaming with a highly pitched cry for the first 36 hours, but he has become more lethargic over the past 12 hours, and he has been unable to feed. Past history is unremarkable. Immunizations are current. There has been no recent contact with anyone ill. He appears acutely ill; pulse 144 bpm, temperature 103.5°F, unremarkable blood pressure and respiration. The anterior fontanelle is bulging. There are no focal neurologic signs. What is the most important next step in the management of this patient?

lumbar puncture - irritability, screaming, lethargy, poor feeding, and a seizure; it is critical to rule out meningitis, even in the absence of meningeal signs (Kernig, Brudzinski, nuchal rigidity) - lumbar puncture is the next and most important step in managing this patient. Cranial CT is advised in a select group of patients before lumbar puncture: those who present in coma, with papilledema or focal neurologic signs, and those with a history of recent cranial surgery or head trauma. A CT before lumbar puncture is not necessary in this patient. - At this age, the most likely etiologic agent of bacterial meningitis is Streptococcus pneumoniae. Haemophilus influenzae type B is much less frequently seen since mass immunization, but it is still of concern in the unimmunized.

Patients with SMA syndrome often describe relief in what positions?

lying prone or in left lateral decubitus or knee-chest positions

Prophylaxis to consider when trying to protect newborn from pertussis ...

macrolide abx, such as azithromycin

When FAI or a labral tear is suspected, this imaging is preferred for better delineation of cartilage anatomy and possible tear, and would be the best choice for this patient if initial radiography is negative ...

magnetic resonance arthrography - Groin pain suggests interarticular pathology and warrants radiographic evaluation before considering treatment options. - The acetabular labrum is not well-visualized with magnetic resonance imaging and requires arthrography to rule out possible tears. - Cam and pincer impingement and borderline hip dysplasia may lead to early osteoarthritis of the hip.

A 14-year-old female cross-country runner is seen for evaluation of ear pain after several weeks of upper respiratory symptoms. She had a fever yesterday, but has remained afebrile today after taking acetaminophen early this morning. Her vital signs are normal. She has a red and bulging tympanic membrane and shotty anterior cervical lymphadenopathy. The remainder of her physical examination findings are normal. Amoxicillin is prescribed. She has a race this afternoon and would like to compete. Her mother inquires about when she can return to sports. Of the following, the BEST advice regarding return to sports is that she

may return as tolerated and limited only by clinical symptoms - acute otitis media, and she may return to physical activity as tolerated - "neck rule" is commonly invoked in these decisions, where patients with conditions above the neck (eg, otitis media, sinusitis, nonspecific upper respiratory tract infections [URIs]) may participate in sport as tolerated. Athletes with conditions that are below the neck (eg, pneumonia, vomiting) or systemic (eg, myalgia, malaise, fever) should be withheld from sport until the symptoms and underlying etiology have resolved

Airborne precautions are used to prevent transmission of organisms that spread by droplet nuclei (≤5 μm in diameter) that can remain suspended in air for long periods, allowing the organism to be transmitted over distances greater than 1 m. Infections caused by common microorganisms that require airborne precautions are

measles varicella Mycobacterium tuberculosis (TB) - Patients should be placed in a negative air pressure room with 6 to 12 air changes per hour (air exhausted to outside); if air is recirculated, it must be processed using high-efficiency particulate air (HEPA) filtration - For patients with suspected (or proven) contagious TB, N95 masks (fit-tested) are recommended for health care providers before entry into the patient room

Up to 1/3 of c diff diarrhea relapses, regardless of therapy used. For recurrence, repeat therapy with what is indicated?

metronidazole - should NOT be used past the 1st recurrence however because of neurotoxic effects from drug accumulation - resistance rates are low - oral vanc is another option

The recommended first-line treatment regimen for trichomoniasis includes

metronidazole 2 g orally or tinidazole 2 g orally as a single dose - The alternative regimen is metronidazole 500 mg orally twice daily for 7 days, if the initial treatment was not effective and there is no reinfection. - It is important that partners be treated to prevent reinfection.

While doing newborn nursery rounds, you are called about a male neonate born 36 hours ago to a mother with diabetes. The nurse is concerned that he has not yet passed any meconium. The baby was born at 36 weeks gestation and had a normal spontaneous vaginal delivery with Apgars of 9 and 9 at 1 and 5 minutes. His mother developed mild toxemia before delivery and was given magnesium sulfate. On exam, you note some mild abdominal distention and the presence of normal bowel sounds. The rest of the exam is normal. Plain abdominal films reveal multiple dilated loops of bowel with absence of rectal gas. A rectosigmoid biopsy is reported normal. What is the most likely diagnosis in this neonate?

meconium plug syndrome - functional immaturity of the colon, specifically the myenteric plexus nerve cells - transient disorder of the newborn colon - intestinal dilatation is characteristic, seen as abdominal distension with delayed passage of meconium for 24-48 hours - bilious vomiting may occur - incidence is increased in premature newborns of mothers with diabetes whose mothers received magnesium sulfate for toxemia - plain films of the abdomen usually reveal multiple dilated loops of bowel with an absence of rectal gas

There is no cure for lichen sclerosis, but it often involutes at or before puberty. First-line treatment is usually

medium- to high-potency topical steroid - Many clinicians initiate therapy with an ultrapotent (group 1) agent (eg, clobetasol propionate or betamethasone dipropionate), tapering the frequency of application or potency as the condition improves - Once control is achieved, the topical steroid can be withdrawn, and maintenance therapy is initiated with a topical calcineurin inhibitor (eg, tacrolimus or pimecrolimus) - Lichen sclerosis is primarily a disease of adult women; 5% to 15% of cases occur in children, almost exclusively girls younger than 7 years of age. - Typical lichen sclerosis lesions are shiny, wrinkled, ivory-colored atrophic patches located in a figure-of-8 or hourglass distribution surrounding the vulva, perineum, and anus. Blisters may occur.

Recognize mucosal neuromas, in which pt most likely has MEN2B and would need to be screened for...

medullary thyroid carcinoma - Evaluation for MTC includes thyroid ultrasonography and a serum calcitonin level test. Calcitonin is produced by the neuroendocrine C cells of the thyroid and is elevated in MTC. - Phenotypic features of multiple endocrine neoplasia type 2B include mucosal neuromas; a tall, thin body habitus; full lips; joint laxity; and alacrima. - Gastrointestinal tract ganglioneuromas may cause intermittent constipation and diarrhea. - Multiple endocrine neoplasia type 2B is associated with early-onset and aggressive medullary thyroid carcinoma, in addition to pheochromocytoma and the physical phenotype. - It is important to recognize the clinical features of multiple endocrine neoplasia type 2B for timely prevention (prophylactic thyroidectomy), detection, and treatment of the associated medullary thyroid carcinoma.

A 3-week-old neonate born at 35 weeks' gestation is admitted to the hospital with a 2-day history of fever and decreased oral intake. In the emergency department, his vital signs reveal a temperature of 39°C, heart rate of 150 beats/min, blood pressure of 52/36 mm Hg, and oxygen saturation of 92% in room air. He appears lethargic and has a capillary refill time lasting 2 to 3 seconds. The remainder of the neonate's physical examination findings are unremarkable. Treatment is initiated with intravenous cefepime and acyclovir. Within 6 hours, his blood culture is reported as positive for Streptococcus agalactiae. Of the following, the MOST likely diagnosis for this neonate is

meningitis - The neonate in the vignette has meningitis caused by late-onset group B Streptococcus (GBS) infection (Streptococcus agalactiae). Late-onset GBS infection presents after the first week of life. The neonate's physical examination findings support the diagnosis of meningitis. - Suspected late-onset GBS should be treated empirically with ampicillin (or cefotaxime) and an aminoglycoside. Antibiotic coverage should be narrowed to penicillin G once GBS infection is confirmed. - Bacteremia should be treated for 10 days. Uncomplicated meningitis should be treated for a minimum of 14 days. Soft tissue infection, osteomyelitis, and septic arthritis may require treatment for 3 to 4 weeks. - Group B Streptococcus meningitis is associated with long-term complications such as cerebral palsy, cortical blindness, and learning disabilities.

The pregnant mother of a 2-year-old patient is concerned about exposure to environmental neurotoxins affecting her developing fetus. She asks you questions about whether the placenta would block any transfer of toxins to a growing fetus. Which environmental neurotoxin transfer to the fetal system is actually enhanced by the placenta?

mercury - Methyl mercury is absorbed almost completely and crosses the placenta easily and then becomes stored in the fetus. - Fetal blood concentrations exceed maternal concentrations by 50-100%. - High doses can result in intellectual disability, spastic paralysis, and death. - Low doses are linked to deficits in neuromotor performance, cognition, memory, and language.

A 4-month-old female infant presents with a mild cough, runny nose, and 'tactile' fever. Her mom also tells you that everyone at home has been sick with a cold. The infant has been feeding well and is still nursing. She appears alert and active; her vital signs are normal. Ears, nose and lungs are clear with mild, clear rhinorrhea. However, while examining her you notice a peculiar keel-shape deformity of her central forehead. When you point this out to mom, she says she has noticed this too, but her husband has a very prominent forehead and the baby likes to sleep on her side a lot. On further exam, you note a light palpable ridge over the suture line and check her head circumference. It has moved from the 25th percentile on her 2-month check up to the 75th percentile. You order a CT scan to evaluate this. Based on these findings, what suture(s) would most likely be affected?

metopic - Synostosis of the metopic sutures causes trigonocephaly, resulting in a keel-shape deformity of the central forehead. Trigonocephaly is usually mild and requires no treatment. - In other cases, once a CT scan is done and diagnosis confirmed, surgery is done if it is severe and causing symptoms or complications. Hydrocephalus, neural tube defects, syringomyelia, and brain tumors are to be ruled out.

The most common manifestations of congenital rubella syndrome include

microcephaly cataracts sensorineural deafness peripheral pulmonic stenosis radiolucent bone lesions

25 day old F infant noted to have hyperdynamic precordium and bounding pulses. Loud, single 2nd heart sound, systolic ejection click, and systolic ejection murmur along left sternal border are present. On CXR, thymic shadow is absent; biventricular cardiac enlargement and prominent pulmonary vasculature are noted. Chromosomal analysis most likely to reveal ...

microdeletion - truncus arteriosus - DiGeorge

A 4-day-old male infant is seen by the pediatric resident in the newborn nursery for bilious vomiting and abdominal distention. The child had been feeding poorly and has been difficult to console according to the mother. He passed meconium shortly after birth and has passed several seedy, yellow stools per day since then. Physical examination reveals abdominal distention. What is the most likely explanation for these findings?

midgut volvulus - Bilious vomiting is caused by intestinal obstruction distal to the ampulla of Vater. - Malrotation, in which the intestine fails to rotate 270 degrees during fetal development, results in malpositioning, obstruction, and volvulus. Ischemia and infarction occurs as the small intestine twists around the superior mesenteric artery. - Although it can appear at any age, volvulus from malrotation typically presents during infancy. - Initially, the child who has malrotation may appear and feed well, but he or she then develops fullness in the right upper quadrant and bilious vomiting. - Abdominal distention, as reported for the infant in this case, is a variable finding among those who have a midgut volvulus. - Abdominal tenderness, when present, suggests advanced ischemia and necrosis. In infants who have bowel ischemia, rectal bleeding and shock may develop secondary to perforation. - The radiologic findings of intestinal obstruction due to volvulus include gastric and proximal duodenal dilatation, paucity of intestinal air, and corkscrew-like appearance of the duodenum.

In infants and toddlers, consider abuse with what type of fractures?

midshaft femur and humerus fractures posterior medial rib fractures complex skull fractures corner fracture, bucket handle fracture

Naegleria fowleri tx ...

miltefosine + medically induced hypothermia to allow medication time to take effect

Drug-induced state in which a pt can respond normally to verbal commands

minimal sedation

Tx goal of exchange transfusion for infant with polycythemia ...

minimize risk of hyperviscosity

Cardiac symptoms are common in adolescents with anorexia nervosa and include auscultatory findings c/w ...

mitral valve prolapse = apical systolic murmur with midsystolic apical click - via myofibrillar atrophy

Salicylism typically causes what kind of acid-base disorder?

mixed acid-base disorder with a metabolic acidosis with an elevated anion gap and a respiratory alkalosis - However, younger children are often not as hyperpneic and will thus only present with a metabolic acidosis and not the respiratory component.

A mother presents with her full-term female neonate and reports difficulty with breastfeeding at 24 hours of life. There is no significant perinatal history. After her birth, she was noted to have complete adduction of both eyes. No facial expressions appear on crying, and her eyes do not close fully during sleep, according to her mother. She is noted to have normal tone and actively moves all four limbs, with normal gag reflex and no dysmorphic features. Her Moro's reflex is symmetric, and deep tendon reflexes elicit a 2+ response with an equivocal Babinski response. What is the most likely cause of this neonate's features?

moebius syndrome - characterized by bilateral facial and abducens nerve paralysis - other cranial nerves (e.g., the hypoglossal) may also be involved, and the condition occurs with equal incidence in males and females - most cases are sporadic, but familial forms have also been reported. This syndrome is believed to be caused by a defect in the development of the rhombencephalon or an interruption in its vascular supply during intra-uterine development. - Deviations of the eye toward the nose are called esodeviations and represent the most common type of strabismus in children

A 14-year-old adolescent boy is seen for a health supervision visit. His growth and development have been normal, and he plays basketball for his high school team. His mother reports that she would like him to use a mouthguard during basketball, but he is very resistant. His team does not require mouthguards during practice or play. He has no history of dental or facial injury, and no dental-related concerns. She is concerned about the potential cost. Of the following, the BEST advice for this patient and his mother is that

moldable "boil and bite" mouthguards can effectively prevent most dental injuries in sports - Dental injuries are common in high school basketball players, and mouthguards can reduce risk by 80% to 90%. Therefore the athlete and his mother in this vignette should be counseled on appropriate mouthguard use. - For young athletes without orthodontics or significant malocclusions, a moldable "boil and bite" mouthguard is a cost-effective way to prevent dental injury and is the best recommendation for this patient. - Boil and bite mouthguards will thin with use and should generally be replaced at the end of each competitive season.

Underlying cause of guillain-barre syndrome

molecular mimicry - b/t infectious agent and pt's Schwann cells

12 yo M with numbness and tingling of hands. PE = large orange-colored tonsils, hepatosplenomegaly, decreased sensation of UEs. This is caused by ...

mutation in adenosine triphosphate cassette transporter protein - tangier disease - AR - familial neuropathy - HDL deficiency - low serum cholesetrol - high triglycerides

A 16-year-old football player is seen for evaluation after 2 days of right arm numbness. He sustained a hit to the left side of his neck by an opponent's helmet. He noted immediate numbness and tingling down his right arm and into his thumb. He was removed from the game, and his parents took him to a local emergency department the following morning. Cervical spine radiographs (anteroposterior and lateral) were normal, and he was advised to see his pediatrician for any persistent symptoms. His neck pain has resolved, and the weakness and numbness are improving gradually. He is otherwise healthy and reports no symptoms in any other extremity. His physical examination reveals free range of motion of the neck, which does not reproduce his symptoms. He has weakness with resisted shoulder abduction and elbow flexion on the right, but otherwise he is neurovascularly intact in all 4 extremities. Of the following, the MOST appropriate next step in his management is

monitoring for symptom resolution - The football player in this vignette has sustained a "burner" or "stinger." These are usually neuropraxic injuries to the upper trunk of the brachial plexus, and their natural history typically results in complete and spontaneous resolution. For the adolescent in this vignette, expectant observation is the best option early in the course of injury. - Burners may occur with traction or compression of the lower cervical spine and are most common during tackling in American football. Pain is often immediate and radiates circumferentially down the arm. Unilateral numbness or weakness may also develop. - An injured player will often come off the field actively shaking the involved arm. - Most burners will resolve within several minutes, but some may take months to fully recover. - Permanent neurologic damage is rare. - Evaluation for a suspected burner should aim to rule out injury of the cervical spine. Significant pain with active neck motion or midline tenderness over the cervical spine should prompt further evaluation, and bilateral upper extremity or any lower extremity involvement must be considered a cervical spine injury until proven otherwise. - Patients may return to contact sports when they demonstrate full and pain-free range of motion of the neck and shoulder, with full strength and normal neurologic examination findings throughout the upper extremity. - Symptoms that persist for greater than several weeks suggest more severe axonal injury or possible nerve root avulsion. - Patients with persistent symptoms should be referred for additional evaluation. - Nerve conduction testing is often performed if symptoms persist beyond 3 to 4 weeks. - Physical therapy may be helpful for restoration of full strength and range of motion with prolonged symptoms, but it is not necessary in cases with rapid improvement.

For pinworms, when should a piece of clear tape be placed over the pt's perianal area?

morning - because eggs are laid at night

5 yo M + nocturnal enuresis + rarely has a dry night + frustrated bc he is toilet-trained during the day. Dad had intermittent nocturnal enuresis until 8 yo. Vitals, PE, and UA nml. Most appropriate next step in management?

motivatonal therapy - primary monosymptomatic nocturnal enuresis - bathroom before going to sleep - restrict fluid intake in evening

Potential side effects of isotretinoin

mucocutaneous side effects photosensitivity arthralgias myalgias bone marrow suppression intracranial HT hepatotoxicity - requires monitoring: serum triglycerides, cholesterol, liver function tests, CBC prior to initiating therapy and various intervals after - highly teratogenic = needs documentation between physician and patient that risk associated with pregnancy have been reviewed Females must - use 2 methods of birth control or abstain from sexual intercourse for 1 mo prior to therapy, throughout course of tx, and for 1 mo upon discontinuing tx - 2 negative tests required to initiated therapy - monthly pregnancy tests during duration of therapy and once upon completion of therapy

A 2-week-old infant presents with history of poor feeding with vomiting off and on and lethargy from 2nd day of life. There is also a history of severe episode of generalized tonic and clonic convulsions. Infant has also developed a generalized erythematous rash with exfoliation for the last 1 week. Baby was normal at birth with a birth weight 2.7kg and the baby cried immediately after birth. Blood examination shows hyperammonemia and metabolic acidosis. What is the most likely clinical diagnosis?

multiple carboxylase deficiency - infant has presented with typical features of feeding problems, lethargy, vomiting, and seizures starting on 2nd day of life though he was normal at birth - classical erythematous maculopapular rash with exfoliation is highly suggestive of multiple carboxylase deficiency - presence of hyperammonemia and lactic acidosis support the above diagnosis. - involves a defect in utilization of biotin which is a water soluble vitamin of B complex group. It acts as a cofactor for all 4 carboxylase enzymes, which are important in the metabolic pathways of amino acids leucine. - rare autosomal recessive disorder - if the condition remains untreated the infant may develop an erythematous maculopapular skin rash with exfoliation and alopecia which may be partial or total. Later on infant may develop intractable refractory seizures which do not respond to the conventional anticonvulsant therapy. - Developmental delay and failure to thrive are common. Immunodeficiency manifests as increased susceptibility to infections. - Laboratory findings show metabolic acidosis, hyperammonemia, and presence of other organic acids. - Treatment with biotin 10mgm/day orally may result in clinical and biochemical improvement. Early treatment is important to prevent irreversible neurological damage. Sometimes large doses up to 80 mgm/day are required for complete resolution.

Can occur when a researcher fails to state a hypothesis before conducting a study and uses many characteristics to make a variety of comparisons ...

multiple comparisons problem

3 yo boy presents to your office with hx of recurrent infections and greasy, foul-smelling stools. On exam, his weight and height are both below 3rd %ile. CBC shows leukocyte count of 1,100/uL and absolute neutrophil count of 100/uL. Underlying pathophysiology that explains his disorder?

mutation in SBDS gene Shwachmann-Diamond syndrome - recurrent infections, pancreatic exocrine insufficiency, neutropenia

During a 2-week-old female infant's exam, you notice a mild ptosis of the left eye. She was born full term by normal spontaneous vaginal delivery without any significant prenatal or perinatal problems. Her birth weight was 7 lbs. 3 oz., and her length was 19 inches. She was discharged after 48 hours and was reportedly nursing well, and she had a normal newborn exam before going home. The mother reports no injuries or problems. She has continued to nurse well and is able to focus on family members. What mechanism of congenital ptosis occurs most commonly?

myogenic - Fifty percent of patients with ptosis (the incomplete elevation of the eyelid) are children. - The most common mechanism causing congenital ptosis is myogenic, due to a striated muscle fiber defect of the LPS muscle.

Tx for acute mastoiditis ...

myringotomy + drainage of subperiosteal abscess + linezolid/IV abx

After several weeks, plain radiographs of discitis may show ...

narrowing of intervertebral disc space - elevated ESR, CRP

Can be performed to detect structural abnormalities of the inner dynein arms, outer dynein arms, radial spokes, microtubular transposition, and other abnormalities.

nasal mucosal biopsy - for electron microscopic examination of the cilia

A 6-week-old infant girl is seen in the office for evaluation of a 4-day history of cough, posttussive emesis, and fussiness. The mother reports that the infant became apneic and limp for a brief period after a coughing episode at home. Her grandmother has had a chronic cough for more than 1 month. The infant has a temperature of 37.2°C, a heart rate of 148 beats/min, a respiratory rate of 37 breaths/min, a blood pressure of 82/44 mm Hg, and an oxygen saturation of 96% on room air. Findings of a respiratory examination are notable for bilateral coarse breath sounds. Several episodes of transient apnea are observed. Findings from the rest of the physical examination are unremarkable. Rapid viral antigen testing via direct fluorescent antibody is negative for respiratory syncytial virus. Of the following, the BEST next diagnostic step in the evaluation of this infant's illness is

nasopharyngeal swab for polymerase chain reaction - pertussis (ie, whooping cough) - highly contagious acute respiratory tract infection caused by Bordetella pertussis, a fastidious gram-negative coccobacillus - diagnosis of pertussis may be challenging in infants younger than 6 months - pertussis in young infants can have an atypical presentation during the early stages of illness characterized by gagging, gasping, apnea, or bradycardia without a "whoop" - Nucleic acid amplification tests using polymerase chain reaction assay from nasopharyngeal (NP) wash, aspirate, or Dacron swab is the diagnostic modality of choice for diagnosis of pertussis - Complete blood count may show marked leukocytosis (15,000 to 100,000 cells/µL) owing to an increase in absolute lymphocytes in approximately 75% of unimmunized children - The treatment and postexposure prophylaxis antimicrobial agent of choice for pertussis is a 5-day course of azithromycin, regardless of age. - Trimethoprim-sulfamethoxazole can be used as an alternative agent in patients older than 2 months who are not able to tolerate azithromycin or are infected with a macrolide-resistant strain. - In addition to standard precautions, droplet isolation for at least 5 days after initiation of azithromycin therapy is recommended for hospitalized patients with pertussis.

A 17-year-old soccer player who was diagnosed with a concussion 3 weeks ago is evaluated for clearance to return to sport. She has had full resolution of symptoms and remains asymptomatic with walking and light strength training. She has returned to her previous level of academic achievement in school. This is her third concussion. Her first concussion was at 14 years of age, and her second concussion was 6 months ago. She recovered uneventfully from both of these injuries and is otherwise healthy. She is eager to return to practice and would like to be cleared for sports participation, but her mother is concerned about the risk of another concussion. Her neurologic examination findings are unremarkable. Of the following, the MOST appropriate advice for this patient is that she

needs to perform a trial of high-intensity exercise and report any return of symptoms Medical clearance for return to contact sports requires: - full symptom resolution - return to prior levels of academic performance - no symptom recurrence with physical activity The patient in this vignette needs to remain asymptomatic during a trial of high-intensity activity as the next step back to full participation

Adolescent is otherwise healthy athlete with primary amenorrhea, the absence of menarche by age 15 years. Her low body mass index (7th percentile), low-normal hemoglobin level, and low free thyroxine (FT4) level are all consistent with

negative energy balance - Low FT4 level with a normal thyroid-stimulating hormone level is consistent with euthyroid sick syndrome, a physiologic adaptation to conserve energy

Urine of prerenal AKI is characterized by

negative urinalysis result urine specific gravity greater than 1.020 urine sodium level less than 10 mEq/L FENa less than 1%

An autistic child has major mood swings and shows instability. Since the beginning of treatment with lithium, his social life and school performance have shown improvement. Recently, his parents have noticed an increase in the child's intake of water and frequent urination. On labs, urine osmolality is lower than plasma osmolality. After water deprivation, subsequently administered subcutaneous desmopressin fails to increase urine osmolality. What disorder is most likely present?

nephrogenic DI - Lithium is the most common cause of nephrogenic diabetes insipidus. - This diagnosis is confirmed by the failure of water deprivation and subcutaneous desmopressin, a synthetic analog of vasopressin, to increase urine osmolality. - Thiazide diuretics represent the only drugs effective in treating nephrogenic diabetes insipidus; their method of action is to increase sodium and water reabsorption in the proximal tubule.

A 12-year-old boy presents with a 6-month history of frequent urination. He describes voiding substantial amounts of light-colored to colorless urine every 2 - 3 hours. He awakes at least once per night to void. There has been no increase in appetite or unusual change in weight. He has been drinking increased volumes of water and sometimes awakes at night to drink water. There has been no dysuria. Past history is significant for ablation of posterior urethral valves in infancy. Family history is unremarkable. Growth and development are normal and immunizations are current. Vital signs are normal, as is the remainder of the examination. What is the most likely cause of his polyuria and polydipsia?

nephrogenic diabetes insipidus - Relieving bladder outlet obstruction in the newborn period caused by posterior urethral valves (PUVs) will decrease the increased intravesicular pressure which is transmitted to the upper urinary tract; however, it does not automatically undo the increased deposition of collagen, hypertrophy and hyperplasia of the detrusor, bladder wall thickening, and decreased compliance. - Eventually, bladder decompensation can occur, with detrusor failure and increased bladder capacity. This is due in part to increased urine production due to tubular dysfunction caused by the effects of high back pressure, with decreased ability to concentrate the urine. - This is nephrogenic diabetes insipidus, in which the decreased concentration ability is due to resistance to antidiuretic hormone (ADH). Patients typically note waking at night to drink, and a preference for water over other liquids.

AFP is a marker of ...

neural tube defects affecting spine and/or cranium - screening at 16-20 weeks of gestation

Boys has AOM and bacterial conjunctivitis. Most likely organisms is:

nontypeable h influenza - Since the introduction of the pneumococcal vaccine, Haemophilus influenzae and Moraxella catarrhalis have become the most commonly isolated organisms in children with acute otitis media.

A 2-year-old boy presents with fever as well as a narrow pupil and dropping of the eyelid on the left side. The left eyeball is also depressed, and it shows heterochromia of the iris. Physical exam finds a large palpable tumor on the neck. What is the most likely diagnosis?

neuroblastoma - Horner's syndrome with heterochromia iridis = cervicothoracic neuroblastoma = 2nd most common solid tumor of childhood - Horner's syndrome reflects damage to the sympathetic chain: miosis, ptosis, enophthalmos, and anhidrosis - Weight loss and fever are frequent non-specific symptoms - Neuroblastoma has a slight male predominance while Hodgkin's disease has a female predominance

Most common malignancy diagnosed before 1 yo ...

neuroblastoma - neuroblasts of the postganglionic sympathetic nervous system - nontender abdominal mass - can cause Horner syndrome - paralysis, bowel/bladder dysfunction - poorer prognosis for >1 yo

Methylmalonic acidemia is an AR condition via deficient D-methylmalonyl-CoA mutase. Appearance at birth?

normal - become symptomatic within first week

In pts with celiac disease, compliance with dietary therapy is confirmed by ...

normal tissue transglutaminase (tTG)-IgA levels - gluten --> autoimmune inflammatory response --> production of several autoAbs

An 11-month-old girl is seen by her primary care provider for evaluation after a daycare exposure. Two and a half weeks ago, her room teacher developed fever, nausea, and vomiting, and 1 week later developed jaundice. She was subsequently diagnosed with hepatitis A infection. The teacher has been out of work since the onset of this illness. Yesterday, the daycare program notified families of the possible exposure and recommended medical evaluation. The girl has been eating, voiding, and sleeping well. She has no underlying medical conditions and is scheduled for her 12-month health supervision visit in a few weeks. Her vital signs include a temperature of 37.1°C, heart rate of 110 beats/min, respiratory rate of 28 breaths/min, and blood pressure of 88/46 mm Hg. Physical examination findings reveal a well-appearing child with no jaundice, abdominal tenderness, or hepatomegaly. Of the following, the BEST next management step for this child is to administer

no prophylaxis - hough she is susceptible to infection with hepatitis A virus, she is too young to be vaccinated (<12 months of age), and immune globulin is not effective if given 2 weeks after the exposure. - Exposures in childcare settings are often not recognized until an adult staff member becomes symptomatic. - Because the average incubation period is 28 days, there may be a significant delay between exposure to the index patient and recognition of symptoms in an adult.

A 14-year-old boy has been playing baseball since the season started about 3 weeks ago. He has been playing catcher position for the past 2-3 weeks; over the last 3 days, has begun to develop tenderness, mild redness, and pain with bilateral knee flexion. On exam, there is mild swelling, tenderness, and erythema over bilateral patella; however, it is worse on the right. There is also bilateral pain on knee flexion. What diagnostic test would be most helpful?

no x-ray needed - possible prepatellar bursitis, a common cause of anterior knee pain - fluid-filled bursa, or sac, between the bony patella and overlying skin reduces pressure and friction exerted on the skin overlying the patella - young athletes who spend a lot of time on their knees, such as baseball catchers, develop an irritated and inflamed bursa that can fill with blood, resulting in significant swelling, tenderness, redness, and pain with knee flexion

10 yo girl seen as first-time visit for health supervision visit. No past med hx and is up to date on vaccines. She is active in dance and does well in school. No known heritable diseases in family. Both parents and grandparents are healthy. Physical exam findings are normal. Most appropriate test to obtain for this girl is:

non-fasting lipid profile - American Academy of Pediatrics (AAP) recommends universal screening for dyslipidemia at age 9 to 11 years and again after age 16 years with a lipid profile that can be obtained in the non-fasted state. - In the non-fasted state, a non-HDL cholesterol level can be calculated as follows: non-HDL cholesterol equals total cholesterol minus HDL cholesterol. If the non-HDL cholesterol level is ≥145 mg/dL (3.76 mmol/L), then a fasting lipid profile should be obtained

A 38-month-old boy is brought to clinic in July to establish care. He emigrated from El Salvador 3 months ago. He has no significant medical history, allergies, or medications. He and his mother were detained at the border in a family shelter while awaiting reunification with family members. He underwent basic medical screening, including tuberculosis screening, which had negative results, and received vaccinations. His mother brings his vaccine record from El Salvador, indicating that he received a combination vaccine of hepatitis B, DTaP, and inactivated polio at 2, 4, and 6 months of age. Two months ago at the border shelter, he received Haemophilus influenzae type B, DTaP, MMR, pneumococcal conjugate, and varicella vaccines. In addition to hepatitis A, the most appropriate vaccine(s) for this boy at this time is (are)

none - The 38 month-old boy in the vignette is up-to-date on his vaccines and, with the exception of hepatitis A vaccine, is not currently due for any more. - He was on the catch-up immunization schedule because he was more than 1 month behind on his vaccines when he immigrated to the United States, having only received his 2-month, 4-month, and 6-month vaccines. - At the border family shelter, he received his 12-month vaccines (MMR and varicella) and his 15-month vaccines (DTaP, pneumococcal conjugate, and Haemophilus influenzae type B). - He is also due to receive the influenza vaccine, which usually expires at the end of June and typically is not available until the fall. - Aside from the annual influenza vaccine and the second hepatitis A vaccine in 6 months, he will not be due for additional vaccines (DTaP, inactivated polio vaccine, MMR, varicella) until he turns 4 years old.

A 15-year-old basketball player with hypertrophic cardiomyopathy comes for consultation regarding return to play. Three months ago, he collapsed at a basketball game and was successfully resuscitated. After additional cardiac consultation, an implantable cardioverter defibrillator (ICD) was placed. He has not received any shocks since ICD placement. The cardiologist has informed him and his family that he may consider returning to basketball at this time, as long as they all understand the potential risks. The patient is highly interested in returning to basketball, but his parents are hesitant and wish to learn more about all his options. Of the following, the principle that is MOST applicable to this decision is

nonmaleficence - requires consideration of the potential harms that may accrue to a patient in a given circumstance.

Neonatal findings in pts with Turner syndrome ...

nonpitting edema in LE's loose skin folds at nape of neck skin (webbing) wide-spaced nipples

Juvenile idiopathic arthritis is defined by 6 weeks' or longer duration of chronic arthritis with no known cause occurring before age 16 years. Juvenile idiopathic arthritis is diagnosed clinically, because laboratory findings do not distinguish JIA uniquely from other diseases. Historical elements that suggest a diagnosis of JIA include morning stiffness or aching lasting a minimum of 15 minutes; a toddler who is grumpy or limps after sleep (mornings or after nap time); improved stiffness and pain progressively throughout the day or after activity; joint stiffness after prolonged inactivity; and restricted range of motion or swollen joints. Pain may not be the primary symptom, because often synovitis or arthritis may be painless. Physical examination clues indicative of JIA include swollen, warm joints with range of motion that is decreased or associated with tenderness. Best initial therapy for low disease activity?

nonsteroidal anti-inflammatory drug (NSAID), such as ibuprofen - The mainstay of treatment for all forms of JIA with low disease activity is NSAIDs, including naproxen, indomethacin, and ibuprofen, and this is the best initial therapy for the girl in this vignette. - Intra-articular glucocorticoid injections may also be initiated for active arthritis. - The use of NSAIDs as monotherapy for more than 2 months is discouraged if arthritis is still active. In that case, a disease-modifying anti-rheumatic drug (DMARD) such as methotrexate should be initiated. - Methotrexate is the recommended initial treatment in patients with higher disease activity. - Other DMARDs include biologic agents such as tumor necrosis factor α inhibitors (etanercept), abatacept, anakinra, and rituximab. - Cyclosporine, a nonbiologic DMARD, is an option for treatment of JIA in those who fail standard therapy. - Adverse effects of the biologic agents commonly include local injection site or infusion reactions, increased infection risk, elevated liver enzymes, cytopenias, and hypogammaglobulinemia. Biologic agents currently carry a warning label regarding the possibility of lymphoproliferative malignancies.

A previously healthy, 13-year-old adolescent boy with an abrupt onset of emesis, abdominal cramps, and watery diarrhea is seen in the emergency department. One day ago, he visited a local park with his younger siblings. The public park has a picnic ground, lake, paddle boats, and a splash pad. The patient's siblings who swam at the lake with him are also ill with a similar gastrointestinal illness. He has a temperature of 38.1°C, dry mucous membranes, and sunken eyes. Capillary refill time is 3 seconds. The rest of the examination findings are unremarkable. Laboratory evaluation reveals a serum sodium concentration of 128 mEq/L (128 mmol/L). Of the following, the MOST likely cause of this illness is

norovirus - Boy developed an abrupt onset of gastroenteritis (AGE) following a visit to a public lake with his siblings. A similar illness is affecting other family members. This scenario is highly suggestive of norovirus infection, given the symptom profile and short incubation period for norovirus (12-48 hours). - Untreated recreational water-associated outbreaks may be caused by enteric pathogens, toxins, or chemicals present in lakes. - In one report from the US Centers for Disease Control and Prevention, a total of 140 recreational water-associated disease outbreaks were reported from 2010 to 2014, resulting in 4,958 illnesses and 2 deaths; enteric pathogens were implicated in 80 of these outbreaks. Norovirus was the most frequent cause, with 21 outbreaks, followed by E coli, Shigella, and Cryptosporidium. - Transmission occurs via ingestion of fecally contaminated water. Sources of fecal contamination include swimmers, storm water runoff, sewage overflows or treatment plant discharges, or animal waste. - Since the introduction of rotavirus vaccine in the United States, human norovirus (previously known as Norwalk virus) is now the leading cause of both sporadic and epidemic AGE in children younger than 5 years. - With the increased availability of sensitive molecular detection methods such as the real-time reverse transcriptase polymerase chain reaction assay, norovirus has also been increasingly identified as a principal cause of foodborne illness and foodborne disease outbreaks in the United States. - Norovirus has been implicated in health care-acquired infections, travel-associated diarrhea, and outbreaks with high attack rates in diverse settings such as long-term care facilities, day care centers, schools, dormitories, military facilities, and cruise ships. - Norovirus is a nonenveloped, single-stranded ribonucleic acid virus of the family Caliciviridae. Immunity from norovirus infection is not permanent, and reinfections can occur throughout life. - Infection with one norovirus serotype may not necessarily confer cross-protection against other serotypes.

Management for healthy kiddos >12 yo with confirmed ingestion of solitary battery </= 12 mm in diameter (and without co-ingestion of another magnet) ....

observation for battery passage at home without initial radiographs

7 yo F with T1D presents with elevated morning glucoses. Receives NPH and regular insulin in the morning, regular insulin with dinner, and NPH insulin at bedtime. Most of her morning blood glucose readings over last mo are over 300. Most appropriate next step in management?

obtain 3am blood glucose for several nights

Girl has Turner syndrome and no signs of puberty at age 12 years. Although she does not yet meet the well-established definition of delayed puberty (lack of breast development by age 13 years in girls), evaluation at this time is indicated, as most girls with Turner syndrome develop primary ovarian failure (hypergonadotropic hypogonadism). Although the risk of developing primary ovarian failure in girls with a mosaic karyotype is lower than in those with a 45,X karyotype, the risk is still high. The best next step in this girl's management is to

obtain gonadotropin levels - to evaluate for hypergonadotropic hypogonadism - Elevated gonadotropin levels (luteinizing hormone [LH] and follicle-stimulating hormone [FSH]) would confirm hypergonadotropic hypogonadism, and pubertal induction with low-dose estradiol would be indicated. - Guidelines recommend starting estradiol for pubertal induction at age 11 to 12 years in girls with Turner syndrome after primary ovarian failure is confirmed. - Therefore, obtaining gonadotropin levels now would allow for pubertal induction at the recommended age if the levels are elevated. - Guidelines recommend starting low and gradually increasing the estradiol dose over 2 to 3 years to an adult replacement dose in order to mimic normal puberty. - The transdermal route of estradiol administration is preferred, as it is the most physiologic. - Progesterone should be added once breakthrough bleeding occurs, or after 2 years of estradiol therapy.

Who should be treated for salmonella?

older adults very young (<3 mos old) immunocompromised

Rapid antigen detection test is recommended if GAS pharyngitis is suspected in children of what age?

older than 3 years - Throat culture should be performed in those who test negative - Diagnosis and treatment are less important in children younger than 3 years because they are unlikely to manifest acute rheumatic fever

Has been shown to decrease the need for intravenous fluid therapy and hospitalization in children in the emergency department with acute gastroenteritis.

one-time dose of ondansetron - The mechanical pathway for vomiting is activated with intestinal stretch (eg, AGE), and/or by the presence of intestinal toxins (eg, food poisoning) - Because this results in activation of 5-hydroxytryptamine (5-HT) and neurokinin receptors, administration of a 5-HT receptor antagonist (eg, ondansetron, granisetron, ginger) to treat vomiting may be appropriate - Oral ondansetron therapy, as a single dose, should be considered for infants and children age six months and older who present to the ED with vomiting related to suspected acute gastroenteritis, and who have mild to moderate dehydration or who have failed oral rehydration therapy.

Healthy children 15-59 mos of age who have not previously received Hib conjugate vaccine require...

only 1 single dose

Infant has multiple café-au-lait macules (CALMs). The presence of 6 or more macules measuring >5 mm in a child is one of the diagnostic criteria for neurofibromatosis type 1 (NF1). What is warranted for evaluation?

ophthalmology examination - to evaluate for iris hamartomas (Lisch nodules) and optic glioma. If present, these findings would meet criteria for diagnosis of NF1. - If she does have NF1, the girl would be at increased risk for central nervous system tumors, however, imaging is not indicated in the absence of concerning symptoms or signs

Who should be consulted when port wine involves the eyelids?

ophtho - 50% of those with sturge weber develop glaucoma

A 17-year-old male patient presents with a 2-day history of a rash on the right flank. It is painful and appears blistered in some areas. The right flank became painful a few days before the onset of the rash. He is current on immunizations. He had chicken pox at age 3. He has been well recently. Past medical history is unremarkable, and he has no known drug allergies. Physical examination shows a vesiculobullous rash on the right flank in a continuous band from the middle of the back spreading around to the mid-axillary line. No other lesions are present. What is the most appropriate treatment option?

oral acyclovir - Herpes zoster (shingles) - acute infection caused by the varicella-zoster virus - less common in children than in adults, but it has been documented in patients as young as 1 week old - most common dermatomes affected are those innervated by C2 to L2 and the 5th and 7th cranial nerves - Oral acyclovir or valacyclovir is effective at shortening healing time, reducing new lesion formation, and minimizing the risk of postherpetic neuralgia. It is most appropriately administered within 72 hours of symptom onset.

An 18-month-old girl is seen for right ear drainage. She is up to date on her immunizations and has a medical history significant for several episodes of acute otitis media. The girl has had a runny nose and cough for 1 week and developed a fever 3 days ago. She was quite fussy overnight and awoke this morning with yellow-red material on her bedding and a crust on her right ear. On physical examination, she is ill appearing but in no distress. Her temperature is 39°C. Her left ear has a normal external ear canal and no pain with manipulation of the tragus or pinna; the tympanic membrane is obscured by thick cerumen. Her right ear has purulent drainage in the canal, which obscures the tympanic membrane. Her pinna and tragus can be moved during evaluation without a notable increase in the girl's discomfort. She has nasal drainage. Of the following, the MOST appropriate initial treatment for this girl is

oral amoxicillin - Girl has otorrhea; her clinical history and physical examination findings suggest acute otitis media (AOM) with tympanic membrane (TM) rupture as the underlying etiology. - Although otic antibiotic drops are appropriate for treating purulent otorrhea in children with tympanostomy tubes, oral rather than otic antibiotics are recommended for children with AOM and TM perforation because the patency of the TM rupture cannot be ensured, and antibiotics administered topically as otic drops may not reach the middle ear. - Amoxicillin (high dose, 90 mg/kg per day) is the preferred initial treatment for AOM. - The most common causes of otorrhea include otitis externa, foreign body in the external ear canal, and AOM with TM rupture.

Recognize Pityriasis Lichenoides et Varioliformis Acuta (PLEVA), and what is the tx of it?

oral erythromycin - 1-2 mo course - appearance of new lesions in successive crops = Pityriasis Lichenoides et Varioliformis Acuta (PLEVA) = pruritic reddish-brown macules and papules --> evolve into vesicular and necrotic lesions just as news crops of papules and macules begin to appear in symmetric distribution - as older lesions resolve, areas of hyperpigmentation and hypopigmentation often occur

On routine exam, 1 yo boy is noted to be pale. He is in 75th %ile for wt and 25th %ile for height. PE is otherwise normal. Hgb 7.4, WBC 7000, Plt count 750,000, retic 0.5%, sickle cell testing negative. stool guac negative. MCV 65. Recommendation?

oral ferrous sulfate - 6 mg/kg of elemental iron divided on a schedule from daily to 4 times daily - tx and therapeutic trial - retic should increase over a week - blood transfusions rarely utilized for tx of IDA - IDA = increased red cell distribution width

A 4-year-old girl presents with recurrent urticaria. She has had 4 episodes in the past 3 months, the last of which occurred 2 weeks ago; they were accompanied by wheezing, with symptoms resolving with an injection of epinephrine in each instance. Each episode occurred after the girl was at a friend's house where she had eaten a peanut butter and jelly sandwich, something she does not eat at home. Past medical history is unremarkable, with no history of atopy or known allergies. Family history is remarkable for her mother's asthma. Growth and development have been normal and immunizations are current. Vital signs are normal. Height and weight are at the 50th and 25th percentiles, respectively. Examination reveals pale-blue, boggy turbinates and puffy, dark lower eyelids. What is the best diagnostic method for the most likely diagnosis?

oral food challenge - Most, but not all, food allergies are due to the formation of food-specific IgE antibodies directed at a component of the offending food. - Peanut allergy currently appears to be the most common food allergy. Other common food allergens are milk, eggs, wheat, soy, tree nuts, fish, and shellfish; peanuts, tree nuts, and shellfish are most likely to cause anaphylaxis. - The definitive test for confirming a food allergy is an oral food challenge (OFC) which consists of gradually introducing the suspected food allergen under medical supervision and monitoring for symptoms.

A 14-year-old girl presents with 3-4 months of bilateral lower extremity achy swelling. It starts in her feet and progressively extends upward to her knees by the end of the day. She has also had other vague intermittent issues, including unexplained fatigue, occasional severe migraine-like headaches, hot flashes or flushes, morning stiffness, and mild depression. On exam, her pulse is 78/min, BP 110/70 mm Hg, and temp 37°C. She has 1-2+ edema in the lower legs, ankles, and feet, which is achy but without pressure points. The rest of her exam is normal except for mild acne vulgaris lesions on her face. Screening lab tests, including CBC, ESR, electrolytes, calcium, magnesium, phosphorus, serum albumin, protein, liver enzymes, TFTs, UA complement studies, cortisol, and ANA, and a chest X-ray are all normal. What is the most likely diagnosis?

orthostatic edema - Orthostatic edema (retention), cyclical edema, and idiopathic edema are generalized systemic disorders with a wide variety of presentations and associated symptoms. - Weight gain typically occurs throughout the day as edema collects in the lower extremities after prolonged sitting or standing. - The exact cause is unknown, but it may be precapillary sphincter dysfunction increasing vascular permeability, with severe engorgement of the microvasculature and orthostatic transudation of fluid into surrounding tissues of the lower extremities. - It occurs almost exclusively in postpubertal women, and it can occur any time during the menstrual cycle. - Patients may also have periodic swelling of the face and eyelids most apparent in the morning. - There may be associated edema of the intracranial contents, causing pseudotumor cerebri. Headaches are common and may be related to increased pressure. - Many patients will also urinate infrequently during the day and have increased urination during bedtime hours when recumbent. - Psychological symptoms are very common, and adolescents often become depressed, anxious, and frustrated. Fatigue, dizziness, light-headedness, syncope, and unexplained tachycardia may be present. - Diagnosis is mainly clinical, but demonstration of consistent weight fluctuations is helpful. - Screening lab tests are done to rule out other possible etiologies. Management is supportive and includes reducing sodium/fluid intake, instituting a progressive exercise regimen, and promoting vasoconstriction to reduce capillary permeability and increase venous return. Pharmacologic therapy with dextroamphetamine has been shown to improve constriction of the precapillary sphincter. Ephedrine sulfate and midodrine have also been used.

In late summer, 5 yo girl noted to have multiple, urticarial, reddish papules - some of which have excoriated - clustered in groups over lower extremities and abdomen. Her mom reports that lesions have intermittently enlarged and itched over last several months. Skin biopsy reveals epidermal and subdermal edema associated with mixed inflammatory infiltrate that contains numerous eosinophils. Most likely etiology of this disorder?

papular urticaria - hypersensitivity to bites - more common in late spring and early months

Boy has edema, hypoalbuminemia, and proteinuria, all of which favor a diagnosis of nephrotic syndrome (NS). The most appropriate treatment of NS in this child is

oral prednisolone - Prednisone or prednisolone is the first-line treatment for idiopathic NS. The recommended starting dose for an initial episode is 60 mg/m2/day (2 mg/kg/day) in two divided doses for 6 weeks, followed by 40 mg/m2 (1.5 mg/kg/day) as a single dose every other day for 6 weeks. - Nephrotic syndrome is known to have relapses and remission. The treatment of relapse includes prednisone at 60 mg/m2/day (2 mg/kg/day) in two divided doses until the urine protein is negative or at a trace amount for 3 days, followed by 40 mg/m2 (1.5 mg/kg/day) as a single dose every other day for 4 weeks. - The single best predictor of long-term prognosis for NS is the initial response to prednisone therapy. Children who experience a relapse on tapering doses of prednisone or within 2 weeks of stopping prednisone therapy are categorized as having steroid-dependent NS. Children who do not respond to an initial course of high-dose prednisone are defined as having steroid-resistant NS. Children with steroid-resistant NS require a renal biopsy to evaluate renal pathology for focal segmental glomerulosclerosis, which has a poor prognosis compared with minimal change disease. - Alternative therapies in steroid-dependent and steroid-resistant NS include mycophenolate mofetil, cyclophosphamide, cyclosporine, tacrolimus, and rituximab. - Angiotensin-converting enzyme inhibitors (enalapril) and angiotensin II receptor blockers (losartan) are used as supplemental therapy to reduce persistent proteinuria in patients with steroid-resistant NS and are not indicated for the child in this vignette, who is experiencing an initial episode of NS.

14 yo girl who is otherwise well is referred for evaluation of persistent mildly pruritic rash, initially diagnosed as varicella, which has now been present 4-5 weeks. Her mom reports that the majority of lesions had spontaneously resolved several weeks earlier, only to be followed by dev't of a new crop of widely distributed lesions. Findings on physical exam are limited to 2- to 5-mm oval and round, reddish-brown papules on trunk and proximal extremities, some of which demonstrate hemorrhagic crusting. Hyperpigmented macules are noted in areas where previous lesions have resolved; mucous membranes are clear. Pt's clinical manifestations are most likely to improve with...

oral tetracycline pityriasis lichenoides et varioliformis acuta (PLEVA) - typically begins as asymptomatic, mildly pruritic, or burning eruption of inflammatory papules and pupulovesicles --> often develop hemorrhagic crusts - spare mucous membranes - hypopigmentation and hyperpigmentation often persists - spontaneous resolution may persist - more extensive and/or symptomatic disease generally responds to extended treatments with tetracyclines, erythromycin, or azithromycin - phototherapy with UV light may also be beneficial

A term neonate is born via precipitous vaginal delivery to a 19-year-old woman who received no prenatal care. She lives with her boyfriend and reports using marijuana and alcohol early in her pregnancy. A rapid HIV test and rapid plasma reagin test performed on the mother soon after delivery were negative. Physical examination reveals a 2.2-kg neonate with microcephaly, hepatosplenomegaly, and petechiae. He is alert, has a good cry, and normal tone and activity. The remainder of his physical examination findings are unremarkable. Laboratory data are notable for thrombocytopenia and hepatitis. Computed tomography scan of the head reveals periventricular calcifications. Evaluation by the ophthalmologist reveals bilateral chorioretinitis. Polymerase chain reaction testing of the neonate's urine specimen on day 2 after birth is positive for cytomegalovirus infection. Of the following, the MOST appropriate antiviral therapy for this neonate is

oral valganciclovir for 6 months - neonate with microcephaly, intracranial calcifications, hepatosplenomegaly, petechial rash, thrombocytopenia, and hepatitis has congenital cytomegalovirus (CMV) infection --> antiviral treatment with oral valganciclovir for 6 months is the recommended treatment for term neonates with symptomatic congenital CMV infection with and without central nervous system disease. Therapy must be started by 4 weeks of age

A 5-year-old girl with no significant medical history is brought to the emergency department with a 2-day history of watery diarrhea mixed with specks of blood, abdominal cramping, and emesis. She was recently hospitalized for cellulitis, and is currently on day 12 of a 14-day course of clindamycin. The girl's temperature is 39°C, and her vital signs are normal. Her physical examination findings are only notable for resolving cellulitis and evidence of moderate dehydration. Laboratory data are significant for a white blood cell count of 28,000/μL (28 × 109/L) and serum albumin of 2.3 g/dL (23 g/L). She is admitted to the hospital for treatment. Of the following, the MOST appropriate antimicrobial agent to treat this girl's illness is

oral vancomycin - Treatment of C difficile disease requires discontinuation of the precipitating antibiotic therapy. - Oral or intravenous metronidazole or oral vancomycin may be prescribed, depending on the severity of the disease. - The treatment of choice for the first occurrence of mild to moderate C difficile disease is oral metronidazole (30 mg/kg per day) every 6 hours for 10 days; intravenous metronidazole is an alternative option. - The C difficile illness in the girl described in the vignette should be considered severe because of the presence of fever and leukocytosis. Recent clinical practice guidelines for C difficile infection in adults and children published by the Infectious Disease Society of America and Society for Healthcare Epidemiology of America recommend oral vancomycin (40 mg/kg per day) every 6 hours for 10 days for the treatment of an initial episode of severe C difficile colitis. - For patients with severe and complicated C difficile disease, combination treatment with oral vancomycin and intravenous metronidazole is recommended for a minimum duration of 10 days. - In addition, in patients with abdominal distention, ileus, or toxic colitis, a vancomycin enema is recommended every 8 hours until clinical improvement.

Laboratory data for a neonate shows hyperammonemia with anion gap acidosis; in the presence of urine ketones, what should be suspected?

organic acid disorder - Common organic acid disorders include isovaleric aciduria, methylmalonic aciduria, propionic aciduria, and maple syrup urine disease. These are all autosomal recessive in their inheritance pattern.

What type of virus is measles ...

paramyxovirus - single stranded helical RNA

A previously healthy, 22-month-old girl is brought to the emergency department in January with a 3-day history of high fever, runny nose, cough, and decreased feeding. On physical examination, she has a temperature of 39.1°C, heart rate of 141 beats/min, respiratory rate of 48 breaths/min, blood pressure of 117/68 mm Hg, and oxygen saturation of 90% in room air. She is tachypneic with nasal flaring and mild subcostal retractions. On lung examination, she has equal breath sounds on both sides with bilateral scattered wheezes and no crackles. A rapid antigen assay from a nasopharyngeal specimen is negative for respiratory syncytial virus and influenza A. A nasopharyngeal specimen has been submitted to test for community respiratory viruses using a molecular assay. Chest radiography shows increased perihilar cuffing with no focal infiltrate. Following oxygen supplementation, her oxygen saturation increases to 92% and improvement in respiratory distress is noted. She is started on intravenous fluids and admitted to the hospital for observation. Of the following, in addition to supportive care, the BEST next management step for this girl is to give

oseltamivir - Bronchiolitis caused by influenza. Given her age (<2 years), she may develop influenza-related complicated disease. Therefore, starting empiric oral oseltamivir therapy is the best next management step.

A 23-month-old girl with fever is seen in the clinic in December. She had been in good health until 2 days ago when she developed rhinorrhea and a mild cough. Last night she woke up crying and had a temperature of 39°C. She received a dose of ibuprofen 3 hours ago. No one has been sick at home, but 2 children in her day care room have been diagnosed with influenza in the last week. She received her first dose of influenza vaccine 3 weeks ago. She has a temperature of 38°C, blood pressure of 98/56 mm Hg, heart rate of 105 beats/min, and respiratory rate of 26 breaths/min. She appears well nourished and is in no distress. There is clear drainage from both nares, and the lungs are clear to auscultation. Of the following, the BEST next step in management is

oseltamivir treatment - The American Academy of Pediatrics recommends antiviral treatment for confirmed or suspected influenza infection in any individual at high risk for complications. - Children younger than 5 years, especially those younger than 2 years, are at increased risk of hospitalization and complications due to influenza.

Small benign bone tumor characterized by oval lucency surrounded by sclerotic bone, most often located in metaphysis of proximal tibia or fibula or spine. Associated pain typically severe and progressive, worsens at night, improved with NSAIDs but not with tylenol

osteoid osteoma - 5-20 yo - surgical removal typically recommended

retinoblastoma is associated with up to 70% risk of developing second high-grade malignancy. Most common tumor associated with retinoblastoma is ...

osteosarcoma

Child has chronic suppurative otitis media (CSOM), with greater than 6 weeks of ear drainage from a perforation in her tympanic membrane. Chronic suppurative otitis media is most common in children between 0 and 5 years of age. Risk factors include anatomical malformations of the ear canal, immunodeficiency, and decreased access to medical care. Antimicrobial treatment for CSOM should always include Pseudomonas coverage. Treatment can typically be achieved with

ototopical agents, such as ofloxacin - ideally administered daily in the office after clearing debris for 7 days - If there is no improvement after 7 days of treatment, a culture of the drainage should be obtained and referral to an otolaryn-gologist should be considered

Hemoglobin S polymerization inhibitor for sickle cell

oxybryta (voxelotor) - ages 4+ - inhibits hemoglobin S polymerization - can be taken with or without hydroxyurea - binds to 20-30% of RBCs in low oxygen conditions --> prevents polymerization and vaso-occlusive process --> 70% change in presence of whether sickle cells were present - makes hemoglobin SS more like hemoglobin A (adult hemoglobin), not as far left as hemoglobin F oxygen dissociation curve - decreased viscosity of blood - other therapies that attack root cause = gene therapy, HU - dosing: 1500mg (around 3 tablets) daily for 12 and older (>/= 40 kg) [1000mg for hepatic impairment]; wt-based for younger + with or without food, with or without hydroxyurea - more common adverse effects ~ abdominal pain, rash (can use antihistamines with)

11 mo old afebrile, well-appearing girl presents with rash of 1 day duration. First began on her trunk but is now characterized by widespread erythematous blanching macules that also involve the face, neck, and upper extremities. Her mother reports that for several days prior to the morning of presentation, the patient had a febrile illness with a temperature as high as 102.6 F. She was treated with only additional fluids and tylenol. Most likely cause of pt's clinical finding?

palpebral and periorbital edema - roseola = trunk --> extremities, face, neck - nonpruritic rash - peripheral halo of vasoconstriction

Cardiac chest pain is rare in children. In taking the history, providers should ask about

palpitations syncope chest pain with exertion family history of genetic or connective tissue disease (eg, Marfan syndrome) history of Kawasaki disease or cardiac surgery

A 6-year-old boy presents with occasional nightmares after the family was involved in a traffic accident 2 years ago. He has been undergoing counseling and behavioral therapy for the past 2 months because of the nightmares; however, for the last 2 weeks his parents have found him sleeping in the hallway in the middle of the night and, on occasion, sleeping on the living room couch. On being asked the next morning, the child does not seem to recall how he got there. They are puzzled by this new development and fear that the boy has been sleepwalking. Under the International Classification of Sleep Disorders, 5th edition (ICSD-5), what is the classification of this condition?

parasomnia - undesirable physical or experiential events that accompany sleep - consist of abnormal sleep-related movements, behaviors, emotions, perceptions, dreaming, and autonomic nervous system functioning - disorders of the arousal and sleep stage transition - manifestations of CNS activation and autonomic nervous system changes with skeletal muscle activity - often occur in conjunction with other sleep disorders (e.g., obstructive sleep apnea syndrome, narcolepsy) and it is common for several parasomnias to occur in the same patient

Most important factor influencing children to wear helmets ...

parental helmet use

Dev'tal regression is common after birth of a sibling and will frequently resolve if ...

parents spend extra time with older sibling

A 9-month-old infant is seen in the clinic for urgent evaluation. Her parents report that she is sleepy, weak, and listless. She developed a runny nose and increased drooling yesterday. Today, she has had 5 loose stools and decreased oral intake. She has had a normal number of wet diapers. She lives with her parents on a farm. She has no known sick contacts and does not attend daycare. She has a temperature of 37.5°C, heart rate of 80 beats/min, respiratory rate of 50 breaths/min, and blood pressure of 90/50 mm Hg. She has decreased tone and is sleepy, but responds appropriately to painful stimuli. Her pupils are constricted (1-2 mm diameter), and she is tearing and drooling. Her lungs are clear to auscultation. Intermittent intercostal retractions are noted. The heart has a regular rate and rhythm, and her abdomen is soft and nontender without distention or hepatosplenomegaly. Her skin is dry, and she has a capillary refill time of 3 seconds. Of the following, the MOST likely source of the toxin causing her symptoms is

parents' clothing - cholinergic excess consistent with organophosphate poisoning - most likely source of her exposure is an agricultural insecticide - children can be exposed to insecticides through accidental ingestion, inhalation of insecticide sprays, ingestion of contaminated food, or transmission on clothing or fabric - organophosphates can contaminate clothing even after they are washed. In this case, her farmworker parents' clothing is the most likely source.

A 4-year-old girl is admitted to the general pediatric floor for asthma exacerbation secondary to influenza. She is up-to-date on immunizations. She has a temperature of 38.4°C, respiratory rate of 30 breaths/min, and heart rate of 110 beats/min. There is swelling anterior to the left ear along the jawline without fluctuance. There is no redness or tenderness posterior to the ear. Her tonsils are 2+ without erythema or exudates. She has moist mucous membranes and normal dentition. The tympanic membranes appear normal, and the external auditory canal is without debris. Pulmonary examination shows bilateral 2+ wheezing on expiration with no retractions. The remainder of the physical examination findings are unremarkable. Of the following, the MOST likely diagnosis for the preauricular swelling is

parotitis - In a young child with infectious symptoms such as this girl, parotitis is the most likely diagnosis given the location of the swelling in the preauricular area. - The parotid gland is located just inferior and slightly anterior to the bottom of the ear and continues along the jawline. Since routine use of the measles, mumps, and rubella vaccine, mumps is an uncommon cause of parotitis. - Viruses associated with parotitis include cytomegalovirus, enterovirus, Epstein-Barr virus, influenza, and parainfluenza. Children with HIV are also at risk. - Painless preauricular swelling that persists over time or is associated with painless adenopathy should raise concern for malignancy.

A 14-year-old female cross country runner has a 3- to 4-week history of vague anterior knee pain noticed most while climbing stairs and after sitting with her legs folded under her. She occasionally notices a crinkling sound and sensation when climbing stairs. On exam, there is no effusion or erythema; range of motion of her knees is normal. She is experiencing slight tenderness over both inferior patellar poles. What is the most likely cause of this patient's knee pain?

patellofemoral pain syndrome - a common cause of vague anterior knee pain, especially in adolescent runners and females. - vague anterior knee pain is common on knee flexion during climbing, squatting, and crawling - crepitus may be present in some cases - affects females more often than males, as the wider pelvis causes a greater angle between the upper and lower leg at the knee joint - if there is no improvement after 4-6 months of conservative treatment and physical therapy, patients should be referred to orthopedic surgeons for possible vastus medialis tightening, tibial tubercle realignment, and lateral retinacular release

14 month old boy, who is receiving antiretroviral therapy for HIV, has yet to receive varicella vaccine. His CD4+ T-lymphocyte percentage has remained >20% for >6 mos. What is the most appropriate plan for varicella vaccination in this patient, with the goal of inducing seroconversion as soon as possible?

patient should receive varicella vaccine during this visit, followed by 2nd dose 3 mos later or later if CD4+ T-lymphocyte percentage remains >/= 15% - varicella vaccine should only be administered if CD4+ count >/= 15% --> 2 doses (1st dose at 12-15 mos) of monovalent varicella vaccine, separated by at least 3 mos (1 mo if >13 yo) recommended

How to decrease risk of fluorosis with brushing teeth in 1 yo

pea-sized toothpaste + using swallowable toothpaste

An 11-year-old boy is having a preparticipation sports evaluation to play on his middle school basketball team. His father has questions about the boy's growth spurt, which is occurring at a younger age than many of his teammates. The boy was born at full term with a weight of 3.5 kg and length of 50 cm, both appropriate for gestational age. His father and paternal uncle are 5'10" tall. Both experienced growth spurts around the same time as their male peers. His mother is 5'2" tall and had menarche at age 11 years. The boy has no siblings. According to the boy's medical record, he started puberty at age 9 years with a sexual maturity rating of 2 for genital development and 1 for pubic hair. His previous height and weight measurements tracked along the 40th and 30th percentiles, respectively. Today his height is 136 cm (60th percentile) and his weight is 34 kg (30th percentile). He has grown 11 cm in the past year. He has mild comedonal acne and no gynecomastia. He has axillary hair and a sexual maturity rating of 3 for genital development and pubic hair. The remainder of his physical examination findings are unremarkable. Of the following, the MOST likely expectation is that the boy will have

peak height velocity that is greater than that of his peers - This boy had earlier than average pubertal onset at age 9 years and is now experiencing an earlier than average growth spurt at age 11 years. Boys with relatively early maturation have greater peak height velocities (PHVs) than their later maturing peers. Transiently, they may achieve a greater height percentile than expected. - However, an earlier initiation of a growth spurt is followed by an earlier cessation of height growth. Consequently, the final adult height for some boys with early maturation may be shorter than prepubertal estimates of final adult height. - For boys, the normal onset of puberty, indicated by an increase in testicular volume to 4 mL or more (or a length of 2.5 cm), occurs between the ages of 9 and 14 years. An average PHV of 9.5 cm/year (range, 6-12 cm/year) is attained by boys with a sexual maturity rating (SMR) of 3 to 4, around age 13.5 years. Height growth then declines until cessation around age 17 years.

Minimal diagnostic criteria for PID

pelvic and/or lower abdominal pain with no other identified cause or if 1 or more of the following 3 minimum criteria are present on pelvic exam 1. cervical motion tenderness 2. uterine tendernes and/or 3. adnexal tenderness

A 16-year-old adolescent girl is seen for a vaginal discharge that began 3 weeks ago at the end of her last menstrual period. The discharge is yellow and is not associated with itching, burning, swelling, foul odor, or dysuria. This week on 2 separate occasions she experienced pain with attempts at vaginal sex. She and her partner agreed to forego sex until she could be evaluated at the clinic. She has not noticed any genital sores or ulcers. She and her male sexual partner are infrequent users of condoms. Her last screen for chlamydia and gonorrhea 6 months ago was negative. She has had 2 previous male sex partners. There is no history of dating violence or sexually transmitted infections. A point-of-care urine pregnancy test is negative. Of the following, the BEST next step in this girl's evaluation is

pelvic exam - dyspareunia (pain with vaginal intercourse) is an indication for a pelvic examination - in sexually active girls, unexplained lower abdominal pain or pelvic pain (including dyspareunia) suggests a diagnosis of pelvic inflammatory disease (PID) - the minimum criteria for the diagnosis and empiric treatment of PID are pain on cervical motion or uterine or adnexal tenderness elicited during the bimanual pelvic examination - other indications to perform a pelvic examination in an adolescent girl include pregnancy, suspicion of a foreign body, known or suspected trauma (including sexual abuse), evaluation of unexplained vaginal bleeding, inability to insert a tampon, and placement of an intrauterine device; a pelvic examination may also help in the evaluation of dysmenorrhea unresponsive to usual interventions or delayed menarche.

An 8-year-old boy is being evaluated for persistent neck pain and torticollis 5 days after a failed front flip on his backyard trampoline. He was initially brought to a local urgent care clinic where cervical spine radiographs were reported as normal. At that time, he was prescribed a nonsteroidal anti-inflammatory drug and provided with a soft collar to wear for comfort. His mother reports no significant change in his symptoms since then. He has no neurologic symptoms, including changes in gait or bowel and bladder function. He was previously healthy with normal growth and development. On physical examination, the boy holds his neck in left lateral flexion with his chin rotated to the right (ie, "cock-robin" position). He is able to actively rotate to the right, but is not able to actively rotate to the left past midline. Findings of a full neurologic examination are normal. Of the following, the BEST next step in this boy's care is to

perform cervical spine computed tomography Boy's history and physical examination findings most strongly suggest atlantoaxial rotary subluxation (AARS). - Given his lack of improvement with initial conservative management, thin-slice dynamic computed tomography (CT) of his cervical spine is the most appropriate next step. - This study will provide a definitive diagnosis, which is needed before proceeding with physical therapy or additional treatment modalities. - Radiography is often problematic; because of difficulties with patient positioning, the interpretation is often compromised. Therefore, some experts recommend proceeding directly to CT scanning without prior radiography in these cases. - Atlantoaxial rotary subluxation, a subluxation of the facet joints between C1 and C2, should be suspected in any child with new-onset painful torticollis. - Atlantoaxial rotary subluxation will typically resolve within 1 to 2 weeks with conservative management, which may include rest, soft collar, nonsteroidal anti-inflammatory drugs, stretching exercises, and/or physical therapy. Prolonged persistence of this subluxation makes definitive treatment more difficult, and these patients should be closely followed to ensure resolution. Children with symptoms that persist despite these measures, or those with delayed presentation, should be referred to a neurosurgeon or pediatric orthopedic surgeon for initiation of traction therapy. Surgical reduction and fixation is indicated for children who do not respond to traction therapy, have neurologic deficits, or have recurrent episodes of AARS.

Boy has clinical and laboratory features of nephrotic syndrome (NS). A history of eyelid swelling, pitting edema of the ankles, and a 3+ protein level on spot urinalysis favor this diagnosis. To meet the criteria for idiopathic NS and to differentiate NS from acute glomerulonephritis, the next step in evaluation is to

perform renal function testing assess serum albumin and complement levels

Facial findings in 50% of patients with mono / EBV ...

periorbital and eyelid edema

Distinctive lab finding in pna d/t C trachomatis

peripheral eosinophilia

Pt has sore throat, fever, dysphagia, trismus, asymmetric peritonsillar swelling, and deviation of uvular. This is likely ...

peritonsillar abscess - requires surgical drainage + abx - ENT consult + hospitalization

Red flags that should raise suspicion for brain tumor, and warrant brain imaging, include

persistent headaches that occur on waking or that wake the child from sleep headache associated with confusion persistent vomiting on waking up papilledema new visual field deficits any focal neurological deficit - MR preferred but CT ok if needed urgently

Most common lead point in pediatric intussusception ...

peyer's patches

Guttate psoriasis is often preceded by...

pharyngeal or cutaneous group A strep infection

Patient has developed a right-sided hemothorax as a result of failed central venous catheter placement in the subclavian vein. The most appropriate intervention is to

place a thoracostomy tube in the affected side of the side of the chest - to evacuate the hemothorax - Hemothorax occurs when blood collects in the pleural space and may result from direct injury to the lung parenchyma, trauma to the chest wall, or injury to intrathoracic vascular structures.

Hyphema management includes

placement of an eye shield bed rest eye rest topical cycloplegics corticosteroids appropriate analgesia - The head of the bed should be elevated to 30 degrees to allow the hyphema to settle at the bottom of the anterior chamber without obscuring the visual axis while preserving blood flow to the eye - Nausea should be managed aggressively because vomiting will cause a rapid increase in intraocular pressure and can exacerbate the hyphema - Most hyphemas can safely be managed on an outpatient basis; however, hyphemas associated with increased intraocular pressure or high-grade hyphemas warrant admission to the hospital - In addition, children with underlying bleeding disorders, sickle cell disease, and those taking anticoagulation medications are at increased risk for complications and warrant admission to the hospital for closer monitoring and to minimize the risk of rebleeding

Hyphema management includes

placement of an eye shield bed rest eye rest topical cycloplegics corticosteroids appropriate analgesia

A 3-year-old boy has been in the pediatric intensive unit since sustaining a severe hypoxic-ischemic brain injury after drowning in the family pool 3 weeks ago. He was pulseless when emergency medical services arrived. Return of spontaneous circulation was achieved after 30 minutes of resuscitation. Over the following 3 weeks, his hemodynamics, oxygenation, and ventilation have improved, but his neurologic prognosis has remained poor. He was extubated twice, but required re-intubation because of hypopnea and hypoxia. He is not currently on any cardiac medications or sedatives, and he is on low ventilator settings. He is tolerating feeds through a nasogastric tube. He is noncognitive. He occasionally opens his eyes spontaneously but does not appear to recognize his parents. He does not respond to verbal stimuli, but responds to painful stimuli with flexor posturing. He occasionally breathes over the ventilator. Cough and gag reflexes are absent. The medical team, including the palliative care service, has had extensive discussions with his parents since early in his admission and has described his likely neurologic prognosis as a persistent vegetative state. However, his parents are convinced that they have seen him communicate and smile in the past few days. They believe that a miracle will occur and that he will eventually wake up, but he needs more time. The medical team has informed them that in order to sustain his life, he would require a tracheostomy and a gastrostomy (G-tube), need constant care, and remain dependent on technology, without neurologic recovery. In response, the parents have expressed their wish to prolong his life and have identified numerous caretakers within the family. Of the following, the BEST next step is to

plan tracheostomy and gastrostomy - There have been extensive discussions with the family regarding the likelihood that he will remain in a persistent vegetative state, yet they wish to prolong his life, despite the need for a tracheostomy and a gastrostomy (G-tube). Therefore, the best next step would be to proceed with the tracheostomy and G-tube placement.

Extremely preterm infants are unable to concentrate their urine (leading to hypovolemia and hypernatremia). What reduces the risk of hypernatremia?

plastic wrap and humidified env't

Children 2 years of age and older scheduled to receive cochlear implants should receive a single dose of what vaccine?

pneumococcal polysaccharide vaccine (PPSV23) - ideally at least 2 weeks before implantation - the pneumococcal polysaccharide vaccine is indicated in children with high-risk conditions; it should be administered at least 8 weeks after any previous pneumococcal conjugate (PCV13) vaccine - pneumococcal vaccines should not be given at the same time as the quadrivalent meningococcal conjugate vaccine MenACWY-D because of interference with immunogenicity to the pneumococcal vaccine

Gram stain of transient Neonatal Pustular Melanosis shows...

polymorphic neutrophils / polymorphonuclear cells

Clinical presentation of CKD depends on the stage and underlying renal disorder. Mild CKD (stages 1-2) may be asymptomatic. Moderate to severe CKD (stages 3-5) may present with

polyuria polydipsia enuresis fatigue anorexia vomiting growth failure

Typical manifestations of neglect are ...

poor growth and dev'tal delay

Complications of ARPKD ....

portal HT (induced by hepatic fibrosis) acute bacterial ascending cholangitis esophageal varices hypersplenism thrombocytopenia/splenic sequestration

In Polyarticular juvenile idiopathic arthritis, what indicates more aggressive disease?

positive ANA, RF, anti-CCP - positive RF = poor prognostic factor

The serologic pattern consistent with a past EBV infection is

positive EBV viral capsid antigen (VCA) IgG negative VCA IgM negative early antigen positive nuclear antigen

During exam of 10 day old boy, midline mass is palpated in lower abdomen. Earlier in course of exam, weak urinary stream was observed during testing for congenital hip dysplasia. Most likely etiology of pt's findings?

posterior urethral valves - most common cause of severe obstructive uropathy in boys

An 18-hour-old male newborn has not voided since birth. He was delivered at 38 weeks' gestation to a 25-year-old primigravida mother via cesarean delivery. Prenatal records indicate that she had oligohydramnios. The neonate's vital signs are stable. He has suprapubic swelling. The remainder of the physical examination findings are unremarkable. Renal ultrasonography shows bilateral hydronephrosis with ureteronephrosis and a distended urinary bladder. Of the following, the MOST likely diagnosis in this neonate is

posterior urethral valves - Delayed passage of urine and a distended urinary bladder with bilateral hydroureteronephrosis on renal ultrasonography favor a posterior urethral valve. - The neonate has features suggestive of a distal obstructive uropathy. The history of delayed passage of urine and a distended urinary bladder with bilateral hydroureteronephrosis on renal ultrasonography in this male neonate favors the diagnostic possibility of posterior urethral valves (PUV). - Renal and bladder ultrasonography (RUS) is the first test to be performed in the diagnostic evaluation of obstructive uropathy and will confirm hydronephrosis. - Posterior urethral valves require an urgent intervention in the form of urinary catheterization to temporarily relieve obstruction and prevent further renal damage. - Fulguration of the membranous folds using a cystoscope is the most definitive treatment of PUV. Once the obstruction is relieved, the child needs to be carefully monitored for postobstructive diuresis. Long-term issues include management of a poorly compliant bladder and may require anticholinergic medications or clean intermittent catheterization. - Chronic kidney disease can be seen in 50% of patients with PUV and requires regular follow up with a pediatric nephrologist.

Patients with velocardiofacial syndrome often experience

postnatal growth restriction deformities of palate hypernasal speech conductive hearing loss cryptorchidism hypextensible hands and tapering fingers congenital heart defects (tet of fallot, right aortic arch, VSD) craniofacial features (narrow palpebral fissures, prominent nose and long, narrow face associated with micrognathia)

A 16-year-old female patient presents with her mother to the pediatrician after missing her period for 3 months. Onset of menses was at age 13, with initial irregularity and then consistent cycles of 28-30 days. She has a boyfriend, but she repeatedly denies sexual activity. She denies recreational drug use. She has been healthy, without any stomach pains, vomiting, diarrhea, vaginal symptoms, or dysuria. Weight is 75th percentile, height is 25th percentile. On further questioning, she admits she has been trying to lose weight and is exercising more to pursue cheerleading; she denies binge eating, purging, or laxative use. There is a positive family history of diabetes. What is the most likely cause of secondary amenorrhea in this patient?

pregnancy

A 16-year-old female patient presents with her mother to the pediatrician after missing her period for 3 months. Onset of menses was at age 13, with initial irregularity and then consistent cycles of 28-30 days. She has a boyfriend, but she repeatedly denies sexual activity. She denies recreational drug use. She has been healthy, without any stomach pains, vomiting, diarrhea, vaginal symptoms, or dysuria. Weight is 75th percentile, height is 25th percentile. On further questioning, she admits she has been trying to lose weight and is exercising more to pursue cheerleading; she denies binge eating, purging, or laxative use. There is a positive family history of diabetes. What is the most likely cause of secondary amenorrhea in this patient?

pregnancy - Secondary amenorrhea is the cessation of menstruation for 3 or more consecutive cycles after at least 24 months of regular monthly cycles when a normal ovulatory pattern should have been established. This most commonly results from some disruption of the hypothalamic-pituitary-ovarian axis. Natural causes of secondary amenorrhea include breastfeeding, menopause, and pregnancy. - Pregnancy is the most common cause of secondary amenorrhea in adolescents. It should be considered in all situations. Denial of sexual activity does not eliminate the need to rule out possible pregnancy.

A 15-year-old girl is brought to the emergency department with right lower quadrant abdominal pain for 48 hours. She reports intermittent, sharp pain that she has not experienced in the past. The adolescent denies vomiting, diarrhea, or fever, but has been feeling nauseated. She has had a decrease in her appetite, but is able to tolerate solids and liquids. The girl had menarche at age 12 years and her menstrual periods are regular. Her last period was 2 weeks ago and lasted for 5 days. She states that she has never been sexually active. Abdominal and pelvic ultrasonography demonstrates a 2.5 × 1.0 × 1.5 cm fluid-filled right ovarian cyst. There is no evidence of appendicitis or other abnormality. Of the following, the BEST next step in this adolescent's evaluation and management would be to

prescribe NSAID - most likely simple functional ovarian cyst - her pain can be managed conservatively with a nonsteroidal anti-inflammatory drug - adnexal masses are classified as functional cysts, benign neoplasms, or malignant neoplasms - most adnexal masses in childhood are benign - postmenarchal adolescents who undergo an imaging study for the evaluation of abdominal pain often have incidental findings of ovarian cysts - the most common types of cysts are known as simple (physiologic or functional) or complex - simple cysts have clear fluid, whereas complex cysts may have loculations, echoic regions, or solid portions - in adolescents, simple cysts are usually asymptomatic. cysts that grow larger than 6 cm may still be asymptomatic, but these patients can have pelvic pain, irregular periods, pelvic fullness, or ovarian torsion - both simple and complex cysts that reach 6 to 12 cm in size are at increased risk for bleeding into the cyst or rupture, as well as torsion, which is a surgical emergency - physiologic cysts that are asymptomatic and less than 6 cm can be managed conservatively with observation only or cycled oral contraceptive pills. oral contraceptive pills can help prevent the development of additional cysts by suppressing the hypothalamic-pituitary-ovarian axis. - if the cyst persists, or increases in size, referral to a gynecologist may be warranted for possible cystectomy.

A 15-year-old adolescent boy is being evaluated for left knee pain of 4 months' duration. The pain began while playing on 2 different basketball teams, but he denies a specific injury. The pain is primarily anterior and medial to the patella, and worsens during and after activity. Several weeks ago he developed intermittent left knee swelling and reports that the knee will occasionally "get stuck" in a given position. He has no other joint complaints and is otherwise healthy. Findings on physical examination of his left knee include a mild effusion, mild tenderness over the anterior medial femoral condyle, and pain with flexion greater than 90 degrees and with resisted extension. The knee is stable to valgus and varus stress testing; Lachman and posterior drawer test results are negative. The adolescent's gait is antalgic. He feels pain with attempts to squat and jump. Of the following, the BEST next management step for this boy is to

prescribe crutches and order radiography of the knee - Although knee pain is common in young athletes, development of an effusion and mechanical symptoms is suggestive of significant internal derangement and warrants additional evaluation. - The differential diagnosis for this scenario includes osteochondritis dissecans (OCD), symptomatic discoid meniscus, meniscal tear, and plica syndrome. Of these, OCD has the highest risk for permanent damage to the knee, and needs to be ruled out before making a treatment plan.

A 2-month-old boy is seen in the office for a sick visit. His mother is concerned that he is breathing fast and eating less. Over the last few days, while breastfeeding, he has tired out sooner, breathed a little faster, and become sweaty. He now seems to be breathing faster when he is at rest and not eating. He does not have rhinorrhea, cough, congestion, fevers, vomiting, diarrhea, or rash. He is afebrile. He has a heart rate of 158 beats/min, a blood pressure of 75/45 mm Hg, and a respiratory rate of 60 breaths/min. He appears comfortable. His oxygen saturation is 95%. His weight remains at the 50th percentile for age. He has a regular heart rate and rhythm with a normal S1 and physiologically split S2. He has a grade 2/6 holosystolic murmur at the left lower sternal border. He has good perfusion and normal pulses. His liver edge is palpated 3 cm below the right costal margin. Of the following, the BEST next step(s) in treatment is (are) to

prescribe furosemide and refer the infant to a pediatric cardiologist - Hemodynamically significant ventricular septal defect. - At 2 months of age, he is demonstrating that his pulmonary vascular resistance has dropped, making it far easier for blood to be shunted through the ventricular septal defect and pulmonary outflow tract instead of to the systemic circulation. Because of this, he has congested lungs with pulmonary edema and is breathing faster. - The next course of action would be treatment with furosemide to help relieve some of the congestion and to refer him to a pediatric cardiologist for further care. He does not have signs or symptoms of sepsis, and he

The gold standard finding used to diagnose a burn wound infection is

presence of 10^5 bacteria per gram of tissue from a burn wound biopsy or the isolation of fungus or yeast on a culture - It is particularly important to counsel parents to ensure that their hot water heater is set so that the temperature of the water does not exceed 120°F at the faucet.

Supernumerary digits are common findings in newborn period. Most important factor that dictates intervention is...

presence of bony articulation of digit with remaining hand structure

Secondary hypogonadotropic hypogonadism presents with low testosterone (or estrogen) levels and low or inappropriately normal FSH and LH levels. Genetic forms are typically spontaneous mutations and can be differentiated based on ...

presence or absence of olfactory senation

A 7-year-old boy presents with a 6-day history of fever (with a maximum of 103.8° F) accompanied by purulent, green-yellow nasal discharge, a daytime cough that worsens at night, and a tickling sensation at the back of his throat. His activity level has decreased, but he had been eating and drinking well until today. He was started on oral amoxicillin with clavulanate 2 days ago, but fever and other symptoms have persisted. In addition, he has developed discoloration around the left eye. He has had no similar episodes in the past and has had no recent contact with ill persons. Past medical history is unremarkable, with no history of atopy. Family history is also unremarkable. Growth and development have been normal and immunizations are current. Vital signs are normal except for a temperature of 102.6° F and a pulse of 124. He is mildly ill-appearing. There is edema of the left upper and lower eyelids with surrounding erythema; extraocular movements are intact. There is bilateral, green-yellow, purulent nasal discharge and lymphoid hyperplasia of the posterior pharynx. The remainder of the examination is normal. What complication of his condition does this patient have?

preseptal cellulitis - complication of acute bacterial sinusitis - orbital and preseptal involvement accompanying sinusitis usually results from direct spread from the ethmoid sinuses - represents the mild end of the spectrum of orbital involvement; it features eyelid edema and erythema with intact extraocular movements - most common extracranial complication of acute bacterial sinusitis in children, representing 80 - 90% of such cases

Infant has a history of prematurity and birth weight of less than 1,800 g. The recommendation on discharge from the neonatal intensive care unit is feeding with

preterm transitional formula at 22 calories/oz - with a goal of 20 to 30 g/day of weight gain after having reached a weight of 2 kg - The higher-calorie formula of 24 calories/oz is reserved for premature infants who have higher metabolic needs, such as those with significant bronchopulmonary dysplasia or who demonstrate poor weight gain while in the neonatal intensive care unit. - For formula-fed infants, preterm infant formulas mixed to 22 to 24 calories/oz provide the calories and higher vitamin content these infants require; therefore, the 19- or 20-calorie/oz formulas are reserved for term infants. - Fortification should be individualized to optimize growth trajectory during the first year after birth.

Infant has history of prematurity and birth weight of less than 1,800 g. The recommendation on discharge from the neonatal intensive care unit is feeding with

preterm transitional formula at 22 calories/oz - with a goal of 20 to 30 g/day of weight gain after having reached a weight of 2 kg - the higher-calorie formula of 24 calories/oz is reserved for premature infants who have higher metabolic needs, such as those with significant bronchopulmonary dysplasia or who demonstrate poor weight gain while in the neonatal intensive care unit

A screening program conducted at a high school is screening student athletes with electrocardiography to identify undiagnosed heart disease. Of 1,000 students, two students are found to have a prolonged QT interval. Of the following, the parameter of long QT syndrome that can BEST be calculated by these data is the

prevalence

A 14-year-old girl is being evaluated for severe abdominal pain. For each of the past 3 months, she has missed 1 to 2 days of school at the start of her menses because of cramping, nausea, and vomiting. She only experiences these symptoms during menstruation. Her mother has been treating the pain with 400 mg of ibuprofen orally every 8 hours, with minimal relief. The girl had menarche at age 12 years. Her periods occur regularly every 28 days, and last for 5 days. She had never experienced menstrual cramping before the previous 3 months. Her mother has a history of fibroids, and her maternal aunt has endometriosis. The girl has a blood pressure of 108/70 mm Hg, heart rate of 80 beats/min, respiratory rate of 18 breaths/min, and temperature of 37°C. There is mild diffuse tenderness of her lower abdomen. There is no guarding, rebound tenderness, palpable masses, or costovertebral angle tenderness. The remainder of the examination findings are unremarkable. Of the following, the girl's MOST likely diagnosis is

primary dysmenorrhea - most common gynecologic condition in adolescent girls, and one of the most common reasons for adolescent girls to miss school - painful menses without pelvic pathology, it is usually associated with crampy, lower abdominal pain that may present a few days before the onset of bleeding and can last for a few days or throughout the entire period - symptoms tend to be most severe during the first few days of bleeding - menstrual cramps tend to begin 1 to 3 years after menarche, when a girl has ovulatory cycles, and can be associated with nausea, vomiting, diarrhea, lower back cramping, headache, and mood lability

Significant risk factor for future radiocontrast media reactions

prior reaction to radioiodine contrast media - other risk factors = female gender, asthma, atopy, cardiovascular disease

A 12-year-old boy with asthma that has been well managed with low-dose daily inhaled corticosteroids is brought to the office by his mother because he is needing to use his rescue inhaler more frequently than previously—up to twice daily on a regular basis. Although he reports that he has not missed any doses of his inhaled corticosteroids, a review of his medication refill history indicates no better than 50% adherence with his medication. On physical examination, he is comfortable at rest, with no increased work of breathing or use of accessory muscles. Air entry is normal but expiration is slightly prolonged, and there is a suggestion of end-expiratory wheeze. The remainder of the examination findings are normal. Of the following, the BEST next intervention is to

problem solve with the patient regarding adherence - Medication nonadherence is the primary cause of asthma treatment failure. - Patient and parent education about asthma is the cornerstone of therapy. - Use of an asthma action plan can help patients with self-directed therapy for exacerbations.

Febrile and ill-appearing 30 month old with 3 day hx of URI noted to be drooling excessively. RR is 40. Limited movement of neck to right with resistance and pain when passively moving head side to side. What best describes radiographic findings in this pt?

prominent prevertebral soft tissue swelling that displaces trachea forward = retropharyngeal abscess - surgical drainage often required

Tx of auricular hematomas >2cm...

prompt incision and drainage and pressure dressing

You are providing volunteer medical care on a medical mission in rural Africa. Your first patient brings in a 9-month old daughter who has become progressively lethargic. The mother recently gave birth to a baby boy; at that time, she weaned her daughter off breast milk and onto an artificial diet. On exam, the girl is apathetic and has peripheral edema. Her hair is now loosely rooted and shows alternate areas of pigmentation and depigmentation. Her skin also shows areas of pigmentation and depigmentation. A physical examination reveals a palpable liver. The laboratory test reveals anemia and hypoalbuminemia. Deficiency of which of the following nutrients is responsible for this child's symptoms?

protein - edema is due to hypoalbuminemia - kwashiorkor may sometimes be seen in infants following the birth of another newborn when they are weaned off breast milk and onto a diet consisting almost exclusively of carbohydrates - protein deficiency in such children is responsible for edema and fatty liver - resultant accumulation of lipoprotein within hepatic cells is responsible for fatty and enlarged liver - areas of pigmentation and depigmentation are seen in the skin and similar changes of pigmentation, called flag sign, are also seen in the hair - hairs are thin and loosely rooted

A 17-year-old boy was stranded for 2 days in the mountains after losing his way while hiking. The ambient temperature reached as low as -10°C (14°F) for approximately 6 hours, with winds as high as 25 mph. He was treated in the field with replacement of wet clothing with dry, loose-fitting clothing. He is conscious, but now presents with numbness of all fingertips, the tip of the nose, and the ears; he has difficulty picking up objects. These areas are mildly edematous, with bluish discoloration of most of the fingertips. There are several clear vesicles at the ears and hands, and a few blood-filled vesicles at the dorsum of the left hand. 24 hours after rapid rewarming, numbness has subsided, pinprick sensation is normal (except for the dorsum of the left hand), edema is reduced (except for the dorsum of the left hand), and color is nearly normal. Pain is minimal. Clear vesicles of the hands and ears, as well as hemorrhagic vesicles at the dorsum of the left hand, are slightly larger. There is a 2 x 3 cm area of purplish discoloration of the dorsum of the left hand, with significant surrounding edema. What is a beneficial next step in management of this condition?

prophylactic abx - Exposure to extreme cold can produce frostbite, a condition in which cells are injured both by the direct freezing effect of cold on cells and the formation of ice crystals within the cells. This leads to cellular dehydration and intravascular stasis, thrombosis, and (eventually) ischemia. - On reperfusion, white blood cell infiltration and cytokine release cause an inflammatory response; this leads to further tissue damage. - Frostbite injuries are classified similarly to burns: first-degree (epidermal), second-degree (full thickness), third-degree (subdermal), and fourth-degree (muscle, bone, tendon involvement). - The cornerstone of treatment is rapid, continuous rewarming with circulating water at 40°- 42° C (104° - 107.6° F) for 20 - 40 minutes for more superficial tissue damage, and up to an hour for deeper injury. - Since there is an area of tissue of questionable vitality, prophylactic antibiotics should be started.

Infant has abnormal newborn screen result with a low T-cell receptor excision circle (TREC) level. This result causes concern for primary immunodeficiency. T-cell receptor excision circle levels may be borderline or have a false positive result in premature infants. It therefore must be repeated until negative or the infant reaches term, 37 weeks. A child in clinic most likely has a primary immunodeficiency, protecting him from infection is of utmost importance. He should be started on

prophylactic antimicrobials immediately (trimethoprim-sulfamethoxazole and fluconazole) - Treatment of infections with trimethoprim-sulfamethoxazole is usually reserved for infants greater than 2 months of age, however, it may be used for prophylaxis (mainly for Pneumocystis jirovecii pneumonia) in infants 4 weeks of age - Antimicrobial prophylaxis should be started urgently for an infant with a suspected primary immunodeficiency. - A low T-cell receptor excision circle (TREC) level on newborn screening raises concern for primary immunodeficiency and should be evaluated by flow cytometry for lymphocyte subsets - The cytomegalovirus (CMV)-positive mother of a neonate or infant with severe combined immunodeficiency disorder can transmit reactivated virus through breast milk, which can lead to CMV viremia in the child

Has been shown to prevent sudden cardiac death in many long QT patients and is first line of therapy ...

propranolol

A neonate is being evaluated 8 hours after birth for decreased perfusion. He was born at 38 weeks' gestation. His mother is a 33-year-old woman with a history of poorly controlled gestational diabetes. Her laboratory testing is significant for group B Streptococcus-negative status and hemoglobin A1c of 9.3% at 36 weeks' gestation. The neonate has breastfed twice. His vital signs show a heart rate of 205 beats/min, respiratory rate of 35 breaths/min, blood pressure of 45/23 mm Hg, and temperature of 37.2°C. On physical examination, he appears plethoric, has a 1/6 systolic murmur at the left lower sternal border, and +1 peripheral pulses. Echocardiography reveals asymmetric septal hypertrophy. Of the following, the pharmacologic therapy MOST appropriate for this neonate is

propranolol - asymmetric hypertrophy of the intraventricular septum - pathogenesis is thought to be related to glycogen loading after hyperglycemia and hyperinsulinemia - functionally, these neonates have decreased cardiac output - they may require a normal saline bolus to maintain adequate preload - some will need pharmacologic treatment with a β-blocker such as propranolol - assymmetric septal hypertrophy resolves over time

A 28-year-old mother with diabetes mellitus having poor glycemic control has delivered a female newborn by cesarean section. The newborn has macrosomia and birth weight is 4.4 kg. At 10 hours of age, she develops respiratory distress. She is dyspneic with a respiratory rate of 70/min. Few bilateral rales are present. Heart rate is 180/min. No murmur can be detected because of marked tachycardia. Liver is palpable 3.5 cm below the costal margin. Chest X-ray shows cardiomegaly. The newborn appears to be in congestive cardiac failure. Echocardiography shows asymmetric ventricular septal hypertrophy. What is the drug of choice for the management of this newborn?

propranolol - most useful agent for management of left ventricular outflow obstruction due to asymmetric ventricular septal hypertrophy - newborns may present with cardiomegaly, heart failure, and poor cardiac output - cardiac output decreases with increasing septal thickness - propranol (β-adrenergic blocker) reduces the ionotropic state of the left ventricle, decreases diastolic dysfunction, and increases left ventricular compliance, reducing myocardial oxygen consumption - most symptoms resolve by 2 weeks, and septal hypertrophy resolves by about 4 months

Any neonate (<2 weeks) + cyanosis and/or shock not explained by something else. What to do?

prostaglandin - ex. CXR with boot shaped heart + decreased pulm vascularity + single S2 = one great vessel = severe tet of fallor / pulm atresia = ductal dependent

Infant has a clinical picture consistent with what with the following: - eosinophilic (allergic) enteropathy as supported by diarrhea, failure to thrive, and edema in the context of a family history of allergies

protein-losing enteropathy (PLE) - The test that would diagnose PLE is a fecal α1-antitrypsin (α1AT) level - Because α1AT is not degraded in the stomach and is not actively secreted or absorbed in the intestine, it is a reliable marker of protein loss in the intestine. - Elevated fecal α1AT levels should prompt further testing for the causes of PLE. - Protein-losing enteropathy is defined by excessive protein loss in the intestine, which can result in hypoalbuminemia and edema. - Intestinal disorders that can cause a PLE can be divided into inflammatory (resulting in increased gut protein loss), infectious, oncologic, rheumatologic, and lymphatic obstruction.

nail-patella syndrome is an AD disorder characterized by absent, hypoplastic, or dystrophic nails and orthopedic anomalies. Mutatio of LMX1B gene. UA reveals ....

proteinuria with or without hematuria

A 16-year-old patient with relapsed Ewing sarcoma is admitted to the pediatric intensive care unit in respiratory distress. The consensus among members of the oncology service is that her acute lung disease is caused by progression of disease and that there are no effective additional oncologic treatments available. The patient and family previously recorded advance directives specifically restricting endotracheal intubation and mechanical ventilation and stating that no cardiopulmonary resuscitation is to be performed in the event of a cardiac arrest. The patient is visibly uncomfortable and air-hungry despite trials of high-flow nasal cannula and escalation to bilevel positive pressure ventilation. After discussion with the family, in an attempt to make the patient more comfortable, she is given a weight-appropriate dose of morphine. Shortly thereafter, she becomes hypopneic with shallow respiratory effort. Of the following, the MOST appropriate next step is

provision of family support and compassionate care - patient is receiving end-of-life care and displays symptoms of discomfort and air hunger as a result of her terminal cancer - it is appropriate for the clinical team to administer therapies that will increase comfort - a weight-appropriate dose of narcotic is administered, the side effect of which causes further respiratory depression

A 3-year-old girl is brought to the clinic by her foster parents. Child protective services removed the girl from her parents' custody because of allegations of physical abuse toward her infant brother. The girl's foster parents are concerned that she will have difficulty forming appropriate emotional connections. Of the following, the factor MOST likely to be associated with the outcome of concern is a history of

psychological abuse of the girl by her parents - psychological abuse is the most highly associated with development of an attachment disorder - children who experience psychological abuse have higher rates of attachment disorders, disruptive behaviors, developmental and educational delays, and mental health problems - long-term effects of psychological abuse experienced in infancy and toddlerhood can be especially profound

Following difficult vaginal delivery, postterm male infant of diabetic mom weights 10 lb 2 oz and has bony crepitus upon palpation along midportion of left clavicle. What is most likely to be identified if this pt's clinical findings are associated with brachial plexus injury to both C8 and T1?

ptosis and miosis of left eye = dejerene-klumpke palsy = isolated paralysis of intrinsic muscles of hand and long flexors of wrist fingers and hand (claw hand) or ipsilateral Horner syndrome (miosis, ptosis, anhidrosis)

4 month old with hx of frequent spit ups/vomiting returns for follow-up several days after being diagnosed with bronchiolitis at a local ED. Stridor is noted on PE. Barium contrast esophagram reveals anterior filling defect in esophagus on lateral projection. Most likely cause of pt's clinical and radiographic finding?

pulmonary sling - only entity with anterior indentation of esophagus = pulmonary artery sling = rare - left pulmonary artery arises distally off right pulmonary artery

A 9-year-old boy is brought to the office for behavioral concerns. His parents recently met with his school's principal because he has been talking back to his teachers and getting into physical fights with his peers. At home, he throws toys and slams doors when he is not allowed to do what he wants. He refuses to do his chores and homework. The boy's parents state that his behaviors have been difficult to manage since he was young. They are concerned that the behaviors will escalate. He has an older cousin who has been in trouble with the law and is involved in a gang. Of the following, the BEST next management step is to

recommend parent management program - With this training, parents are taught strategies to encourage and reinforce desired behaviors and techniques to decrease unwanted behaviors. - Psychosocial interventions such as individual therapy, parent training, and multimodal treatments are first-line approaches for treating the child or adolescent with antisocial behaviors.

A 2-day-old female neonate is seen in the office for an initial visit. Her mother is 22 years old and has a history of epilepsy treated with levetiracetam. The neonate was born at home with the assistance of a midwife. The delivery was uncomplicated, followed by administration of intramuscular vitamin K and ophthalmic erythromycin. The neonate is breastfeeding and had 2 stools today. Physical examination findings are unremarkable. Her mother recalls no testing being done after the birth. She has no paperwork from the delivery. Of the following, the BEST screening test to perform now is

pulse ox - In 2011, screening with pulse oximetry for critical congenital heart disease was recommended. As home births increase in popularity, pediatric providers should ensure that neonates continue to be screened to optimize outcomes. - The hearing test, screen for inherited diseases of metabolism, and pulse oximetry test should be conducted in a birth center or primary care provider's office. Thus, the neonate in the vignette should undergo pulse oximetry screening. - Screening for critical congenital heart disease should be completed 24 hours after birth or 24 hours after the last respiratory support. - If there is a more than 3% difference between the pre- and postductal saturation values, the test should be repeated in 12 hours. Preductal saturation should be checked on the right hand. Postductal saturation may be measured on either foot. If the neonate fails the test again, the neonate should be referred for echocardiography. Pulse oximetry does not detect all forms of critical congenital heart disease. In particular, coarctation of the aorta and tetralogy of Fallot are less likely to be detected with pulse oximetry screening.

The extinction technique for treating behavioral insomnia involves

putting the child to bed drowsy but awake and waiting progressively longer intervals before checking on the child - The interval is lengthened each subsequent night over a period of 2 weeks until the child falls asleep independently

Homocysteinuria is AR and caused by a defect in the gene coding for cystathione beta-synthase (converts homocysteine into cystathione during metabolism of dietary protein). You can give some pts what enhance the activity of the deficiency enzyme?

pyridoxine (200-1,000 mg/day) - folic acid and B12 also recommended in pts who do not initially respond (folate depletion may cause pt to be unresponsive to pyridoxine) - aspirin also recommended for stroke prophylaxis - close monitoring of protein dietary intake important

Severe combined immunodeficiency represents a group of genetic primary immunodeficiencies defined by the absence or dysfunction of T cells. The definitive test is

quantifying lymphocyte subsets via flow cytometry - condition is curable with allogeneic hematopoietic stem cell transplant

An 8-year-old boy is being evaluated for repeated episodes of wheezing with viral respiratory infections, nocturnal waking with cough and occasional wheezing, and decreased exercise endurance. His history is significant only for respiratory syncytial virus infection at age 1 year. The mother smoked cigarettes during the first few weeks of her pregnancy but then stopped. The boy lives with his family on a dairy farm. On physical examination, he has a respiratory rate of 15 breaths/min, heart rate of 80 beats/min, oxygen saturation of 99% in room air, and body mass index greater than the 95th percentile. There is no increased work of breathing or use of accessory muscles. Breath sounds are clear with good aeration throughout, even with forced expiration. His parents ask about the boy's risk of asthma persisting into his adolescence. Of the following, the factor MOST associated with an increased likelihood of this persistence is

rapid weight gain in childhood - Obesity and vitamin D deficiency are now recognized as major factors in both the development and persistence of asthma. - Rapid weight gain in childhood is associated with persistence of asthma into adolescence and beyond. - Residence on a farm and frequent exposure to endotoxins may be protective against the early development of allergies and wheezing.

You are evaluating a 17-year-old young man who had been stranded for 2 days in the Cascade Mountains after losing his way while hiking. The ambient temperature reached as low as -10°C (14°F) for approximately 6 hours, with winds as high as 25 mph. He was treated in the field with replacement of wet clothing with dry, loose-fitting clothing. He was conscious, but he noted numbness of all fingertips, the tip of the nose and the ears, and difficulty picking up objects. These areas are mildly edematous, with bluish discoloration of most of the fingertips. There are several clear vesicles at the ears and hands and a few blood-filled vesicles at the dorsum of the left hand. What is the central component of management of this condition?

rapid, continuous rewarming - Exposure to extreme cold can produce frostbite, a condition in which cells are injured by both the direct freezing effect of cold on cells and formation of ice crystals within the cells. This leads to cellular dehydration and intravascular stasis, thrombosis, and (eventually) ischemia. - On reperfusion, white blood cell infiltration and cytokine release cause an inflammatory response, leading to further tissue damage. Areas most frequently affected are the hands, feet, nose, ears, and lips. - The aims of treatment are to salvage tissue, maximize return of function, and prevent complications. - The cornerstone of treatment is rapid, continuous rewarming with circulating water at 40° - 42°C (104° - 107.6°F) for 20 to 40 minutes for more superficial tissue damage and for up to an hour for deeper injury.

13 yo, previously health male presents to clinic for mild jaundice. He has been recently ill with viral illness with minimal intake. Liver enzymes are normal. Bilirubin is 4. Mostly unconjugated. Does not take any meds. Appropriate clinical action is....

reassurance - jaundiced after period of minimal food = Gilbert syndrome

A 13-year-old adolescent boy is seen in the emergency department for right lower quadrant abdominal pain and diarrhea of 2 days' duration. He has had 5 nonbloody, loose stools. He has not had any fever. One week ago, the family visited a poultry farm. No other travel was reported. The adolescent's history is significant for acute lymphoblastic leukemia treated with chemotherapy at age 3 years. His disease has been in remission since then, he is fully immunized, and he is otherwise healthy. His vital signs include a temperature of 37.5°C, heart rate of 90 beats/min, respiratory rate of 15 breaths/min, and blood pressure of 105/60 mm Hg. Physical examination findings are significant only for increased bowel sounds and mild right lower quadrant abdominal tenderness. Abdominal ultrasonography shows a normal appendix. A stool multiplex polymerase chain reaction panel detects Campylobacter jejuni. Of the following, the BEST next management step for this boy is

reassurance and supportive care - acute gastroenteritis caused by Campylobacter jejuni - best next management step for him is supportive care - mild symptoms of abdominal pain and nonbloody diarrhea, does not appear dehydrated, and currently does not have any underlying immune deficiency or chronic condition that predisposes him to severe disease - mainstay of management is adequate replenishment of fluid losses and correction of any electrolyte imbalance - antibiotics are not indicated in otherwise healthy children with mild to moderate symptoms - antibiotics can be considered for a child with Campylobacter gastroenteritis with high fever, bloody diarrhea, worsening symptoms, symptoms lasting longer than 7 days, or a compromised immune system - antibiotic treatment shortens the duration of illness by 1 to 3 days - when indicated, the antibiotic treatment choices include azithromycin for 1 to 3 days and erythromycin for 5 days

Tx for labial adhesion in infant (if asymptomatic)

reassurance that it is self-limited - Labial adhesions in prepubertal females are most likely the result of chronic irritation of the labia minora. - Girls younger than 5 years are most susceptible to developing labial adhesions because of difficulties with hygiene. - Nearly all children with labial adhesions will have complete self-resolution within 18 months, therefore asymptomatic individuals should receive reassurance. Symptomatic individuals (those with urinary problems such as urinary tract infections or urinary retention) should first undergo a trial of twice daily topical estrogen cream. - Adverse effects of estrogen cream, such as vulvar hyperpigmentation or breast budding, will reverse once the cream is discontinued. - If adverse effects become unacceptable or if adhesions are still present after 8 weeks of topical treatment, manual separation in the office may be performed under local anesthetic with postprocedural care involving daily application of a lubricant for several months to prevent reformation of adhesions. - Referral to a specialist for surgical lysis may be necessary for patients who are unresponsive to conventional treatments.

Current guidelines for asymptomatic infant with c diff in stool

reassurance/no tx

The most compelling and successful method of engaging clinicians in quality improvement endeavors is to

regularly provide them with specific data and frequent reminders to promote process change

A 3-year-old girl is brought to the clinic for evaluation of vaginal "itching." Her parents have observed the girl putting her stuffed animals between her legs while laying face down and moving back and forth on top of it while she is in bed before falling asleep or after waking up. She is otherwise healthy, and her growth and development are normal. She attends preschool, and her teachers report similar behaviors during nap time. Physical examination findings are normal. Of the following, the next BEST step in management of this child is to

reassure the child's parents about this behavior - Masturbation, or self-exploration, is a common childhood behavior and a normal part of development. It involves the self-stimulation of genitalia and may be concerning to parents. - Masturbation in prepubescent children frequently presents as: stereotyped posturing of the legs and/or mechanical pressure on the subrapubic or perineal area associated with grunting, facial flushing and sweating, and irregular breathing. The episodes may be variable in duration and frequency, cease with distraction, and cause no alteration of consciousness. - The parents in the vignette should be reassured that her behavior is normal and can be ignored when it occurs only in a private setting. They should guide her to understand that masturbation should be limited to private settings. - Parents should not ignore sexual behaviors that occur in public settings, as it will reinforce the belief that masturbation is acceptable in public. - Parents should avoid punishing the child for normal sexual behaviors. Behavioral modification and redirection may help limit these behaviors to private settings. - Masturbation may develop in infancy and increase through early childhood, peaking at year 5 and then decreasing as a child understands and adapts to social norms. - Private masturbation continues through adolescence when it may increase, particularly in boys. - It is imperative for the pediatrician to differentiate between normal and abnormal sexual behaviors, particularly because sexual behaviors may be a red flag for physical and/or sexual abuse or neglect. - According to the American Academy of Pediatrics Clinical Report on evaluating childhood sexual behaviors, problematic behaviors are those that are "developmentally inappropriate, intrusive, or abusive" (Kellogg ND). The girl in the vignette is not displaying uncommon or rarely normal sexual behaviors, so she does not need an evaluation for sexual abuse.

Guttate psoriasis is characterized by sudden appearance of numerous, small, discrete ("drop-like"), erythematous papules and plaques. Up to 80% of patients with this disorder have clinical and/or lab evidence of ...

recent or current strep infection - rash = fine or overlying scale; lesions most prominent over trunk and proximal extremities --> usually resolves spontaneously with only symptomatic emollient therapy and topical steroids

A 4-year-old boy is brought to the office by his mother who is concerned that her son is delayed in his development. He speaks in 2- to 3-word phrases and sentences and can follow 2-step commands. He can balance on each foot and hop. He has difficulty catching a ball. The boy can draw a 3-part person and tell his age and sex. He knows 1 color and can copy a circle but not a square. He can undress independently; he requires assistance with dressing. His mother is concerned that the boy may have similar challenges with learning as her brother, who received special education services in school for intellectual disability. Of the following, the area of development that correlates MOST closely with this mother's concern is

receptive language - The boy in the vignette has delays in multiple areas of development. He is behind in his expressive and receptive language, fine and gross motor skills, and adaptive skills. - Receptive language is an important early indicator of cognition, and of the choices given, most strongly correlates with this mother's concern about possible intellectual disability. - For a child younger than 5 years, the appropriate term for delay in multiple areas of development is global developmental delay. Once a child is 5 years or older, a diagnosis of intellectual disability can be made if the child is significantly below age expectations for both cognitive and adaptive development.

A 4-year-old boy is brought to the office by his mother who is concerned that her son is delayed in his development. He speaks in 2- to 3-word phrases and sentences and can follow 2-step commands. He can balance on each foot and hop. He has difficulty catching a ball. The boy can draw a 3-part person and tell his age and sex. He knows 1 color and can copy a circle but not a square. He can undress independently; he requires assistance with dressing. His mother is concerned that the boy may have similar challenges with learning as her brother, who received special education services in school for intellectual disability. Of the following, the area of development that correlates MOST closely with this mother's concern is

receptive language delay - The first sign of intellectual disability in a young child can be delay in language development, particularly receptive language, as this correlates with cognitive function.

Forty-eight hours after birth, a neonate born at 35 weeks' gestation is undergoing a car seat challenge prior to discharge home from the newborn nursery. During the 90-minute observation with cardiorespiratory monitoring, he experiences a sustained 20-second oxygen desaturation to 88% in room air, requiring stimulation. His weight is 2,400 g, heart rate is 140 beats/min, respiratory rate is 50 breaths/min, and pre- and postductal oxygen saturations are 98% and 98% on room air, respectively. His physical examination findings are otherwise unremarkable. Of the following, the BEST next step in management is to

rechallenge him in a car bed - The 2017 American Academy of Pediatrics' (AAP) Guidelines for perinatal care recommend that preterm infants be screened with an infant car seat challenge prior to discharge from the hospital. This process involves monitoring the neonate/infant for bradycardia, apnea, or oxygen desaturation for 90 to 120 minutes while in an infant car seat (their own is preferred). - Risk factors for cardiorespiratory events while restrained in a semi-reclined car seat include prematurity, low birth weight, hypotonia (eg, Down syndrome), micrognathia (eg, Pierre Robin sequence), and congenital heart disease. - Neonates/infants who continue to "fail" the car seat challenge should not be discharged to home until investigation for cardiopulmonary abnormalities is performed. Of note, the car seat challenge is controversial, as little evidence currently exists demonstrating an association between long-term neurodevelopmental outcomes and hypoxemic events noted during a car seat challenge.

If an infant experiences a sustained desaturation during a car seat challenge, the best next step is to

rechallenge the neonate in a car bed

An 8-year-old boy presents with a 1-day history of dark colored urine, fever, and lethargy. With the exception of a case of impetigo around his nose approximately 2 weeks ago that was treated with a topical antibacterial cream, his past medical history is non-contributory. No history of sore throat or upper respiratory infection is elicited. His physical examination is significant for a blood pressure of 140/95 mm Hg, facial pallor, slight edema of the extremities, and a healing skin lesion around the left nostril. Urinalysis reveals red blood cell casts, proteinuria, and occasional white blood cells. Complete blood count reveals a mild normochromic anemia. Serum potassium is 6.2 mEq/L, BUN is 33 mg/dL, and creatinine is 1.7 mg/dL. What is the most reliable test to identify the etiology of the patient's condition?

reduced CH50 - Acute glomerulonephritis - hematuria, edema, and hypertension - pathogenesis is likely related to the development and deposition of circulating immune complexes in the kidney - patient develops a nephritic syndrome 1-2 weeks after a streptococcal infection - involvement may range from asymptomatic microscopic hematuria to acute renal failure - depending on the degree of renal involvement, patients develop varying levels of edema, hypertension, and oliguria - acute phase generally resolves in approximately 1 month, but urinary abnormalities may persist over 1 year - Urinalysis generally reveals red blood cells with associated red blood cell casts and proteinuria - Serum chemistries will show varying degrees of hyperkalemia, hyperphosphatemia, and elevated BUN and creatinine.

Liver biopsy of patient with Alagille syndrome shows...

reduced number of bile ducts - as children get older, there is progressive destruction of bile ducts

Most common mechanism to cause SVT in neonate, infant, or child ....

reentry within AV node

A 16-month-old girl is being evaluated for nasal congestion and a runny nose. She has had thick, greenish-yellow nasal discharge for about 1 week, most prominently from her left nostril but present bilaterally. She has occasional sneezing and cough, and her parents have noted a "foul" odor to her breath. She has had no fevers and has normal energy, appetite, and demeanor. The girl has no significant medical history, including no personal or family history of atopy. On physical examination, she is well appearing with age-appropriate vital signs. She has crusting at both nares and thick purulent discharge inside her left nostril. The remainder of her physical examination findings are normal. Of the following, the BEST next management step for this girl is to

reexamining after suctioning of nasal discharge - foreign body in nose

A 14-year-old adolescent girl is seen for a health supervision visit. Her mother, who experienced menarche at age 13 years, is concerned that her daughter has never had a period. The girl had breast budding at age 11 years. She has no history of chronic illnesses or hospitalizations. The girl states that she "eats healthy" and exercises everyday. Her mother reports that her daughter has stopped eating some of her favorite foods and has become a vegetarian. In private, the girl reveals that she wants to lose weight and increase her exercise efforts because her "stomach pooches out" and her "thighs are too big." She is not concerned about her lack of periods. She reports no binge eating, purging behaviors, or substance use. A review of systems is unremarkable; she has no history of gastrointestinal symptoms, fatigue, cold intolerance, or syncope. She just completed her first semester of high school with excellent grades and has a role as a dancer in the school musical. Physical examination reveals a temperature of 36.6°C, heart rate of 64 beats/min, and blood pressure of 105/70 mm Hg with normal orthostatic measurements. Her height is 160 cm (50th percentile), weight is 45 kg (25th percentile), and body mass index is 18 kg/m2 (25th percentile). Review of her growth from age 7 to 13 years reveals a height trajectory along the 50th percentile and body mass index trajectory along the 75th percentile. Her sexual maturity rating of 2 for breast and 3 for pubic hair development is unchanged from 1 year ago. She has hypoactive bowel sounds and dry skin, and her pelvic bones appear prominent. The remainder of her physical examination findings are unremarkable. Laboratory evaluation reveals a normal complete blood cell count, comprehensive metabolic profile, thyroid-stimulating hormone level, and free thyroxine level. Serum levels of estradiol, luteinizing hormone, and follicle-stimulating hormone are low for age. Electrocardiography reveals normal sinus rhythm, with a heart rate of 65 beats/min, a QTc duration of 0.380 seconds, and normal voltage. The diagnosis is discussed with the girl and her mother. ` Of the following, the BEST next management step for this girl is to

refer for family-based therapy - Lack of binge eating or purging for this girl is consistent with restricting-type AN. The best next management step is to refer her for family-based therapy (FBT). - Treatment of AN requires a multifaceted approach. Outpatient treatment options for AN include FBT, cognitive behavioral therapy (CBT), and dialectical behavioral therapy (DBT). - Phase I of FBT puts the parent in control of food choices and meal preparation to facilitate nutritional rehabilitation and healthy eating. - Phase II gradually transitions control back to the patient. - Phase III addresses associated psychosocial issues. - Family-based therapy has shown promise for the treatment of AN among younger adolescents with a short duration of illness.

constitutional delay of puberty treatment

referral for consideration of testosterone therapy - short course of a testosterone ester intramuscularly once per month for 3 to 4 months can accelerate pubertal development and promote earlier initiation of the pubertal growth spurt - can have a positive effect on the psychosocial distress that can accompany constitutional delay - no significant effect on final adult height - reassurance with observation would also be an acceptable management option

2 yo boy + moderate-sized hydrocele that transilluminates well. Testicle otherwise unremarkable. This is a new finding and was not present at the 18 mo WCC. Child is otherwise doing well and is experiencing no symptoms from hydrocele. Most appropriate recommendation this time would be...

referral for surgical evaluation - hydroceles that last past 1 yo may be caused by inguinal hernia --> dev't of new hydrocele or increase in size of congenital hydrocele ~ continued patency of inguinal canal = risk factor for inguinal hernia - acquired hydroceles do not tend t resolve spontaneously

A 14-year-old basketball player is being evaluated for a recurrent left ankle injury. Several days ago he sustained his third inversion injury to this ankle over the past several months. These injuries result in mild swelling over the lateral ankle for several days, which he treats with a compression wrap, ice, and several doses of ibuprofen. He was able to return to full basketball participation within 7 to 10 days after each injury. However, with the current injury, the ankle feels unstable and he tapes it before practice. The patient is otherwise healthy. On physical examination, the adolescent's left ankle is slightly swollen and minimally tender inferior to the lateral malleolus. He has full and pain-free strength and range of motion in both ankles. There is increased laxity with anterior drawer testing on the left compared with the right. His gait is normal and he moves comfortably throughout the evaluation. The adolescent has some difficulty with single leg hop and balance testing on the left leg compared with the right. Of the following, the BEST next step in management for this patient is

referral to a physical therapist - Full recovery after ankle sprains includes restoration of function and normalization of strength and balance. If these are not achieved before return to full activity, the risk of recurrent sprain is significantly increased. - The single leg hop is a good method for assessing strength in the office setting. If this is impaired in the injured leg compared with the uninjured leg, the child would benefit from instruction on therapeutic exercise.

Dizziness in a patient after a concussion is predictive of prolonged recovery. Patients with persistent vestibular symptoms after a concussion should be identified and

referred for rehabilitation - A brief vestibulo-ocular assessment can be readily performed in the provider's office

A 15-year-old adolescent boy has recurrent episodes of sneezing, runny nose, itchy eyes and nose, and cough after cutting the grass. He has treated himself with over-the-counter antihistamines with some symptom relief, but he still wakes up each morning with nasal congestion and sneezing. He has a strong family history of seasonal allergic rhinitis. Physical examination reveals infraorbital edema, pale and swollen nasal mucosa with a slight bluish appearance, and thin rhinorrhea. The posterior pharyngeal wall has a cobblestone appearance but no visible postnasal drainage. His palpebral conjunctivae also have a cobblestone appearance. The remainder of his physical examination findings are unremarkable. Of the following, the pathophysiologic mechanism MOST likely responsible for this adolescent's findings is

release of histamine and leukotrienes from nasal mast cells - The boy has seasonal allergic rhinitis characterized by paroxysmal episodes of sneezing, itching, and rhinorrhea after exposure to pollens to which he is sensitized. - The mechanism of allergic rhinitis involves antigen binding to antigen-specific immunoglobulin E (IgE) on mucosal mast cells and basophils. Bridging of 2 IgE antibodies by antigen leads to a cascade of immunologic reactions, which release preformed mediators from mast cell granules (histamine, tryptase) and generate leukotrienes and other inflammatory mediators from the mast cell membrane. The secondary mediators include chemotaxins for both eosinophils and neutrophils, but the typical allergic reaction is characterized by intense eosinophilic infiltration of the involved area.

A urine specific gravity less than 1.010 and a fractional excretion of sodium of more than 2% is suggestive of

renal tubular injury - Nephrotoxic medications are a common cause of acute kidney injury in hospitalized children.

A black male infant is born at term after a pregnancy with no complications. On routine predischarge screening at 36 hours of age, he is noted to have room air oxygen saturation of 98% on pulse oximetry in his right upper extremity and 93% in his right lower extremity. He is vigorous and nursing well. The remainder of his physical examination findings are normal. Of the following, the MOST appropriate next management step for this neonate is to

repeat pulse ox in 1 hour - The neonate in the vignette has a discrepancy in oxygen saturation between the upper and lower extremities as measured with pulse oximetry. The immediate concern would be for differential perfusion between the upper and lower extremities. Based on the 2018 American Academy of Pediatrics guidelines for use of pulse oximetry to screen for critical congenital heart disease, the most appropriate next step is to repeat pulse oximetry in the right extremities in 1 and 2 hours before investigating any further. - Using a cutoff of less than 95% as abnormal, there is a 76.5% sensitivity and 99.9% specificity for detection of critical congenital heart disease. - For infants with saturation between 90% and 94% and a differential of at least 3% between the upper and lower extremities, repeating measurement in 1 and 2 hours is recommended.

Pinworm infestation is a common cause of anal pruritus in children. Reinfection and autoinfection with pinworms is common, particularly when there is a high parasite burden, in childcare settings, and among family members. Management of pinworm reinfection includes

repeat treatment with the same agent and treatment of all household members - Because of high transmission rates among family members, treatment of reinfection should include an antiparasitic agent for the affected child along with all household members. Treatment options include 2 doses of 1 of the following medications administered orally 2 weeks apart: 1) Pyrantel pamoate (11 mg/kg/dose); available over the counter 2) Albendazole (400 mg/dose) 3) Mebendazole (100 mg/dose) A repeat dose 2 weeks later is recommended for eradication of eggs and hatching larvae.

Nonmaleficence

requires consideration of the potential harms that may accrue to a patient in a given circumstance

The ill-appearing infant described in the vignette has a history of prematurity, an antecedent upper respiratory tract infection, tachypnea, retractions, and wheezing with radiographic evidence of peribronchial cuffing, as well as atelectasis that is consistent with the diagnosis of bronchiolitis caused by a community respiratory virus. Bronchiolitis is a common inflammatory disease of the bronchioles in infants and young children younger than 2 years. Although bronchiolitis is caused by many community respiratory viruses, what are leading causes and can result in severe disease in preterm infants and immunocompromised hosts?

respiratory syncytial virus (RSV) and human metapneumovirus - both members of the Paramyxoviridae family

A 9-year-old girl is seen for diarrhea. Three days ago, she started to have nonbloody, nonbilious emesis and nonbloody diarrhea. The vomiting stopped yesterday, but her diarrhea has continued. She has remained afebrile and has maintained good hydration with an appropriate oral rehydration solution. She has no other symptoms. Before this illness, she had been struggling to find a good maintenance regimen for chronic constipation but she has no other medical problems. The girl's vital signs and physical examination findings are normal. Her weight is tracking at the 50th percentile. Her father asks what kind of diet he should give her at this time. Of the following, the BEST advice to give this girl's father is to

resume a healthy, age-appropriate, well-balanced diet - acute gastroenteritis (AGE) and is well-hydrated - she should resume a healthy, age-appropriate, well-balanced diet - children with AGE who resume a normal, healthy diet early in the course of the illness have improved nutrition compared with those who delay refeeding

2 week old boy presents with his parents for his 1st evaluation following discharge from newborn nursery. No complications noted during pregnancy, but he had bilateral triphalangeal thumbs, wide-set eyes, and a "snub" nose at birth. He appears pale and has a hgb of 7.9. What labs findings is most likely to be identified in this pt?

reticulocytopenia = congenital hypoplastic anemia = Diamond-Blackfan anemia - most presents within first 6 mos of life

During morning report, a third-year pediatric resident discussed the case of a 16-year-old girl who was admitted to the hospital 2 days ago for abdominal pain. The patient is sexually active and her mother is not aware. The patient's nucleic acid amplification test is positive for Chlamydia trachomatis, and her presentation is consistent with pelvic inflammatory disease. Because of resident work hour limits, this patient had 3 different residents in charge of her care over a 48-hour period. Brief handoffs were performed without using the system that was recently adopted by the hospital to help reduce errors in patient care. A key piece of information not transmitted during handoff was that the mother was not aware of the girl's sexual activity and positive finding of Chlamydia infection. This information does not have to be disclosed to the mother, because adolescents have a legal right to testing and treatment of sexually transmitted infections without parental consent. The mother was informed by a resident that the girl tested positive for C trachomatis and pelvic inflammatory disease. The mother became irate that she was not informed that her daughter was sexually active at the time of admission, and the patient was extremely upset that her confidence had been broken. A patient advocate was contacted to assist with calming the mother. The discussion in morning report focused on what adolescents can legally consent to without parental knowledge, and that omission of important information during sign-out can lead to unfavorable situations. Of the following, the BEST next step in the management of this situation is to

review the hospital's handoff process, highlighting the importance of both verbal and written communication - The pediatric residents described in this vignette did not protect the confidentiality of the patient and upset her mother, which could have been avoided with good written and verbal communication, and by following the hospital's guidelines for appropriate handoffs between providers. - Patient safety requires that all team members, the patient, and family members are informed, attentive, and communicate openly with one another. - Use of a standardized handoff process has been shown to reduce medical errors. - Although there are numerous handoff methods, the effectiveness of the I-PASS process is the most evidence based. - The I-PASS method was created to standardize verbal handoffs; studies at several pediatric institutions have shown that it reduces communication errors and preventable adverse events. I-PASS stands for:Illness severity; Patient summary; Action list; Situation awareness and contingency planning; and Synthesis by receiver. - Having a structured sign-out process that involves direct communication between providers and having the receiver of the handoff, the person who is assuming care, summarize the important patient issues highlighted by the resident transferring the care of the patient, has been an excellent tool in the effort to promote patient safety.

Children allergic to milk, nuts, and soy may find what to be a good alternative source of vitamin D and calcium?

rice milk or oat milk - but the family will need to recognize and address the decrease in protein intake - multivitamin supplements or special formulas may be needed in some cases

What biologic or chemical agent would most likely cause an acute gastrointestinal illness?

ricin - One clinical syndrome category involves gastrointestinal illness manifested by possible abdominal pain, vomiting, profuse diarrhea, hypotension, and cardiovascular collapse. - Possible biologic or chemical etiologies include ricin, staphylococcal enterotoxin E, and arsenic. - Ricin is a plant-derived, heat-stable toxin extract of the castor bean that enters cells through endocytosis, is transported to the Golgi apparatus and endoplasmic reticulum, and acts as a protease-resistant, enzymatically active structure that interacts with the sarcin-ricin domain of the large ribosome subunit RNA. This causes a disturbance of translation by preventing the binding of elongation factors to the ribosome and it also inactivates nonribosomal nucleic acid substrates. Rapidly dividing tissues, particularly the gastrointestinal epithelium, are most susceptible such that when ingested it can produce a syndrome of severe gastrointestinal upset, vomiting, hemorrhagic gastroenteritis, shock, and cardiovascular collapse. - Ricin may be ingested, inhaled, or injected. - Ricin has been associated with terrorist activity in the United States when ricin-containing letters were found in the US postal system in 2003 and again the following year in the mail sorter of a congressional post office. Multiple confiscations have also occurred in the US and abroad over the last 5 years. - Treatment is supportive and there is no approved vaccine.

Drug of choice for children less than 1 month old exposed to n. meningitis ...

rifampin

Prophylaxis for household transmission of h influenza to 5 yo sibling ...

rifampin

Up to 15% of pts with suspected cow-protein allergy will have cross-reactivity with ...

soybean protein - can switch to hydrolyzed formula

In contrast to herpetic gingivostomatitis caused by HSV, lesions associated with herpangina generally ...

spare the tongue and gingival surfaces

A 4-year-old girl is seen in the clinic for a routine health supervision visit. Her mother notes that in some photographs the girl seems to have a "lazy eye." Her medical history is uncomplicated, and she has had normal growth and development. On physical examination, the girl's left eye appears to be slightly medially deviated, and the corneal light reflex on the left is off center. Her red light reflexes are normal. Strabismus is suspected. Of the following, the examination finding that BEST supports the girl's suspected diagnosis is when her

right eye is covered, her left eye moves laterally - The parent of the girl in the vignette is concerned about a "lazy eye" and the appearance of medial deviation of her left eye at rest. - Children with strabismus must be referred to ophthalmology for evaluation and treatment to optimize their visual outcome, whereas children with pseudostrabismus do not need additional evaluation or treatment. - The examination finding that best supports a diagnosis of strabismus (manifest esotropia of the left eye) for this girl is that when her right (fixating) eye is covered, her left eye moves laterally. - Because her left eye was inwardly deviated at rest, it would not be expected to move further medially when the right eye is covered. In addition, her fixating (right) eye would not be expected to move either laterally or medially when the left (affected) eye is covered.

10 yo F + dyspnea on exertion and fatigue. Symptoms have been worsening over the past mos. Several mos ago, seemed to tire when playing with her friends. O2 88%. 3/6 medium-pitched holosystolic murmur heard best at lower left sternal border. Mild cyanosis of lips and clubbing of fingers. CXR = dilated central pulmonary arteries, abrupt attenuation and termination of peripheral pulmonary artery branches, right heart enlargement, RVH. What best describes her condition?

right-to-left shunt - Eisenmenger syndrome

Langerhans cell histiocytosis is associated with what on electron microscopy?

rod-shaped granules with latticed matrix - tennis racket shaped cytoplasmic birbeck granules

A 6-month-old unimmunized female infant is brought to the emergency department in January with a 6-hour history of fever, vomiting, and watery, foul-smelling nonbloody diarrhea. One month before presentation, she and her parents arrived in the United States with refugee status from Syria. There is no history of sick contacts. On physical examination, her temperature is 39°C with a heart rate of 130 beats/min, respiratory rate of 54 breaths/min, and a blood pressure of 75/50 mm Hg. She appears severely dehydrated, with dry mucous membranes and a capillary refill time of about 3 seconds. Stool examination is negative for occult blood. Of the following, the MOST likely pathogen causing this infant's illness is

rotavirus - vaccine-preventable, highly contagious disease leading to substantial morbidity and mortality in low-income settings without a universal vaccination program - can range from asymptomatic infection to life-threatening disease because of shock and acidosis from severe dehydration - primary symptoms include abrupt onset of emesis, followed by watery diarrhea and fever - duration of rotavirus gastroenteritis is generally 3 to 8 days

A neonate is born at term via precipitous vaginal delivery to a 22-year-old woman who recently emigrated from El Salvador and did not receive any prenatal care. A rapid human immunodeficiency virus test and rapid plasma reagin test performed on the mother soon after delivery have negative results. The neonate weighs 2.2 kg and has microcephaly. He is alert and has a good cry, normal tone, and normal activity. Red reflex is decreased in the right eye and absent in the left eye. He has mild hepatosplenomegaly, petechiae, and a grade 2/6 systolic ejection murmur on auscultation of the left upper chest. The remainder of his physical examination findings are unremarkable. Laboratory data are notable for thrombocytopenia. A computed tomographic scan of the head reveals calcifications. Audiologic evaluation reveals absent auditory brainstem responses in both ears. Skeletal survey shows the presence of transverse lucent bands in both proximal humeral metaphyses. Evaluation by an ophthalmologist reveals bilateral cataracts. Of the following, the MOST likely etiology for the findings in this neonate is

rubella - clinical manifestations of microcephaly, cataracts, sensorineural hearing impairment, radiolucent osseous lesions, and possible cardiac disease, all of which are highly suggestive of congenital rubella syndrome (CRS) - diagnosis of CRS is confirmed by detection of rubella-specific serum IgM antibody (usually within the first 6 months after birth) or detection of virus in culture or polymerase chain reaction (ie, throat, nose, urine) - documentation of stable or rubella-specific rising IgG titers over the first 7 to 11 months after birth is also suggestive of congenital infection - treatment of CRS is supportive; no antiviral therapy is available - infants with CRS must have close follow-up with audiologic, ophthalmologic, and neurodevelopmental services - infants with CRS often shed the virus in saliva or urine for up to 1 year after birth. Contact isolation for at least 1 year after birth is recommended for infants with proven or suspected CRS

A 9-month-old female infant is presented by the mother. The patient is the mother's first child, and the pregnancy was unplanned. The mother states that the patient eats well, but she seems to purposely spit up the meals within minutes of eating. The infant arches her back and strains while holding her head back. She tightens her abdominal muscles and makes sucking movements with her tongue. The mother says this has been going on for about 3 months. Visual exam reveals that the infant's weight is lower than average, and she appears to have minor symptoms of malnutrition. A complete GI workup is unremarkable, including stool specimen and imaging. What is the most likely diagnosis?

rumination - After eliminating gastrointestinal or other medical conditions, the best tentative diagnosis is rumination: repeated regurgitation of food that persists for at least 1 month. - It is believed that a lack of stimulation, neglect, or a poor parent-child relationship may be responsible for an infant developing this disorder. - It is usually associated with another mental disorder when its onset is in older children or adults.

Teen with SI + no method/plan. Next step?

safety plan + f/u in short interval = lower risk - restriction of lethal means - mental health resources - close f/u

A 6-month-old infant presents for a well-child visit. The boy's parents are concerned about the shape of his head, which they believe is unusual. There are no other complaints. Past medical history and family history are unremarkable. Growth and development are normal and immunizations are current. Vital signs are normal. Weight, height, and head circumference are at the 50th percentile, but the skull appears narrowed from side to side and elongated from front to back. The anterior fontanelle is barely palpable, but is still patent. Ridging of 1 of the cranial sutures is noted. No dysmorphic features or limb abnormalities are present. Craniosynostosis is suspected. What suture is most likely involved?

sagittal suture - most commonly involved suture in craniosynostosis - with premature fusion of the sagittal suture, the anterior-posterior dimension of the skull is elongated while the biparietal dimension is narrowed, resulting in a pattern known as dolichocephaly or scaphocephaly. Cranial growth continues parallel to the sagittal suture but is limited perpendicular to it

fracture that extends through the physis and extends through the epiphysis

salter harris 3

Plain radiograph of a wrist reveals irregularity, metaphyseal fraying and cupping in addition to widening of epiphyses in distal radium and ulna. What clinical manifestations is most likely associated with these radiographical findings?

scoliosis rickets associations - craniotabes = softening at occiput or over parietal bones - widening of costochondral junction (rachitic rosary) - growth plate widening - softening of ribs - scoliosis - enlargement of wrists and ankles - valgus or varus deformities - anterior bowing of tibia and fibula - coxa vara

A 5-year-old boy is brought to the office by his mother who is concerned that her son has been intentionally banging his head. When he is watching videos, he may bang his head several times against the back of his chair. He may bang his head against the wall when he does not want to do something or when he is upset. At bedtime, the boy bangs his head on his pillow until he falls asleep. His mother is worried that he is having headaches or may cause himself serious injury. Of the following, the MOST appropriate next step is

screening for dev'tal delay - Head-banging is a repetitive motor behavior (stereotypy) that usually resolves by age 4 years in typically developing children. Persistence after age 5 years is associated with developmental disorders such as autism or intellectual disability. Thus, the most appropriate next step would be to screen for those conditions in this 5-year-old boy. - Head-banging occurs in 5% to 15% of typically developing children. It usually begins at 9 to 18 months of age and stops by age 4 years. - The behavior can last for less than 15 minutes to more than an hour.

An 8-year-old girl is brought to the office to discuss recent behavioral changes. Her mother reports that her daughter has been having frequent dreams about getting lost in a forest and wakes up crying. Every morning before school, she will persistently ask if she can go to work with her mother instead of going to school. During the day, the school nurse has called to report that the girl refuses to do her assigned work and instead will suddenly cry for no apparent reason. At home, she behaves well with no emotional outbursts, enjoys outings with her mother, and completes her homework without problems. Her mother reports that the behavioral problems started after her husband was deported. Of the following, the MOST likely diagnosis for this girl is

separation anxiety disorder - The child in this vignette most likely has separation anxiety disorder given that she fulfills 3 of 8 diagnostic criteria: her symptoms worsen when separated from her mother and abate when she is physically near her, she is reluctant to go to school because she would be separated from her mother, and she has repeated dreams about separation.

FABER test / Patrick's test / figure of 4 test involves flexion of hip and knee, with abduction and external rotation at hip, in such a way that the ankle of the ipsilateral leg lies atop the opposite knee in a "figure of 4" configuration. Downward force applied on bent knee and opposite hip causes extension at sacroiliac joint ipsilateral to flexed knee. Maneuvers that twist the pelvis, such as the FABER test, are painful in pts with...

septic arthritis at the sacroiliac joint - usually also febrile, irritable, generalized malaise, decreased appetite - may mimic appendicitis or UTI

A 14-year-old adolescent girl is seen in the emergency department for a 4-week history of cough, headache, muscle aches, fatigue, and intermittent fever. Two weeks ago, she was treated with a 10-day course of amoxicillin after chest radiography showed a lower lobe pulmonary infiltrate. Despite treatment, her cough has worsened and she has developed night sweats and left chest pain. Her immunizations are up to date. She has no history of ill contacts, exposure to animals, or international travel. She resides in Phoenix, Arizona, and visits the Grand Canyon frequently. On physical examination, the adolescent has a temperature of 38°C, heart rate of 81 beats/min, respiratory rate of 18 breaths/min, and oxygen saturation of 94% in room air. Breath sounds are decreased over the left lower lung field. Repeat chest radiography demonstrates a persistent opacity in the left lower lung, a small pleural effusion, and left paratracheal and hilar lymph node enlargement. Her white blood cell count is 16,100/μL (16.1 × 109/L) with 60% neutrophils, 30% lymphocytes, 7% monocytes, and 3% eosinophils. An interferon-γ release assay result is negative. Of the following, the BEST next step to establish this adolescent's diagnosis is to perform a

serologic examination - Adolescent resides in Phoenix, Arizona and has complicated community-acquired pneumonia that did not respond to antibiotic therapy. This presentation is suggestive of coccidioidomycosis. - Coccidioidomycosis (ie, San Joaquin fever) is a systemic fungal infection caused by the endemic dimorphic fungus, Coccidioides. The diagnosis should be established serologically. - Coccidioides species are present in arid soil in California, Arizona, and other areas of the southwestern United States. The disease is also prevalent in areas of Mexico and Central and South America. - The 2 Coccidioides species include Coccidioides immitis (primarily found in California) and Coccidioides posadasii (found in Arizona, Utah, New Mexico, and Texas). - The life cycle of Coccidioides species consists of a free-living mycelial phase (found in soil) and a spherule phase (found in infected host tissues). The spherules contain numerous endospores that are released on spherule rupture, resulting in the formation of new spherules in the infected tissue. - Primary pulmonary infection occurs via inhalation in dry and dusty endemic environments after soil-disruptive activities. - Previously healthy individuals can become infected. However, approximately 17% of affected individuals have an underlying immunodeficiency disorder, and 30% to 50% of these develop disseminated (extrapulmonary) disease. - Laboratory diagnosis is made by detecting anticoccidioidal antibodies in serum. Serologic methods include enzyme immunoassay, immunodiffusion test, and complement fixation (CF). - Serologic tests may have negative results in early disease. - In primary infection, enzyme immunoassays and immunodiffusion tests can detect IgM antibodies in 90% of cases by 2 to 3 weeks of illness, but these wane by 5 months. - Although the antibody level does not correlate with disease severity, an elevated CF titer of 1:32 or higher may suggest disseminated disease. Quantitative complement-fixing antibody assays are helpful in monitoring clinical response to therapy. - Anticoccidioidal CF antibody can be detected in the cerebrospinal fluid in the setting of intracranial infection. - Children with uncomplicated coccidioidomycosis pneumonia often improve without antifungal therapy; clinical evaluation, serologic tests, and chest radiography are recommended at follow-up to ensure resolution. - Treatment with fluconazole for 3 months to 1 year is recommended for severe pulmonary disease (eg, consolidation of more than half of 1 lung or areas of both lungs, >10% weight loss, severe chest pain, duration of symptoms >2 months). - Intravenous amphotericin B may be required for critically ill patients (eg, diffuse pneumonitis, extrapulmonary disease) followed by step-down therapy with fluconazole. - In children with disseminated disease (eg, bilateral reticulonodular or miliary pattern on imaging) or diffuse pulmonary disease, therapy with fluconazole is recommended for at least 1 year. =- Given the high risk of relapse, some experts recommend continued treatment for many years. - Central nervous system disease requires lifelong therapy with oral fluconazole.

Most important test to differentiate PSGN from other forms of acute glomerulonephritis

serum complement 3 (C3) - C3 level is usually low and C4 level is normal in children with PSGN - management of PSGN includes restriction of fluids and dietary sodium intake to prevent complications of hypertension and fluid overload - furosemide may be considered for initial management of hypertension and edema in children with PSGN - calcium channel blockers are the preferred agent to treat persistent hypertension - complications of acute kidney injury are managed symptomatically and may rarely require dialysis - poststreptococcal glomerulonephritis is a self-limiting illness with a good prognosis. - typically, the gross hematuria resolves in 1 to 3 weeks; hypertension, azotemia, and proteinuria resolve in 3 to 4 weeks; low C3 levels normalize in 8 to 12 weeks; and microscopic hematuria may persist for 1 year - if low C3 levels persist beyond 12 weeks, a renal biopsy may be indicated to confirm a diagnosis of C3 glomerulopathy or membranoproliferative glomerulonephritis

2 tests of choice to screen newborns for CF ...

serum immunoreactive trypsinogen levels or DNA analysis for mutations in CFTR gene

Patients with psoriasis are at increased risk of developing

several comorbidities, including obesity, metabolic syndrome, arthritis, psychiatric disorders including anxiety and depression, and less commonly, Crohn disease, uveitis, and rheumatoid arthritis

Bartonella henselae skin findings ...

several cutaneous papules - cutaneous papules/pustules at inoculation site

For neonates and infants (up to age 8 weeks) with hematemesis, what differentiates whether the source of the blood is the mother or the neonate/infant?

the Apt-Downey test

Therapy with a gonadotropin-releasing hormone agonist for youth undergoing gender-affirming care shows beneficence by suppressing pubertal progression with the goal of improving gender dysphoria. Guidelines recommend waiting until youth undergoing gender-affirming care achieve

sexual maturity rating 2 to initiate gonadotropin-releasing hormone agonist - This timing allows confirmation of the gender dysphoria diagnosis (gender dysphoria worsens with the onset of puberty), and prevents unnecessary treatment in a prepubertal child

A female infant born at 26 weeks' gestational age required tracheal intubation and ventilator management for 6 weeks. She then underwent extubation and was placed on nasal continuous positive airway pressure (CPAP) and eventually weaned to nasal cannula oxygen at 0.1 L/min. Since her discharge from the hospital at 4 months of age, the girl has had poor growth and repeated episodes of respiratory illness with noisy breathing, stertor, and coarse wheezing that does not respond to inhaled bronchodilators. She is currently 9 months old, and her growth trajectory has continued to decline since hospital discharge despite high-calorie infant formula and additional nutritional support. Of the following, the MOST accurate statement regarding this girl's symptoms is that

she will likely require tracheostomy and long-term ventilator management - infant has critical tracheomalacia impairing ventilation, resulting in chronic respiratory failure, which is leading to growth failure. Since she was successfully weaned from ventilator support, the infant has had progressive respiratory symptoms unresponsive to bronchodilator treatment, which are negatively affecting her health. She will likely require tracheostomy and long-term ventilation to support her respiratory status and promote a good long-term outcome.

HbFAS on hemoglobin electrophoresis ...

sickle cell trait - complication most commonly associated = renal papillary necrosis + gross hematuria; hyposthenuria (inability to concentrate urine)

Klinefelter syndrome is diagnosed with a standard karyotype. Classic laboratory abnormalities include

significantly elevated follicle-stimulating hormone concentration increased luteinizing hormone concentration low testosterone levels 47,XXY karyotype - Medication regimens during transition into adulthood focus on testosterone replacement therapy

A 7-year-old girl is seen in the emergency department for an abnormal gait and change in speech for the past 2 days. Her family recently returned from a camping trip in rural Pennsylvania. On physical examination, the girl is drowsy but arousable. She has bilateral ptosis, her speech is dysarthric, and she is drooling. Lower extremity strength is 4/5 bilaterally at the ankles and knees, and she has diminished grip strength. She is areflexic in all 4 extremities, and her toes are unresponsive to plantar stimulation. She has difficulty maintaining an upright seated position. Sensation is intact to light touch, pinprick, and temperature. No sensory level is identified. Finger-to-nose testing is normal. She can ambulate with significant assistance with a wide based ataxic gait. Of the following, the BEST next step in the evaluation of this child is

skin exam - Child has a rapidly progressing ascending paralysis. This clinical presentation is consistent with several neurological conditions including Guillain-Barré syndrome, early transverse myelitis, botulism, tick paralysis, or encephalitis. - In children with a history of outdoor exposure, such as camping in a tick-endemic area, a thorough skin examination is a critical component of the initial diagnostic evaluation and management. If a tick is identified on examination, removal can result in neurological improvement within an hour and full recovery within a few days. - Tick paralysis causes a rapid, ascending paralysis, which can involve the bulbar, cranial nerves, and respiratory muscles. An unsteady ataxic gait can precede the paralysis. Associated cranial neuropathies can include pupillary dysfunction, ptosis, bilateral facial weakness, and extraocular muscle weakness. Sensory domains, and bowel and bladder function are preserved. The presentation and severity vary with the duration of symptoms. The neurological symptoms are secondary to a neurotoxin secreted by the female tick, which blocks acetylcholine release. In North America, the Rocky Mountain wood tick (Dermacentor andersoni) and American dog tick (Dermacentor variabilis) are the most common vectors.

16 yo male + admitted to psychiatric hospital for depression + has not responded to outpatient intervention + positive for depressed effect and Parkinsonian-like tremor. Found to unexpectedly have elevations in serum aminotransferases and bilirubin concentrations ...

slitlamp exam - Wilson disease

For this child with both acute splenic sequestration crisis and hypoxia in the setting of acute chest syndrome, a packed red blood cell transfusion is warranted. However, the volume of blood to administer should be carefully calculated. Administration of a large volume may lead to cardiac output failure or cause hyperviscosity, which may precipitate a stroke. In the setting of an acute splenic sequestration crisis, the blood cells are trapped in the spleen; they are released back into circulation once the event is reversed. Therefore, transfusions should be administered in what volumes?

small aliquots of 3-5 mL/kg - with monitoring of the complete blood count and frequent spleen checks to ensure it is not enlarging

11 month old male. Fever started yesterday. Has been very irritable. Has had cold symptoms but no vomiting or diarrhea. Mom is frustrated because "he always seems to be sick." Is in church nursery 1x/week. Has had multiple ear infections and pna twice. UTD on vaccines except pneumococcal conjugate vaccine because there has been a shortage- he has received only 1 shot so far. Has had dry rash for several weeks. He is fussy but consolable. His right tympanic membrane is bulging. Has nasal congestion and cervical LAD. Has had multiple dry eczematous patches on trunk, arms, and legs. Spleen tip is palpable. Remainder of exam is normal. CBC shows thrombocytopenia, upper-end normal WBC, low hemoglobin and hct. What other findings would you expect to see with this child's dx?

small platelets - thrombocytopenia + small platelets = diagnostic for WAS = triad: thrombocytopenia, eczema, recurrent infections = x-linked recessive

A 3-year-old boy is receiving a health supervision evaluation. He is an only child with no significant medical history. His teacher at the preschool he has attended for 9 months mentioned that the boy rarely interacts with other children in the classroom and only engages in solo play. He becomes upset when the teacher attempts to involve him in circle time or other group activities. The boy's mother describes him as "shy." He speaks in 3- to 4-word phrases but not all of his words can be understood by a stranger. He follows complex verbal commands, can fill his water glass from a small pitcher, and draws rough circles. He cannot yet draw a figure, and when he dresses himself, he sometimes puts his shirt on backwards or his shoes on the wrong foot. He jumps, climbs, and can walk on tiptoe. He is not yet fully toilet trained. Of the following, the MOST accurate assessment of this boy's development is

social-emotional delay - The 3-year-old boy in the vignette rarely interacts with other children and only engages in solo play; his social-emotional development is delayed. Other realms of his development are age-appropriate. - Social-emotional development does not occur in isolation; normal social-emotional development requires that children concurrently acquire language and fine motor skills.

A 4-year-old girl is evaluated in the emergency department for severe headache and 2 episodes of emesis at home. The parents administered a weight-appropriate dose of acetaminophen prior to arrival without any improvement in her headache. The girl has a 3-week history of intermittent headaches. There is no history of head trauma, vision changes, fever, neck pain, or gait change. She has not had any recent illness and was in her normal state of health prior to the onset of headaches. She was born prematurely at 29 weeks' gestation, requiring a 6-week stay in the neonatal intensive care unit, and has mild, intermittent asthma. The patient appears well developed and in moderate discomfort. Ocular/fundoscopic and neurologic examination findings are normal. Vital signs include a temperature of 37°C, heart rate of 115 beats/min, respiratory rate of 22 breaths/min, blood pressure of 187/105 mm Hg, and oxygen saturation of 97% on room air. Brain computed tomography results are normal. Shortly after the child returns to the emergency department after her scan she has a generalized tonic-clonic seizure that lasts approximately 4 minutes. Of the following, the MOST appropriate next step in this child's treatment is

sodium nitroprusside IV - hypertensive emergency that would be best treated with sodium nitroprusside, intravenously - a hypertensive emergency exists when the blood pressure is significantly elevated for age and there is evidence of end-organ damage, usually involving the central nervous, renal, or cardiovascular systems - this child's seizure is evidence of end-organ damage - sodium nitroprusside, given intravenously is a very potent vasodilator (arterial and venous) with instantaneous onset of action with infusion that can be titrated as a continuous infusion - sodium nitroprusside has very little effect on cardiac output and does not cause reflex tachycardia.

Infant born at term has meningomyelocele, microcephaly, small nose with flat nasal bridge, small mouth with then upper lip, and epicanthal folds. What med did mom take during pregnancy?

sodium valproate - anti-folate actions - first-trimester exposure --> spina bifida

Foods to avoid in ppl with ACD with nickel allergies ...

soy licorice cocoa powder clams legumes cashews

Risk factors for PNALD are premie, low birth wt, IUGR. What is the lipid that has been implicated in the pathogenesis of this?

soy-based lipid emulsions

Experts recommend that children w/ severe atopic dermatitis <12 months of age be evaluated with

specific IgE to peanut (or directly referred to allergy for skin prick testing): -If negative: early feeding/intro of peanut -If positive; referral to allergy for skin prick testing to determine safety of peanut intro

Second stage of tylenol toxicity characterized by ...

stage 1 symptoms + elevated liver enzymes, liver enlargement, RUQ pain or asymptomatic

Pharmacologic intervention for essential hypertension in children is indicated for

stage 2 hypertension symptomatic children presence of end-organ damage (eg, left ventricular hypertrophy) stage 1 hypertension not responsive to lifestyle modification

A 12-year-old girl is seen for evaluation of possible scoliosis after her mother noticed asymmetry of the back when bending forward. The mother underwent brace treatment for scoliosis when she was an adolescent. The girl is otherwise healthy and is premenarchal. She reports no pain or other back-related symptoms. Initial inspection of the back shows slight elevation of the left shoulder. She has full and pain-free range of motion through the hips and spine, but scoliometer measurement with Adams forward bend reveals 8° elevation of the right hemithorax. Of the following, the BEST next step in management is

standing radiographs of spine - Girl has a curve greater than 7° on scoliometer assessment, and therefore needs radiographs for further evaluation. - Girls should be screened at age 10 years and again at 12 years. - Boys should be screened at age 13 or 14 years. - Office assessment should be performed using the Adams forward bend test to obtain scoliometer readings - A scoliometer measurement of greater than 5° to 7° is considered a positive screening result and should be evaluated further with radiographs. - Two key pieces of information from scoliosis radiographs are the Cobb angle and Risser score. The Cobb angle describes the degree of curvature of the spine, and decision-making about referral and treatment is based upon this measure. The Risser score is an assessment of skeletal maturity based upon the appearance of the iliac crest apophysis. With radiographic curves less than 25°: - Evaluation every 6 months during periods of rapid growth and every 12 months when linear growth is slower should be continued until skeletal maturity. - Patients who have a Risser score of 0 to 2 with curves greater than 20° and that have progressed more than 5° should be referred for possible treatment. With radiographic curves of 25° or greater: - Patients should be referred for possible bracing or surgical evaluation. - Bracing will likely be recommended for those with curves between 25° and 40°, but more severe curvature may require surgical treatment. Compliance with an appropriate bracing regimen may minimize curve progression, but bracing does not reverse curvature that has already developed. Bracing is generally considered for patients with scoliosis who are still growing and with curves approaching 25°.

A 10-year-old boy presents with a 1-week history of high grade fever, chills and rigors, weakness, malaise, and loss of appetite. Physical examination reveals a temperature of 103º F; the patient looks toxic. Auscultation of the heart shows tachycardia; there is a pan systolic murmur in the mitral area; it is conducted to the left axilla. The spleen is palpable 1.5cm below the left costal margin. Painless and non-tender erythematous patches are present over the palms and soles. Fundus examination shows retinal hemorrhages. Laboratory investigations show polymorphonuclear leukocytosis and raised ESR. Urinary examination shows microscopic hematuria. What causative organism is most likely responsible for the illness in this child?

staph aureus - high-grade fever, chills and rigors, malaise, weakness, anorexia, murmur of mitral regurgitation (pansystolic murmur in mitral area conducted to the left axilla) with splenomegaly, fundal hemorrhages (Roth spots), and painless non-tender erythematous patches on the palms and soles (Janeway lesions) = acute infective endocarditis - Staphylococcus aureus usually causes an acute and fulminating form of infective endocarditis, which is rapidly progressive - The diagnosis is further supported by the presence of polymorphonuclear leukocytosis, raised ESR, and microscopic hematuria. Staphylococcal endocarditis commonly occurs in patients without any underlying heart disease. - Murmurs of mitral, aortic, and tricuspid regurgitation may appear and may rapidly progress resulting in congestive cardiac failure. - Janeway lesions are more common in acute infective endocarditis.

Discitis is an indolent, intervertebral disc space infection usually involving lower lumbar discs, most common in children younger than 5 yo. Associated signs include gradual onset of malaise, low-grade fever, irritability, low grade fever, loss of strength in lower extremities, decreased deep tendon reflexes, limp at times, refusal to walk. Blood cx's are often sterile. Majority of cultures that are positive grow...

staph aureus - WBC usually normal - acute phase reactants generally elevated

Most common causes of chronic rhinosinusitis...

staph aureus fungi aerobic gram-negative bacilli anaerobes - >12 weeks of symptoms despite medical treatment

11 yo girl of Asian descent + hx of seizure disorder presents to ED with altered mental status and hemiparesis. A noncontrast CT of head reveals blood in basal ganglia, thalamus, and ventricular system. Additional eval with cranial MRI reveals dilated collateral vessels in basal ganglia and thalamus. Most likely to be identified during eval of pt?

stenotic lesions in distal internal carotid arteries around circle of willis on angiography - Moyamoya disease - may present as altered mental status, recurrent headaches, chorea, transient ischemic attacks, hemorrhagic strokes, focal neuro deficits

A 4-year-old child presents with swelling of his eyes in the morning and swelling of his legs and feet at the end of the day. Physical examination finds soft and pitting edema. Blood pressure and serum complement levels are normal. A timed 24-hour urine collection finds heavy proteinuria. Red blood cell casts are absent. Renal function is normal; serum urea and creatinine concentrations are normal. What is the most appropriate next step in the management of this patient?

steroid therapy - In children younger than 6 years of age, the presence of nephrotic syndrome with the absence of hypertension, hypocomplementemia, and azotemia strongly suggests minimal change disease (lipoid nephrosis). - More than 90% of children respond to 8 weeks of steroid therapy with a decrease in proteinuria; therefore, steroid therapy should precede a renal biopsy. While in the acute phase, diuretics can worsen volume depletion, they can control edema in the chronic phase. Management of edema should rely on salt, not fluid, intake restriction.

Dehydroepiandrosterone sulfate is made by

the adrenal gland - marker of adrenal androgen production

A 15-year-old boy presents with bloody diarrhea and abdominal cramping. A double contrast barium enema shows fine serrations and narrowing of the rectum and sigmoid. Stool contains mucus, blood, and white blood cells, but no parasites or bacterial pathogens. Endoscopy shows inflamed mucosa and pseudopolyps. A biopsy finds an extensive inflammatory process in the mucosa and submucosa. The glands are filled with eosinophilic secretions; there is also mild involvement of the terminal ileum. Sulfasalazine treatment is attempted without improvement. What is the most appropriate next step in management?

steroids - Fine serrations (ulcers) and pseudopolyps suggest colitis ulcerosa (ulcerative colitis). - It is usually limited to the colon, but the terminal ileum is occasionally affected (backwash ileitis). - More severe ulcerative colitis cases require corticosteroids in addition to sulfasalazine or 5-amino-salicylacid.

Pyoderma gangrenosium is associated with IBD. 1st-line therapy?

steroids - PG = pathergy = often preceded by trauma --> may be initially erythematous papule that expands and erodes into deep ulcer - characteristic "overhanding" or "undermined" cutaneous border with grey color

15 yo F with sickle cell + severe lower back pain, leg and arm pain, fatigue. Her pain resembles prior sickle cell crises, but she feels more fatigued. 6 days ago, she presented to the ER with a typical sickle cell crisis. At that time, er hgb was 6.7, with retic of 10% and negative type and screen. Received 2 units of pRBCs and was pain controlled with narcotics. Did not require admission because her pain subsided. Today, her hgb is 5.7 with retic of 10%, LDH of 560, and haptoglobin <30. Next step in management?

steroids - recent transfusion in last 5-10 days + increased LDH + decreased hgb compared to pre-transfusion levels = hyperhemolysis (alloAbs and autoAbs) - steroids, rituximab, IVIG can be used to treat delayed hemolytic transfusion reaction

A 5-month-old male infant is seen in the emergency department for refusal to feed, a weak cry, and constipation for the past 2 days. The infant was born at full term via spontaneous vaginal delivery. The perinatal and postnatal periods were uneventful. He has been breastfed since birth. His physical examination findings were normal, with appropriate weight gain at his 4-month health supervision visit. On physical examination today, he is afebrile with normal vital signs. He is alert but has a weak cry and sucking reflex. He has notable head lag, bilateral ptosis, and dilated pupils with a sluggish reaction to light. Neurologic examination reveals reduced movement of all 4 extremities, hypotonia, and decreased stretch reflexes. He responds appropriately to pain. The remainder of his examination findings are normal. Of the following, the test MOST likely to confirm the cause of this infant's symptoms is

stool studies - infant with hypotonia, poor head control, weak cry and suck reflex, and a history of constipation, has a clinical picture suggestive of infant botulism - detection of the botulinum toxin on a stool specimen confirms this diagnosis - Treatment includes the administration of human botulism immune globulin intravenous (BIG-IV) and supportive care

Retropharyngeal abscess most often caused by...

streptococcus pyogenes (group A beta-hemolytic strep) staph aureus respiratory anaerobe

The most common pathogens associated with infective endocarditis are

streptococcus viridans group and Staphylococcus aureus

Recognize little league shoulder via radiograph, in which management includes

stress fracture through the growth plate on the right, known as "little league shoulder," "proximal humeral epiphysitis," or "proximal humeral epiphysiolysis" --> initial treatment is a period of rest to allow for bony healing = cessation of throwing for about 3 months - Tenderness to palpation usually is seen over the proximal humerus. - Pain may be elicited with all range-of-motion testing, but is typically most severe with internal and external rotation. - Strength testing may also elicit pain. - Radiographic evaluation should include anteroposterior, scapular Y, and axillary views.

Most common cause of toilet training refusal ...

strong-willed child - when refusal occurs, encourage parents to stop all reminders to use toilet for period of 2-3 mos --> once reintroduced, let child be completely in control

18 yo M in ED + SI attempt with ingestion of powdered poison + sz but remained awake throughout event + periodic muscle spasms in back + received IV midazolam + some improvement in symptoms. Most likely cause of symptoms?

strychnine - contaminant of some street drugs - glycine antagonist - awake sz - tx = benzos

Gold standard for diagnosing Hirschsprung's ...

suction cup rectal biopsy

Most common mechanism of injury for ACL tear

sudden deceleration and pivoting of knee - Lachman test = specific = anterior translation of tibia on femur

Child meets the criteria for Henoch-Schönlein purpura (HSP) as characterized by palpable purpura, abdominal pain, and features of microscopic hematuria and minimal proteinuria suggesting glomerulonephritis. This child has a mild presentation of disease, and the most appropriate initial treatment is

supportive care Henoch-Schönlein purpura - peak incidence at 4 to 6 years of age - combination of lower extremity purpura, arthritis, gastrointestinal involvement, and renal disease - skin rash is purpuric, palpable, and usually localized to extensor surfaces of the lower extremity, buttocks, and forearms - rash slowly fades with time but can reappear during the first 3 months of initial presentation - approximately 30% to 70% of children also have nonpitting edema of hands, feet, and scalp - arthritis occurs in 60% to 80% of children and presents as swelling and tenderness of the ankle, wrist, knee, or finger joints - Gastrointestinal tract involvement occurs in 50% to 70% of HSP patients and presents with intermittent colicky abdominal pain, vomiting, and blood in stool - Children can also develop complications in the form of intestinal ischemia, perforation, or intussusception - Renal involvement is seen in 30% to 50% of children and presents with varying severity including microscopic hematuria, macroscopic hematuria, proteinuria, nephrotic syndrome, nephritic syndrome, and rapidly progressive glomerulonephritis - Urinalysis should be performed at presentation and repeated at least once a month for 6 months, as long as urine studies are negative for blood and protein - If microscopic hematuria or mild proteinuria is present, urinalysis is repeated weekly to monitor for worsening of proteinuria until it has resolved - Renal function tests are ordered in patients with hematuria, proteinuria, or hypertension - Mild arthritis is managed conservatively, but severe arthritis is an indication to start nonsteroidal anti-inflammatory drugs (NSAIDs). However, NSAIDs should be used cautiously in children with signs of renal involvement.

A 2-month-old male infant is brought by his mother for his 2-month well-baby assessment. He had a normal birth history and has been growing well. The mother nursed him for 3 weeks, then switched to a cow milk-based formula due to nipple soreness. He took the formula well but became fussy 1 week later, crying nonstop for more than 3 hours a day, usually in the evening. The crying does not seem to be related to feedings, and there is no vomiting or diarrhea. He has not been sick or congested and has had normal stools and wet diapers. He seems gassier during these crying episodes, and he is inconsolable by holding and rocking. The mother has two toddlers at home and is becoming frustrated and anxious. His vitals on and physical exam are normal. What is most appropriate management for this infant?

supportive measures - Infants with colic cry excessively (>3 hours per day, >3 days per week, >3 weeks) without any identifiable need. - Colic typically begins at age 2 weeks, peaks at 6 weeks, and usually resolves completely by 16 weeks. - Crying spells are episodic and unrelated to feeding with a diurnal pattern; crying is increased in the evening and night. Such infants are inconsolable for long intervals and cry with extreme intensity. - They draw up their legs, have tense abdomens, arch their backs, and become gassy. This suggests a GI etiology, but aerophagia (swallowing air) will occur with excessive crying, leading to gas.

The majority of children with meningomyelocele have an associated Chiari II malformation and accompanying hydrocephalus. Signs and symptoms of a Chiari II malformation include

swallowing difficulties breath-holding spells apnea stridor hoarse voice choking

In a child with rectal prolapse, what initial diagnostic test would you consider?

sweat chloride - functional constipation and prolonged straining = most common causes of rectal prolapse, followed by chronic diarrhea caused by enteric and parasitic infections

wide complex tachycardia + pulses + unstable -->

synchronized cardioversion - 0.5 - 1 J/kg --> if unaffective, 2nd dose at 2 J/kg - consider sedation first since painful

Neonate is experiencing narrow complex, regular tachycardia and has poor perfusion. Therefore, the treatment of choice is

synchronized cardioversion - Adenosine would often be used to diagnose and treat a narrow complex tachycardia, but in this setting of poor cardiac output (hypotension and poor perfusion), synchronized cardioversion is warranted. - If a child with an arrhythmia becomes unstable, synchronized cardioversion or defibrillation may become necessary. - More organized rhythms warrant synchronized cardioversion to avoid delivering energy during repolarization (shocking on a T wave). - Less organized rhythms (pulseless ventricular tachycardia, ventricular fibrillation) necessitate defibrillation.

For patients with extensive s aureus folliculitis and/or folliculitis that fails to resolve or recurs after topical tx's, we suggest...

systemic abx therapy = dicloxacillin and cephalexin

Combination of hemoptysis with multifocal densities on chest radiography, urinary findings (dark urine and borderline elevated creatinine), anemia, and thrombocytosis suggests

systemic vasculitis such as granulomatosis with polyangiitis (previously called Wegener syndrome) - Measurement of serum antineutrophil cytoplasmic antibodies (ANCA) would be the appropriate diagnostic test

A tall, 10-year-old boy presents with a 2-week history of right leg pain. At first, the occasional pain did not seem significant. Lately, he has been waking up due to pain during the night. Physical examination reveals swelling and a palpable mass on the right femur. Plain X-ray finds a laminated onion skin-type periosteal reaction. A biopsy finds a uniform population of homogenous cells with hyperchromic nuclei and no appreciable extracellular matrix. What disorder is most likely present?

t(11,22) - Ewing's sarcoma - Highly undifferentiated Ewing's sarcoma cells show the translocation t(11,22) which is found in tumors of the peripheral nervous system - The disease has a male predominance and is more common in taller persons - In cases of Ewing sarcoma the femur is the most commonly involved bone, but any bone can be affected. - Lung and lymph nodes are the usual sites of metastasis. - The onion skin-like new bone formation can be seen only in a minority of cases, and it is not pathonomic. - Doxorubicin, cyclophosphamide, and vincristine represent the standard therapy, in addition to surgery. Occasionally, extraosseous Ewing's sarcoma arises from the soft tissues of extremities or the paravertebral region.

Side effects that make ketamine not ideal for patients with cardiac disease, HT, or increased ICP (such as in trauma)...

tachycardia HT worsening ICP

A 1-month-old premature male infant with bronchopulmonary dysplasia (BPD) remains intubated and monitored in the NICU. He has been doing relatively well and is being gradually weaned from the respirator. Suddenly, his O2 saturations and heart rate plummet, and he becomes very dusky. On quick exam, there are decreased breath sounds on the right with an asymmetric chest rise. What is the most likely explanation for his sudden respiratory and clinical change?

tension pneumothorax - Air trappings can occur in patients with lower airway diseases such as BPD and may result in tension pneumothorax. - Since the tracheal tube tends to enter the straighter right main bronchus, a tension pneumothorax most frequently occurs on the right side. - This presents as a marked sudden deterioration in oxygenation, marked bradycardia, and central cyanosis. - Hyperresonance to percussion on the affected side and tracheal deviation away from the affected side may also be present, but it is better appreciated on a larger patient.

First sign of puberty in males ...

testicular and scrotal enlargement without enlargement of penis

3 mo old M + testicular mass + nontender + send of AFP and hCG + scrotal US done. Que es?

testicular teratoma = UC: cystic-appearing lesion with heterogeneous echogenicity and calcifications

Resolved anemia but microcytosis unresponsive to iron replacement therapy ...

thalassemia

The diagnosis of nutritional rickets heavily depends on

the detailed history - can explore many predisposing factors (e.g., decreased exposure to sun, food faddish parents, winter season, overcrowded housing). - Both calcium and phosphorus levels are low in the serum.

Appropriate postexposure tetanus prophylaxis depends on 2 factors: if the child has received at least 3 doses of tetanus toxoid (typically from a primary series of vaccinations) and if the wound is considered to be clean or dirty. Dirty wounds include burns, bites, crush wounds, and wounds that may be contaminated with soil or dirt. If the child has previously received 3 doses of tetanus toxoid and sustained a clean wound, then no postexposure prophylaxis is indicated if the last dose of tetanus toxoid was within the previous 10 years. If the child previously received 3 doses of tetanus toxoid and sustained a dirty wound, a tetanus vaccine is indicated if

the last dose was more than 5 years earlier - A child who did not have 3 previous doses of tetanus toxoid (or if it is uncertain if they did) and who sustained a clean wound requires administration of only a tetanus vaccine, while a dirty wound necessitates administration of both a tetanus vaccine and tetanus immune globulin. - Children younger than 7 years who require a tetanus vaccine under this management algorithm should receive the DTaP vaccine, children 7 to 10 years old should receive Td, and children 11 years and older should receive Tdap.

Based on the history, physical examination findings, and laboratory results, child most likely has a diagnosis of acute idiopathic thrombocytopenic purpura (ITP). This diagnosis is supported by the low platelet count with large (increased mean platelet volume, mean corpuscular volume) platelets, normal white blood cell count and differential, and the fact that the child is otherwise well with no lymphadenopathy or hepatosplenomegaly. This diagnosis is one of exclusion. Idiopathic thrombocytopenic purpura is an immune phenomenon. In most cases, there is a history of recent viral illness. Autoantibodies created toward a glycoprotein on platelets lead to their destruction and clearance, mainly by the spleen and also the liver. Though platelet antibody testing is available, a negative antibody test result does not exclude the diagnosis. In the United States, treatment is usually offered when ...

the platelet count is less than 10 × 103/µL (10 × 109/L) - especially in young individuals or those with a higher risk of bleeding; due to the risk of spontaneous, unprovoked bleeding at such low platelet counts

The mother of a 14-year-old adolescent boy is worried about her son's recent behavior. After school he and 2 friends were recently seen vaping in a garage. She is disappointed and worried he may be susceptible to peer pressure. The boy and his mother live with his maternal grandmother in a low socioeconomic neighborhood. There is no household exposure to tobacco products. He attends the local middle school where he has a good attendance and conduct record. He gets good grades and enjoys reading and playing video games. He has no chronic health conditions. Although his puberty was slower to start than for many of his friends, he is now experiencing a "growth spurt." Of the following, the factor that BEST explains this boy's recent risky behavior is

the pursuit of pleasure or excitement - Risk taking may be associated with positive adolescent development by promoting independence and autonomy. - In general, adolescents have a decreased ability to anticipate danger and a decreased physiologic response to fear. Contextual factors such as living in a low socioeconomic neighborhood, especially a neighborhood with few opportunities and services, may be associated with an increased likelihood of risky behavior. However, the driving factor for adolescent risk taking is the pursuit of excitement and pleasure.

Nephrogenic DI is caused by resistance to vasopressin in the renal tubules. In a water deprivation test, if percent of change in urine osmolality before and after DDAVP is less than 9%, nephrogenic DI is present. Tx/polyuria is reduced and urine osmolality is increased with use of ...

thiazide diuretics

Girl has pneumonia with radiographic evidence of a pleural effusion. Despite receiving 24 hours of broad-spectrum antibiotics, oxygen, and incentive spirometry, her overall status has worsened. Best next step is

to insert a chest tube - Children with pleural effusions initially may not exhibit symptoms beyond the underlying cause. In such scenarios, management can be limited to the treatment of the underlying condition. - However, if the pleural effusion is large or if the child has limited reserve caused by congestive heart failure or respiratory insufficiency, the presence of fluid in the pleural space may impair lung expansion and lead to hypoxia and hypercapnia. - If breathing is significantly impaired, insertion of a chest tube may be warranted regardless of the etiology. - Management of most parapneumonic effusions centers around treatment of the underlying pneumonia, including providing supportive care, antibiotics, and oxygen if needed. - Indications for insertion of a chest tube for a parapneumonic effusion include significant breathing impairment, impairments in oxygenation or ventilation, and overall declining clinical course.

A 9-month-old infant is seen for a health supervision visit. Her initial visit was 5 weeks ago when her new adoptive parents brought her to the clinic, and she received her initial set of vaccinations including DTaP. At that time, she was well nourished and developing well. The infant's mother has heard a news story about pertussis and inquires about when her daughter can have her second dose of DTaP. Of the following, the BEST response to the mother is

today - The Centers for Disease Control and Prevention catch-up vaccination schedule recommends 4 weeks between the first and second dose of DTaP. For the infant in this vignette, the first dose was given 5 weeks ago, making today the preferred response for the timing of the next dose. Children should receive 5 doses of DTaP prior to the age of 7 years. Typically the first dose is given at 2 months but can be given as early as 6 weeks. The following doses are typically given at 4 months, 6 months, 15 months-18 months, and between 4 and 6 years of age. If a child does not receive vaccines on a typical schedule, the minimum intervals between doses are as follows: Between first and second doses: 4 weeks Between second and third doses: 4 weeks Between third and fourth doses: 6 months Between fourth and fifth doses: 6 months

Treating alopecia areata is challenging, and those who have significant disease are best treated by a dermatologist. First-line therapy typically includes

topical corticosteroid, often in conjunction with topical minoxidil - The topical corticosteroid selected usually is an ultrapotent agent (group I [eg, clobetasol propionate]), or for children <10 years of age, a potent agent (group II [eg, fluocinonide]) - To prevent steroid-induced atrophy, application is in a "pulsed" fashion (applied consecutively for 4-5 days, followed by 2-3 days without treatment) - Other options include intralesional or oral corticosteroids, methotrexate, excimer laser, anthralin topically, topical immunotherapy, or oral or topical Janus kinase inhibitors - Locks of Love (www.locksoflove.org) provides hairpieces to financially disadvantaged children younger than 21 years - The National Alopecia Areata Foundation (www.naaf.org) provides information and support for patients and families

Bacterial Superinfection/ Impetigo Most often S. aureus For localized infection, use what?

topical mupirocin (2% cream x 2 weeks) - For more extensive infection, use oral antibiotics (cephalosporin or penicillinase-resistant PCNs)

If, in an adolescent with acne, the majority of lesions are comedonal and noninflammatory, what is the treatment of choice?

topical retinoids - keratolytic (comedolytic)

Recognize granuloma annulare, which is treated with

topical steroid cream - counsel that this can cause skin atrophy

For adults and children with mild perioral dermatitis who desire an attempt to accelerate improvement, we suggest treatment with

topical therapy = topical pimecrolimus, topical erythromycin, and topical metronidazole - topical tacrolimus is an additional option for therapy

Should be considered in infants who have recurrent wheezing that does not seem to be reactive....

tracheomalacia - Most infants with tracheomalacia will improve by 18 to 24 months of age and do not have respiratory compromise requiring ventilator support. - Premature infants and those who have had tracheal intubation are the patients most at risk for tracheomalacia.

3 mo old infant born at 25 weeks gestation + has been intubated through hospital stay, first for respiratory distress syndrome and now for bronchopulmonary dysplasia. Has been weaned to minimal settings, and extubation has been attempted 4 times. Each time the endotracheal tube is removed, he develops increased WOB, expiratory stridor, elevated PCO2. What is most likely preventing successful extubation?

tracheomalacia - dynamic collapse of trachea during expiration - caused by prolonged intubation

18 mo old + evaluated for pallor + drinks 18 oz of whole mild per day + picky eater + mild URI last week but otherwise healthy + hgb 4 + nml WBC and platelet count + low retic count. Cause of symptoms?

transient erythroblastopenia of childhood - TEC - associated with preceding viral illness - children 1-4 yo - increasing pallor and fatigue

5 yo M + 3 days of rt knee pain and limp + no hx of trauma + abducted and externally rotated hip + significant pain with internal rotation. Cause?

transient synovitis

24 hour old infant admitted to NICU for persistent tachycardia. Born at 39 weeks to G1P1 mom with minimal prenatal care and no lab testing. Mom complains of heat intolerance and has bilateral fine hand tremors. PE for baby = bilateral proptosis and diffuse symmetric goiter. Most likely cause of infant's presentation?

transplacental passage of thyroid-stimulating IgG Abs - Graves dz --> passed from mom

A neonate who appears "dusky" is evaluated in the special care nursery. The neonate is vigorous and without distress. The oral mucosa, hands, and feet have a bluish-purple color. Vital signs include a heart rate of 145 beats/min, respiratory rate of 50 breaths/min, blood pressure in the right upper extremity of 75/45 mm Hg, and a room air oxygen saturation, measured by a pulse oximeter located on the right hand, of 65%. When the pulse oximeter is placed on the left lower extremity it measures an oxygen saturation of 80%. Of the following, this child's MOST likely diagnosis is

transposition of greaty arteries - The oxygen saturation levels in the pre- and postductal extremities should either be equal, or the postductal extremity should be lower than the preductal extremity. - The postductal oxygen saturation would be lower if the ductus arteriosus is shunting right to left (ie, pulmonary artery to aorta), as seen in pulmonary arterial hypertension or coarctation of the aorta. The neonate in the vignette demonstrates "reverse differential cyanosis," where the oxygen saturation levels are unequal but in the reverse of the order expected. This can only happen in cases of dextro-transposition of the great vessels with arch obstruction or pulmonary hypertension. - Transposition is the second-most common form of cyanotic congenital heart disease. It typically presents in the first 1 to 2 weeks after birth, if not prenatally diagnosed. A failure in the normal septation of the common trunk into 2 great vessels causes the aorta to be positioned anteriorly, arising off of the right ventricle while the pulmonary artery arises off of the left ventricle; this is referred to as ventriculoarterial discordance. This results in deoxygenated blood returning from the body to the right atrium, then into the right ventricle and back out to the body. Similarly, the oxygenated blood runs in a parallel circuit. Without a site for mixing, oxygenated blood cannot be delivered to the body and deoxygenated blood to the lungs. In fetal life, the patent foramen ovale and patent ductus arteriosus allow for this vital mixing. - Postnatally, a prostaglandin infusion to keep the ductus arteriosus open and sometimes an atrial septostomy are required to maintain this critically important mixing.

For children ages 0-4 years with recurrent wheezing triggered only by respiratory tract infections, the recommendation is =

treat acute viral illnesses with a short course (seven to 10 days) of twice daily ICS at symptom onset and a short-acting beta2-agonist (SABA) as needed. - This contrasts with the prior recommendation of as-needed SABA use with consideration for oral steroids based on illness severity

Think of midline defects for what syndrome ...

trisomy 13

A pregnant mother brings her older child for his health supervision visit. She has questions regarding her recent abnormal ultrasonography findings. She is at 18 weeks of gestation and her prenatal ultrasonography this week showed intrauterine growth restriction, ventriculomegaly, nuchal thickening, choroid plexus cysts, cleft lip and palate, micrognathia, microphthalmia, rocker bottom feet, clenched hands with overriding fingers, short radial ray, and a cardiac defect. Maternal quadruple screening test results revealed decreased values for a-fetoprotein, estriol, human chorionic gonadotropin, and inhibin A. Maternal cell-free fetal DNA analysis is ordered and is notable for an aneuploidy. The sensitivity of this type of prenatal screening for aneuploidies is discussed with the mother. Of the following, the MOST likely result is

trisomy 18; 97% sensitive with diagnostic amniocentesis indicated - The prenatal ultrasonography findings of intrauterine growth restriction, ventriculomegaly, nuchal thickening, choroid plexus cysts, cleft lip and palate, micrognathia, microphthalmia, rocker bottom feet, clenched hands with overriding fingers, short radial ray, and a cardiac defect are highly suggestive of a trisomy 18 diagnosis. - A noninvasive way to screen for these disorders is through maternal cell-free fetal DNA (cfDNA) analysis. - Maternal noninvasive cfDNA analysis has been available for several years to screen for aneuploidies in pregnancy. It does not detect microdeletions, microduplications, unbalanced translocations, or single gene mutations. The high sensitivity and specificity of noninvasive cfDNA testing has led many health care professionals to believe incorrectly that this is a diagnostic test. An abnormal cfDNA analysis should always be confirmed with a diagnostic test such as an amniocentesis or chorionic villus sampling.

Tall girl. Dev'tal delay. Dx?

trisomy X

A 5-year-old child with nephrotic syndrome presents with a 1-week history of fever and a 2-day history of shortness of breath. Physical examination reveals temperature of 101°F, shift of trachea to the left, diminished vocal fremitus over the right mid and lower zones, dull percussion note, and diminished breath sounds over the same area. Chest X-ray shows shift of trachea and mediastinum towards the left and diffuse haziness over right mid and lower zones. Right-sided pleural effusion was suspected, and a pleural tap was done. Pleural fluid analysis shows: Xanthochromic fluid, specific gravity 1.025, proteins 4.0 g/dL, glucose 30 mg/dL (blood glucose 90 mg/dL), pH 7.25 (blood pH 7.4), and cells-600 cells/mm3 with 85% lymphocytes. Pleural fluid to serum protein ratio is 0.7. Pleural fluid to serum LDH ratio is 0.8. Pleural fluid to serum glucose ratio is 0.3. What is the most likely cause of pleural effusion in the child?

tuberculous pleural effusion - history of fever, which suggests an infective pathology - pleural fluid appears xanthochromic with a specific gravity of 1.025, and protein content of 4.0 g/dL suggests that the fluid is an exudate - glucose is reduced to 30 mg/dL (blood glucose 90 mg/dL), suggesting a bacterial infection - cytology of the fluid shows 85% of the cells are lymphocytes, which supports the diagnosis of tuberculous pleural effusion

subependymal giant cell astrocytoma is an uncommon tumor but is almost exclusively seen in ...

tuberous sclerosis

Your patient presents at 9 months of age with sudden onset of seizures. He has been ill with an ear infection and has a temperature of 102 degrees Fahrenheit. His height is at the 90th percentile, weight at the 50th, and a head circumference at the 50th percentile. His complete blood count (CBC), lactate/pyruvate, comprehensive metabolic panel, and urinalysis were unremarkable. He has good head control but is not yet sitting. The patient has a 6-year-old sister with behavioral issues, including attention deficit hyperactivity syndrome (ADHD). A maternal uncle has idiopathic epilepsy. The patient's maternal grandmother had a thoracic aneurysm in her 50's. In speaking to his mother in the emergency room, you notice small bumps around the cuticles of several of her nails that she has always had. What is the most likely diagnosis?

tuberous sclerosis - classified a neurocutaneous disorder (phakomatosis) - hallmarks of this condition include seizures with tubers visualized on brain imaging, cardiac rhabdomyoma, hypopigmented macules, facial and periungual fibromas, shagreen patches, and forehead plaques - autosomal dominant condition - often mildly affected individuals can go unrecognized into adulthood and are ascertained only after their symptomatic child is diagnosed - in addition, psychiatric/behavioral deficiencies are common, and given this history, it is possible this patient's sister is affected

occurs when the investigator rejects the null hypothesis when it is in fact true

type I error - probability of a type I error is typically known as alpha (α)

failing to reject the null hypothesis (accepting the null hypothesis) when it is in fact false

type II error - the probability of a type II error is known as beta (β) - The power of a study is defined as (1 - β)

Individuals with recent, documented SARS-CoV-2 infection (including those who are diagnosed after initiating a vaccine series) should have at least recovered from acute infection and met criteria for discontinuation of isolation precautions before receiving a vaccine dose. Additionally, given the low risk of reinfection soon after prior infection, it is reasonable for individuals with SARS-CoV-2 infection to wait to receive a vaccine dose how long after infection?

until three months after infection - This applies to receipt of any primary series or booster dose. - Potential reasons not to delay the vaccine dose in this population include high risk for severe infection, high rates of community transmission, and circulating variants associated with a high risk of reinfection.

16 yo comes to your office for help with managing GERD. She complains of heartburn, chest pain when eating or lying flat, frequent sore throat, bad breath, intermittent episodes of vomiting with occasional streaks of blood. She has completed at 6 week trial of a histamine blocker without any improvement in her symptoms. Her vitals, growth parameters, and PE are normal. What is the most appropriate next step in management of her GERD?

upper endoscopy with biopsy - most likely esophagitis via GERD

Affected in most cases of brachial plexus injuries ...

upper trunk of brachial plexus

Most common cause of significant hyperammonemia in infants is ...

urea cycle defect - not acidotic on presentation - without tx, as ammonia rises, pt will progress to respiratory alkalosis

You are asked to evaluate a 7-day-old male infant; he was born at term. He is the 3.5 kg product of an uncomplicated pregnancy, labor and unassisted vaginal delivery of a 27-year-old G2P1Ab0 woman; the patient presents with the sudden onset of vomiting, lethargy, and tachypnea. He is bottle-fed and was feeding vigorously until today. He has had no fever, rhinorrhea, cough, diarrhea, or abdominal distention. An older sister had the onset of similar symptoms at 5 ½ years of age, as did their mother, in both of whom these symptoms would recur episodically, usually with an intercurrent infection. A maternal uncle died in infancy with very similar symptoms; he also experienced seizures. Examination reveals a normal temperature, a pulse of 134, a blood pressure of 88/52 mm Hg, and a respiratory rate of 62. He is lethargic, but arousable, with tactile stimuli. The anterior fontanelle is slightly sunken. There is mild skin tenting and moderate hypertonia. The lungs are clear. There is no organomegaly or petechiae. A urine sample has a distinctive odor of cat's urine (ammoniacal). You immediately hospitalize the child and administer appropriate rehydration; you obtain blood, urine, and cerebrospi

urea cycle disorder - Generally, aminoacidopathies, disorders of carbohydrate intolerance and urea cycle defects tend to present in the neonatal period to infancy, while fatty acid oxidation defects, glycogen storage diseases, and lysosomal storage diseases tend to present in infancy to childhood. - Decompensation may be triggered by changes in diet or intercurrent infections, leading to an episodic recurrence or exacerbation of symptoms. These are features present in the above case, which is an example of ornithine transcarbamylase (OTC) deficiency, a urea cycle defect. - Urea cycle defects are inborn errors of metabolism due to a variety of enzymatic defects that hamper elimination of waste nitrogen and detoxification of ammonia, largely in hepatic mitochondria. They are characterized by episodes of severe hyperammonemia (often exceeding 1,000 mcg/dL), lethargy, vomiting, tachypnea, and neurologic abnormalities, including seizures, altered tone, cerebellar signs, and coma. - These disorders are inherited as autosomal recessive conditions, except for OTC deficiency, which is an X-linked dominant condition.

A 7-year-old girl presents with recurrent urinary tract infections. Ultrasonography of the abdomen shows hydronephrosis and hydroureter on the left side. Excretory urogram shows hydronephrosis and hydroureter on the left side with a round filling defect on the left side of the bladder. What is the most likely clinical diagnosis?

ureterocele - much more common in girls than boys and often presents as urinary tract infection - cystic dilatation of the terminal ureter due to the obstruction caused by a pinpoint sized ureteral orifice - in girls it often may be associated by ureteral duplication - can be ectopic or orthotopic - ectopic uretrocele extends submucosally into the urethra and may cause bladder outlet obstruction, leading to bilateral hydronephrosis - orthotopic ureterocels are commonly discovered while investigating for UTI

Preferred urine collection in 12 mo old ...

urethral cath

A male neonate was born at term 3 days ago via cesarean delivery because of failure to progress. His 27-year-old mother reports no significant medical history. She recently moved from out of state and her prenatal records are not available. The neonate's birthweight is 3.9 kg, length is 53 cm, and head circumference is 35 cm. He has been taking 80 to 90 mL of formula every 2 hours. His temperature is 38°C, heart rate is 147 beats/min, respiratory rate is 61 breaths/min, and blood pressure is 65/46 mm Hg. Physical examination is notable for mild jitteriness of the arms and legs at rest, a diaper rash, and excoriations on the face. Of the following, the test MOST likely to diagnose this neonate's condition is

urine drug screen - Neonate displays evidence of opiate withdrawal with low-grade fever, mild tremor, and facial excoriation. Because these signs and symptoms of neonatal abstinence syndrome (NAS) began on day 3 after birth, this neonate is most likely withdrawing from a long-acting opiate such as methadone. - Urine drug screening is commonly used to test for intrauterine drug exposure. This testing only reflects exposure in the preceding 3 to 5 days.

A 14-year-old girl presents with menstrual irregularities; there is an unexplained absence of the last four consecutive menstrual cycles. Since menarche at age 12, she has had occasional irregularities, but this is a much longer absence. She states she is told frequently that she should eat more; she feels that she is just careful with her diet. Her mother adds that pregnancy is not possible because she takes good care of her daughter, who is fully focused on her studies and not going to parties. Physical examination finds a lower blood pressure and a body weight 25% below normal. What laboratory is the most useful for monitoring improved eating habits in this patient?

urine ketones - restricting type of anorexia nervosa - Monitoring urine ketones can help to detect the failure of the therapeutic partnership with the patient and the continuation of fasting - Rapid and significant weight loss, hypokalemia, hypotension, and prerenal azotemia are indications for hospitalization. At weights >35% below normal, sudden death may occur.

A 5-year-old male patient presents with sudden onset of a stumbling gait following a 3-day history of a spreading "itchy" rash. His mother notes that the rash began as red "bumps" but has progressively changed to fluid-filled blisters. He had a low-grade fever, abdominal pain, lethargy, and a headache a few days ago. The anxious mother also notes slightly slurred speech. He has had no known exposure to sick children. On physical exam, the patient has a clumsy wide-based gait and difficulty articulating certain words. He has numerous vesicles with a cloudy fluid over the entire body, with a few scabbed-over lesions and numerous erythematous papular lesions. Temperature 99.4°F. The patient has not been followed by a healthcare provider and is not current with childhood immunizations. What is the most likely etiology of these findings?

varicella - presents initially as erythematous macules that develop quickly into papules and thin-walled vesicles surrounded by an erythematous base - will have successive crops of lesions that scab over in 5-7 days with occasional residual scarring - mucosal involvement of the oropharynx and vagina is common. Marked pruritus may be seen. - The incubation period is 14-21 days, and the prodrome consists of malaise, fever, and headache.

A 1-day-old neonate born to a 24-year-old mother with varicella (chickenpox) is being evaluated in the newborn nursery. Her mother developed pruritic vesicular skin lesions 4 days before delivery. A swab from a skin lesion was positive for varicella-zoster virus on polymerase chain reaction testing. Maternal rapid plasma reagin and HIV antigen-antibody tests were nonreactive. The neonate was born at term via spontaneous vaginal delivery. After delivery, Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. Her birthweight was 3.1 kg. The neonate's physical examination findings are normal. Of the following, the BEST next management step for this neonate is administration of

varicella-zoster immune globulin - This asymptomatic neonate is at risk for severe neonatal varicella infection because her mother was diagnosed with varicella between 5 days before and 2 days after delivery. - Neonatal varicella occurs when the mother develops infection around the time of delivery (perinatal transmission), with the risk to the neonate being greatest when maternal varicella infection occurs 5 days before through 2 days after delivery. In this setting, neonatal disease usually manifests between 5 and 10 days after birth and can be life-threatening (eg, severe pneumonia, disseminated disease) with a case fatality rate of approximately 20%. - As postexposure prophylaxis, administration of VZIG to high-risk susceptible neonates prevents symptomatic infection in approximately 50% of cases and decreases disease severity in those neonates who develop varicella infection. Varicella-zoster immune globulin should be administered to neonates with high-risk perinatal exposure as soon as possible. - Varicella-zoster immune globulin is maximally effective when given within 96 hours but may be given up to 10 days after exposure.

A female newborn is evaluated in the neonatal intensive care unit. The pregnancy was complicated by intrauterine growth retardation. Prenatal ultrasonography showed a single kidney on the right side and vertebral segmentation defects throughout the thoracic and lumbar spine. A few minutes after birth, the neonate experienced respiratory distress that necessitated intubation. She has anal atresia and no right thumb. A nasogastric tube curls up in the esophagus, and radiographs suggest a tracheoesophageal fistula and show the vertebral defects. Of the following, the additional birth defect(s) MOST likely found in this neonate is (are)

ventricular and atrial septal defects VATER/VACTERL association - nonrandom occurrence of two or more congenital malformations that occur together more often than can be explained by chance - coined in 1972, encompassing vertebral defects (V), anal atresia (A), tracheoesophageal fistula (TE), and renal defects (R) - over the next 2 years, researchers proposed expanding the abbreviation from VATER to VACTERL, adding cardiac defects (C) and limb defects (L) - at least three of the above-mentioned congenital malformations must be present to arrive at a diagnosis of VACTERL association - the neonate in the vignette has vertebral defects, anal atresia, tracheoesophageal fistula, renal aplasia (single right kidney), and limb defect (absent thumb) - cardiac malformation, most commonly ventricular and atrial septal defects, can be seen in VACTERL association

During physical altercation after high school football game, 15 yo boy suddenly falls to ground after being hit in chest with closed fist. Within <1 minute, 1 of the football coaches and a trainer being CPR after noting that the boy was unresponsive. Resuscitation efforts continue during transport to the ED. Pt never regains consciousness, and he dies within 1 hour of injury. Most likely cause of pt's sudden death?

ventricular fibrillation - commotio cordis - sudden increase in intracardiac pressure

An 11-year-old boy presents with a 3-month history of extreme itching of both eyes and increased tearing. His mother tells you that he was treated with antibiotic eye drops 2 months ago; there was no resolution of the symptoms. She also states that the child had similar symptoms 1 year ago. There is a family history of atopy. On examination, you find large, flattened, cobblestone-like papillary lesions of the palpebral conjunctivae; there is a milky conjunctival pseudomembrane. A conjunctival exudate is present. A smear of the exudate reveals many eosinophils. What is the most likely diagnosis?

vernal conjunctivitis - bilateral recurrent inflammation of the conjunctiva that usually occurs during warm weather - occurs mainly in the 5 to 20-year-old age group, affecting boys more commonly than girls - usually a family history of atopy - cobblestone papillae with milky exudates are characteristic, as is the finding of eosinophils on the smear

False positive ANA's are common in pediatric population with respect to...

viral infection

For children ages 48-59 mos, what vision requires referral to eye specialist?

visual acuity worse than 20/40

Child has macrocytic anemia, (mean corpuscular volume [MCV] >80 fL),with pallor and tachycardia on physical examination. Macrocytic anemia is most consistent with a folate or vitamin B12 deficiency. Neurological findings of loss of deep tendon reflexes makes what more likely?

vitamin B12 deficiency - A strict vegetarian diet with a lack of any animal fats, the most common source of vitamin B12, significantly increases the risk. - It is important to counsel vegetarian families on how to ensure adequate intake of vitamin B12, especially mothers of breastfed infants. - Vitamin B12 deficiency should be considered in children with nonspecific symptoms (including generalized symptoms of fatigue, weakness, poor appetite, irritability), and those with failure to thrive. - Neurological findings of B12 deficiency may include hypotonia, ataxia, seizure activity, and loss of deep tendon reflexes.

Recognize metaphyseal flaring, which is associated with...

vitamin D deficiency - Vitamin D deficiency may present with skeletal abnormalities caused by rickets (genu varum or valgum, costochondral beading, and metaphyseal widening, particularly in the wrists)

A 4-year-old boy presents to your office for further evaluation. He has had a history of frequent nosebleeds since the age of 1. His father has a similar history but has not sought medical evaluation. There is no history of hospitalization or surgery. The child was treated with iron for a diagnosis of anemia when he was 15 months old. On physical exam, the child looks well and is very active. The only abnormal finding is dried blood in both nares. There is no rash, no organomegaly, and no pallor. What is the most likely diagnosis?

von willebrand - one of the most common hereditary bleeding disorders - inherited as an autosomal dominant trait - caused by underproduction of the von Willebrand protein, or by synthesis of a dysfunctional protein = present with mucocutaneous hemorrhage, menorrhagia, prolonged oozing from cuts, and increased postoperative bleeding

A 24-month-old typically developing girl is seen for a routine health supervision visit. She can jump, kick a ball, and throw a ball overhand. Of the following, she is MOST likely to also be able to

walk down stairs while holding the rail - The 2-year-old, typically developing girl in the vignette is most likely able to walk down stairs while holding a hand rail. - Two-year-old children typically walk down stairs by placing both feet on each step, rather than alternating their feet. - The typical 24-month-old can jump, kick a ball, throw a ball overhand, and walk down stairs holding the rail, placing both feet on each step.

Hodgkin's lymphoma associated B symptoms ...

weight loss >10%, night sweats, fevers

Creatinine is a popular nutritional supplement used in what sports?

weightlifters sprinters hockey players wrestlers offensive and defensive linemen shot putters

What age group has highest protein requirement?

younger infants = 1.5 - 2 g/kg/day - protein requirements gradually decrease over time - by age 6 months, protein requirements are down to 1.5 g/kg/day - by early childhood and adolescents undergoing growth spurt, protein requirement decreases to 1 g/kg/day - adults = 0.8