Pediatrics

12 y/o boy has been performing poorly in school recently. Mother caught him talking to a wall. Says he's hearing voices. Speech is slurred, with occasional drooling. Slight tremor present. Labs: Elevated ALT/AST. Dx?

Wilson's Dx! Suspect in kid with abnormal LFTs, personality changes, psychosis, and tremor.

Mode of inheritance of Duchenne and Becker muscular dystrophies?

X-linked recessive (affect males)

Tinea capitis

a "dermatophyte" infection of scalp, common in African American kids. Causes scaly patches on scalp. Tx: Oral Griseofulvin or Terbinafine

Sturge-Weber Syndrome

a nurocutaneous disorder, associated with capillary malformation (port wine stain), along trigeminal nerve distribution Leptomeningeal capillary-venous malformation in brain Seizures, MR

Meniere's disease

accumulation of fluid in inner ear, leading to hearing loss, vertigo, and tinnitus

Rare complication of infectious mono?

acute airway obstruction S+S: throat tightness, difficulty swallowing. Patients develop severe tonsillar enlargement, experience difficulty swallowing, and have labored breathing. Tx: stat corticosteroids.

Gene defect in SCID

adenosine deaminase deficiency

Mullerian agenesis?

aka Mayor-Rokantansky-Aschoff Syndrome Female with 46 XX genotype, and looks female (phenotype), but had failure of Mullerian ductal system to differentiate into: Fallopian tubes, Uterus, and vagina.

Kartagener syndrome

aka Primary ciliary dyskinesia MOA: mutation in ciliary dynein arms, causes dysmotile cilia and poor mucociliary clearance H/P: dextrocardia (PMI of apex displaced to right side of chest), recurrent sinusitis, bronchiectasis

Helmet cells

aka Schistocytes fragmeneted RBCs seen in traumatic microangiopathic hemolytic anemia, DIC, HUS, TTP

Nursemaid's elbow

aka radial head subluxation fix: 1. hyperpronation of forearm 2. supination with elbow flexion

ACE inhibitors

allow for peripheral vasodilation, decreasing afterload and LV remodeling Diuretics (FTBS) will decrease preload

Disinhibited social engagement disorder

another outcome of early neglect. unhesitant approach to unfamiliar adults/strangers.

When to admin. meningococcal vaccine?

at 11-12y/o, followed by a booster at 16 due to risk of college breakouts

Complement deficiency

at increased risk of disseminated encapsulated bacterial infections

Breastfed infants have decreased risk of?

decreased risk of: 1. otitis media 2. GI and resp. infection 3. UTIs 4. Nec. enterocolitis 5. DM Type 1 6. childhood cancer

Standing does what for HCM?

decreases preload and increases intensity of murmur.

HyperIGM Syndrome

defect in CD40 ligand, causes: High IGM, low IgG, and IgA S+S: severe, and recurrent sinopulmonary infections MOA: CD40 ligand is present on T cells, and binds to CD40 on B cells, allowing B cells to class switch from IgM to other immunoglobulins.

Cause of Marfans syndrome?

defect in Fibrillin 1 gene mutation.

MOA of SCID

defective T-cell development, leading to severe B-cell maturation. Hallmark: extremely low lymphocyte count Tx: stem cell transplant

Lesch-Nyann Syndrome

deficiency in HGPRT leads to accumulation of Hypoxanthine and Uric acid in serum and CNS. Dev. delay hypotonia chorea dystonia self-mutil/spasticity

infant with N. meningoccemia likely to die bc?

develop Waterhouse-Friderichson syndrome, causing adrenal gland hemorrhage, and vasomotor collapse. Causes severe hypotension, sepsis, skin rash. 100% mortality

Inguinal hernias

does NOT transilluminate Bowel becomes trapped?

Erythema multiforme

erythematous target lesion with dusky center, caused by HSV1/2

Streptococcal perianal dermatitis

erythematous, "sharply demarcated" perianal rash, associated with pruritis, and pain.

Malignant l. nodes

firm, immobile, larger than 2cm

Tx. of Ingestion of Clostridium botulinum spores?

infantile botulism, caused by C. botulinum spores, colonize gut and lead to release of neurotoxin. Causes life-threatening, descending flaccid paralysis. S+S: ptosis, constipation, hypotonia. Tx: Human-derived botulism immune globulin

Common complication of prematurity/LBW infants?

intraventricular hemorrhage

Transient synovitis

joint pain and decreased ROM, but no fever or significant increase in WBC, ESR/CRP Tx: just supportive care, bed rest, and NSAIDs

Preseptal cellulitis

just eyelid swelling, erythema

Pheylketonuria deficiency of?

lack of: Pheylalanine hydroxylase, causes buildup of Phenylalanine. S+S: M.R., fair skin, eczema, "musty odor" Diagnostic test: Quantitative amino acid analysis Tx: Treat with low-Phenylananine diets

Chlamydial conjunctivitis

less severe S+S: watery, serosanguineous, only mildly purulent DC. Tx: Oral Azithromycin

Cause of edema in patient with Turner's Syndrome?

lymphatic network dysgenesis TQ: Pts develop congenital lymphadema due to dysgenesis of the lymphatic system. Dysfunctional lymphatic system causes accumulation of protein-rich interstitial fluid in hands, feet, neck--->cystic hygroma (severe obstruction of lymphatic vessels)

Common cause of infertility in males with CF?

males with CF have obstructive azoospermia, due to bilateral absence of Vas Deferens, causing infertility.

Cherry hemangioma

most common benign capillary tumors in adults

PSGN

most common cause of NEPHRITIC syndrome in kids. Look for: HTN, hematuria, and RBC casts

Selective IgA deficiency

most common primary immune deficiency. Presents with recurrent sinopulmonary and GI infections, as well as Atopic issues (eczema, etc.) Labs: low IgA, normal IgG, M, D, E. Hallmark: production of Anti-IgA antibodies causes anaphylaxis during a blood transfusion.

3 week old boy comes to ER for seizures. Has a 4 minute tonic clonic seizure. Has a full anterior fontanelle, and brain imaging shows edema, and hemorrhage of left temporal lobe, with no intracranial calcifications. Vertical transmission of what?

neonatal HSV causes encephalitis, seizures, temporal lobe hemorrhage, Tx: Acyclovir

Affects of "transplacental maternal estrogen exposure" in newborn?

newborn can have mammary gland enlargement, vaginal discharge, swollen labia. Tx: just observe and routine care

Howell-Jolly Bodies?

nuclear remnant within RBCs, typically removed by spleen. Presence suggests Hyposplenism.

Congenital Rubella Syndrome S+S?

often occurs through First-trimester maternal-fetal transmission of Rubella, in unvaccinated mom S+S: cataracts, PDA, sensoneural hearing loss Best prevented by vaccination prior to conception.

What do patients with just Galactokinase deficiency present with?

only cataracts

Viral conjunctivitis

viral conjunctivitis is most commonly due to Adenovirus S+S: Pts present with viral infection, followed by 1-2 weeks of watery/mucoid eye DC and redness. Note: Viral conjunctivitis only lasts about 1-2 weeks, no more.

HCM gets worse with?

worse with standing, Vlasalva,

Serum sodium in patients with D.I.?

would be high (hypertonic serum, with dilute urine)

Causes of Viral myocarditis?

Coxsackie B Adenovirus Influenza CXR: shows cardiomegaly

Osteosarcoma

"Sunburst"/Codman's triangle. Most common primary bone tumor in kids.

Osgood-Schlatter disease aka?

"Traction apophysitis of tibial tubercle" Radiographic findings show: Anterior soft tissue swelling, lifting of tubercle from shaft, and irregular fragmentation of tubercle.

Osteoid Osteoma

"central nidus" of lucency. Pain worse at night, but quickly better with NSAIDs

PDA

"continuous flow murmur/machine-like murmur"

VSD

"holosystolic murmur at left lower sternal border"

ASD

"wide, fixed"split to S2)

Best way to diagnose ALL?

ALL is the most common cancer in kids, usually between 2-5 years old. S+S: petechiae, pallor, anemia, and thrombocytopenia, due to bone marrow infiltrate. Bone marrow biopsy required to confirm dx. of ALL. Presence of >25% lymphoblasts is diagnostic of ALL.

Complications of ARPKD?

ARPKD manifests in infancy, as LARGE bilateral flank masses. Also causes pulmonary hypoplasia and causes respiratory distress due to oligohydramnios. Potter facies (flattened ears, nose, jaw) bc of oligohydramnios

Acute Tubular Necrosis

ATN occurs in patients with Acute Kidney Injury, from ischemia/nephrotoxicity Labs: "Muddy Brown" Granular casts, hematuria, and Renal Tubular epithelial cells

4 month old with Macroglossia. P.E. shows enlarged tongue, and a reducible umbilical hernia. High Right UEX and LEX are larger than his Left extremities. Next best step in management of this pt?

Abdominal Ultrasound TQ: Pt has Beckwith-Wiedmann Syndrome S+S: Macrosomnia, Macroglossia, umbilical hernia/omphalocele, and hypoglycemia TQ: Must do an Abdominal U.S. to moniter for pts risk of developing Wilms Tumor

3 day old boy brought to ER for worsening SOB, fever, cough. Recently had lots of diarrhea from Giardia infection. Also had lobar pneumonia, requiring hospitalization, and numerous ear infections, treated with antibiotics. P.E. shows "small tonsils." Cause?

Abnormal B lymphocyte maturation TQ: X-linked Agammaglobulinemia (Bruton's) occurs due to failure of B cell development. P.E. "small tonsils," with absent lymphoid tissue, causing recurrent sinopulmonary and GI infections.

Cause of Achondroplasia?

Achondroplasia is due to defect in Fibroblast growth factor receptor 3 (FGFR3) gene mutation. Inhibits Endochondral bone formation (long bones)

3 hour old boy has cyanosis. Cyanosis worsens at rest, and he turns pink with crying. Cyanosis also worsens when feeding. Dx?

Choanal atresia should be suspected in a newborn with cyanosis that worsens with feeding/rest, and relieved by crying. Cause: congenital nasal malformation of the posterior nasal passage to canalize completely.

12 y/o girl comes to office complaining of Left sided ear discharge for past 3 weeks. Completed 2 courses of antibiotics so far. Complains of hearing loss. Otoscopy shows granulation tissue and skin debris in left ear. Dx?

Cholesteatoma Hallmark: new-onset hearing loss, chronic ear drainage, and granulation tissue/skin debris

52 hour girl has bilious N/V and abdominal distension. She has not yet passed meconium. No stool in rectal vault, but has normal tone. Has loops of small bowel, with paucity of air in large intestine and rectum. Viscous meconium is irrigated and evacuated primarily from Ileum. What comorbidity is she gonna develop?

Chronic rhinosinusitis TQ: Kid has Meconium Ileus (NOT Hirschsprung Disease, bc of her normal rectal tone) Meconium illeus, commonly occurs in Ileum, as thick meconium plug plugs up illeum. Clue that pt has CF, and at risk for recurrent sinopulmonary infections

Turner's Syndrome is associated with what heart defect?

Coarctation of the Aorta Diminished LEX pulses, with UEX HTN.

Neonates are at increased risk for?

Communicating (nonobstructive) hydrocephalus (IVH) MOA: accumulation of blood irritates Arachnoid villi, and impairs absorption of CSF.

7 y/o boy brought to ER after FOOSH. He has a broken humerus. On exam, the arm is swollen, pale, and edematous. Radial pulse is intact. Continues to complain of more and more pain, despite being given lots of pain meds. Reason?

Compartment syndrome TQ: common complication of Supracondylar humerus fractures is Compartment Syndrome. Causes severe: pain pallor poikilothermia and paresthesias, and later on causes pulselessness and paralyis.

Most common heart defect in Down Syndrome?

Complete AVSD Cause: failure of endocardial cushions to merge (Note: Endocardial cushion defect is an AVSD)

Second most common posterior fossa tumor in kids?

Medulloblastoma occur in Cerebellar vermis, which is important for gait and balance. S+S of Medulloblastoma: truncal ataxia, gait instability, and given the proximity to the fourth ventricle, can cause obstructive hydrocephalus with signs of increased ICP (papilledema, N/V).

S+S of Medulloblastomas?

Medulloblastomas arise from Cerebellar vermis, and cause Truncal ataxia and truncal instability due to hydrocephalus and compression of Cerebellum.

14 year old girl with facial puffiness and pretibial edema, has Heb B infection. Cause of facial puffiness?

Membranous nephropathy TQ: common cause of Nephrotic syndrome (edema, facial puffy, proteinuria, and hypoalbuminemia), which occurs in pts with active Hep B infection.

Cause of Mesenteric Adenitis?

Mesenteric adenitis is an inflammation of the mesenteric lymph nodes in the abdomen. It can be caused by the bacterium Yersinia enterocolitica. If it occurs in the right lower quadrant, it can be mistaken for acute appendicitis, often preceded by a sore throat.

What is prescribed to opiate addicted preggos?

Methadone (mu-opiod analog, to prevent withdrawal S+S in mother and fetus.

Reye Syndrome liver biopsy would show?

Microvesicular fatty infiltrate Microvesicular steatosis on liver biopsy, leading to fulminant hepatic failure and encephalopathy

What kind of ear infection does Moraxella catarrhalis cause?

Middle ear infection (otitis media)

1 month old boy, comes to office for painless blood streaked stools. He has been exclusively breastfed since birth. Breastfeeds every 2-3 hours, but regurgitates a large amount of breast milk with every feed. He has eczema. Fecal occult blood test is positive, Dx?

Milk protein-induced enterocolitis TQ: Babies can have milk/soy protein allergy, a condition exclusive to infants. Causes eczema, and painless bloody stool. Tx: Mom must eliminate all dairy and soy from diet. Formula fed infants should switch to hydrolyzed formula (containing predigested proteins) Note: parents can be reassured that infants will be able to tolerate dairy and soy by 1 y/o.

Common complication of Rheumatic fever?

Mitral valve stenosis

When to use Modafinil?

Modafinil is a wakefulness-promoting agent, used to treat Narcolepsy

Lymphadenopathy in Mono?

Mono has Posterior cervical lymphadenopathy

Lead poisoning

Must confirm with Venous lead level Tx: 1. Succimer for kids 2. EDTA and Dimercaprol for adults, OR for emergency situations (Acute Encephalopathy)

Treatment of Right eye esotropia (strabismus)

Must patch the good eye (left eye), to force use of Right eye. Otherwise, right eye is at risk of Amblyopia (vision loss due to disuse)

Side effect of Hydroxyurea?

Myelosuppression (Neutropenia, anemia, thrombocytopenia)

Antidote for Acetaminophen (Tylenol) O.D.?

N-acetylcysteine (restores Glutathione, which will breakdown Acetaminophen's toxic metabolites (NAPQ1) into nontoxic form.)

18 month old brought to ER for fever, vomiting, and lethargy. Develops rash on LEX, and nonblanching pinpoint rash on trunk and LEX. +Brudsinki sign. Organism? (Strep. pneumo not listed)

N. meningitis N. meningitis is the #2MCC of bacterial meningitis in kids 3 month-10 years old (after Strep. pneumo) Key: The risk of acquiring meningococcal disease is x10 higher in kids <2y/o, compared with older kids.

Lisch nodules

NF1 (peripheral nerve sheath tumors) cafe-au-lait macules, neurofibromas (aka peripheral nerve sheath tumors) Lisch nodules

Complications of NF1?

NF1 is Aut. dominant mutation in NF1 (tumor suppressor) gene., which codes for: Neurofibromin on: Chromosome 17 S+S: cafe au lait spots neurofibromas Lisch nodules optic gliomas (compress CN2) and lead to decreased vision/proptosis.

30-minute old baby boy presents with cyanosis in NICU. Was born at 28 weeks gestation. Mom got steroids and Mg2+ Sulfate shortly after onset of labor. P.E.: grunting, intercostal and subcostal retractions, nasal flaring, and shallow respirations. Diminished bilateral breath sounds. Key: CXR: shows diffuse, fine, reticular granularity and perihilar linear opacities. Dx?

NRDS Suspect in infants with grunting, flaring, and retractions after brith. Cause: decrease surfactant Tc: Positive air pressure ventilation and surfactant

2 y/o boy has a "barking cough" with inspiratory stridor, and was given Oral Dexamethasone. He continues to develop intercostal retractions and inspiratory stridor at rest. Treatment?

Nebulized Racemic Epi Croup (Laryngotracheitis) is due to Parainfluenza virus infection. First admin. corticosteroids (Dexamethasone), and if stridor continues at rest, admin. immediate Racemic Epi.

Workup of neonatal bilious emesis?

Neonatal bilious emesis signifies a SBO. Requires Contrast enema studies to determine the level of obstruction, and decide if it's a Meconium Illeus or Hirschsprung's Disease.

What cells do Neuroblastomas arise from?

Neural crest cells Neuroblastomas arises from neural crest cells (precursors of SNS chain and Adrenal Medulla) Labs: Elevated HVA/VMA

1st line agents for smoking cessation?

Nicotine replacement therapy (gum/patch), Bupropion, Varenicline Note: Varenicline can increase risk for suicide Bupropion cannot be given to patients who have eating disorders bc it lowers seizure threshold.

Can preggos get Varicella vaccine?

No! Varicella vaccine is made from live virus. C.I. in preggos and neonates <1y/o. Post-exposure prophylaxis in these patients would be the Varicella Immunoglobulin.

7 y/o boy with severe facial acne, 98th percentile height, coarse pubic and axillary hair, high bone age. Baseline levels of LH are low, and do NOT increase once GnRH agonist is given. Dx?

Nonclassic CAH, due to 21-OH deficiency causes excessive circulating Androgens to shut down GnRH, and LH/FSH. High peripheral androgens causes precious puberty and secondary sexual characteristics.

5 y/o old with edema, fatigue, abdominal pain. Has periorbital edema, and pretibial edema, as well as Proteinuria. What would be found on kidney Biopsy?

Normal findings Child has Minimal Change Disease (A Nephrotic Syndrome). Will show NORMAL renal architecture. Hallmark of MCD: effacement of podocytes. Tx: Steroids

Tx of Group A Strep Pyogenes (Strep throat)?

Penicillin and Amoxicillin

Description of Pericardial effusion (Pericardial tamponade)?

Pericardial effusion/tamponade can occur within days/months after cardiac surgery, and is called "postpericardiotomy syndrome." Causes life-threatening accumulation of fluid around heart: S+S: "Distant heart sounds," hypotension from low cardiac output, JVD. Tx: pericardiocentesis

12 y/o boy with "pins and needles" sensation in both feet. Ten days ago, had a febrile diarrheal illness. P.E. shows symmetric flaccid paralysis, absent DTRs, and decreased superficial touch and vibratory sense in both legs. What is being impaired?

Peripheral nerves GBS, occurs with ascending polyneuropathy, after a recent GI/URT infection. MOA: demylination of peripheral motor nerves.

13 y/o boy has 2 weeks of rhinorrhea, followed by week of irritating dry cough. Coughing worsened, occurs without warning, and he's vomited 2 times. P.E. shows nasal congestion, and subconjuctival hemorrhages. How to confirm dx?

Pertussis PCR testing TQ: Pertussis is suspected in patients with irritating "paroxysmal" cough and "postussive emesis"

Most common brain tumor in kids?

Pilocytic astrocytoma Can cause seizures, weakness, sensory changes.

16 y/o girl developed pruritic rash on her back 5 days ago. Since then, she developed multiple new lesions on chest and back. There are multiple erythematous macules with minimal scaling on chest, and a larger erythemaous patch on upper back with scaling. Spares palms and soles.

Pityriasis rosea Solitary herald patch followed by clusters of small lesions in a Christmas tree patters. Self-limited

Long-term complication of VUR that is left untreated?

Renal scarring TQ: VUR can cause recurrent UTIs, which lead to Pyelonephritis and Renal scarring. TQ: For all kids <2y/o, with first febrile UTI, must do a Renal U.S. to look for anatomical abnormalities. Kids with recurrent febrile UTIs then need a VCUG to actually evaluate for a VUR

White eye reflex

Retinoblastoma

"White reflex" in Left eye clue for?

Retinoblastoma (Leukocoria) causes white pupillary reflex.

4 day old girl has bilateral mucopurulent DC of eyes. What tx. would have prevented this?

Topical Erythromycin for infant TQ: Gonococcal conjunctivitis in untx. mothers causes mucopuruluent bilateral DC in baby about 2-5 days later. Can ulcerate, scar, and blind. Tx: Topical Erythromycin ointment at birth.

Treatment of Gonococcal conjunctivitis

Topical Erythromycin ointment

4y/o boy presents with rash. Developed bumps on left arm, which became painful. Covered in thick-golden-yellow crusts. Tx?

Topical Mupirocin TQ: Non-Bullous impetigo occurs due to S. aureus/GAS. pyogenes. Hallmark: painful pustules and honey-colored crusts Tx: Topical Mupirocin

1st line treatments of Tinea corporis

Topical antifungals (Clotrimazole, Terbinafine)

2 hour old infant has worsening Cyanosis. Normal first heart sound, with a single, LOUD S2, no murmur. Cause of infants cyanosis?

Transposition of Great Vessels #1MCC of congenital cyanosis. Presents as cyanosis in first few hours of life, with Single, Loud S2 heart sound.

Imaging choice for gynecological problems?

Transvaginal pelvic U.S.

Vasovagal syncope

Triggers: prolonged standing, physical or emotional stress H/P: Presyncope (lightheadedness, pallor, diaphoresis) followed by immediate return to baseline after event.

Cause of Fragile X syndrome?

Trinucleotide repeat expansion of FMR1 gene.

Renal angiomyolipomas are associated with?

Tuberous Sclerosis

HYPOpigmented macules?

Tuberous sclerosis (Ash-leaf spots)

IgE mediated HSN reaction?

Type 1 (immediate) HSN reaction, caused by IgE. Causes: Anaphylaxis, Uticaria

Evaluation of kid with suspected UTI?

UA, Urine culture, and Straight Urethral catheterization (to obtain a sterile specimen not contaminated by stoole/skin)

Cause of UTIs in women

UTIs most commonly arise from bacteria ascending into the bladder from the vaginal introitus

1 month old girl brought to office for newborn exam. She has 4 skin creases along left inner thigh, and 1 crease on right thigh. When supine with knees flexed, left knee appears inferior to right knee. Next best step?

Ultrasound of Hips TQ: this patient has Developmental dysplasia of hip Hallmarks: asymmetrical thigh creases, and apparent leg-length discrepancy Tx: Get a Hip U.S. and put pt in a Pavlik harness

hallmarks of acute otitis media

bulging tympanic membrane, and pneumatic insufflation (immobility of TM)

5-Alpha-reductase deficiency?

cannot convert Testosterone--->DHT (via 5-alpha reductase) Males with 46 XY genotype, male internal genitalia, but undermasculinized external genitalia (at birth), so raised female. Then, at puberty, start to "turn male," due to more Testosterone (increased phallus size, deepening of voice, muscle growth)

Fanconi's anemia

caused by "chromosomal breaks" on genetic analysis Causes Aplastic Anemia (progressive bone marrow failure) and Thrombocytopenia, and Neutropenia S+S: short stature, abnormal thumbs, deafness, infections, bleeding gums

Erythema infectiosum

caused by Parovirus B.19, causes classic "slapped cheek" appearance

S+S of Neonatal Abstinence syndrome?

caused by infant's withdrawal to opiates S+S: irritability, high pitched cry, poor sleeping, sneezing, poor feeding, N/V.

Bordatella pertussis?

causes "whooping cough" in 3 phases: catarrhal (mild cough only), paroxysmal (really bad cough), and convalescent Paroxysmal cough has severe coughing fits and posttussive emesis Tx: Should always have vaccinated them with Acellular pertussis vaccine

Juvenile myoclonic epilepsy

causes generalized seizures during adolescence, with myoclonic jerks, most prominent in first hours after awakening.

Glomus tumor

common, benign vascular tumor in nailbed (subungal). Sensitive to touch

Treatment of vaginal foreign body

commonly occurs due to toilet paper. Presents as foul-smelling vaginal discharge. Just irrigate with warmed fluid

Korsakoff Syndrome

complication of Wernicke encephalopathy S+S; Retrograde and Anterograde amnesia, with preserved long-term memory. Confabulation, apathy, lack of insight. Cause: Vit B1 Thiamine deficiency (in Alcoholics)

Metatarsus ADDuctus

congenital foot deformity, in which forefoot turns inward. In most cases, goes away

Constitutional growth delay

constitutional growth delay causes delayed growth spurt, bone age, and puberty. Eventually normalizes.

Breath holding spells

crying, followed by breath-holding, cyanosis, and LOC.

Complication of HSV1?

Eczema herpeticum is a complication of superimposed HSV-1 TQ: Eczema herpeticum is a primary HSV infection, associated with: Atopic dermatitis

Seborrheic dermatitis

scaly, oily rash of scalp

Hallmarks of Autism spectrum disorder

solitary play, lack of eye contact, don't response to name being called

Depression screening age?

start at 12y/o

Best long term treatment of SCID?

stem cell transplant defective T cell development inhibits B cell development. Increased infections with viruses, fungi (Candida thrush), and opportunistic pathogens (P. jirovecci). Also sinopulmonary infections (pneumonia, otitis media). FTT, chronic diarrhea

Tx of Croup

steroids (Dexamethasone) for mild cases of subglottic narrowing (Steeple sign), and Racemic Epi for stridor even at rest

Cause of SIDS

sudden death <1y/o. 1. should avoid second hand smoke 2. give pacifier 3. sleep in supine position 4. firm bedding 5. room sharing, without bed sharing

Tx of Hand foot mouth dx?

supportive care

Catalase + Bugs

1. S. aureus 2. Serratia 3. Burkholderia 4. Aspergillus 5. Nocardia

Atopic dermatitis

Eczema, affecting cheeks, scalp, trunk, extensor surfaces. Can cause severe pruritis. Clue: pts with atopic dermatitis have a family history of atopic disorders (asthma, allergic rhinitis)

Cause of pinworm infection?

Enterobius vermicularis, causes severe perianal itching, especially at night

9 y/o boy has burning sensation in chest after eating. Was given a PPI, but is not helping. Endoscopy shows circular rings, and thickened, linear furrowing of esophagus. Has history of eczema. Dx?

Eosinophilic Esophagitis Esophageal inflammation triggered by food allergens, frequently associated with patients who have eczema. Causes dysphagia, epigastric pain, N/V, food impaction. Esophageal biopsy will show circular rings, and +Eosinophils.

Kid has sudden onset difficulty breathing overnight. He is anxious, drooling, with inspiratory stridor. Most comfy when he sits upright, with his neck extended. Dx?

Epiglottitis (causes fever, drooling, tripod position) Cause: H. influenza type B is #1MCC

Newborn who chokes and coughs with first feeding. Had polyhydramnios.

Esophageal atresia with distal tracheoesophageal fistula

Innocent murmurs

Innocent/functional/physiological murmurs result from blood flow through a structurally normal heart. Common in kids at some point in their lives. Usually decrease with standing.

S+S of congenital Rubella?

Maternal-fetal transmission of Rubella is most teratogenic in 1st trimester. Triad: 1. Sensorineural hearing loss 2. PDA 3. Cataracts Also can have blueberry muffin spots

Side effects of Antihistamines (Diphenhydramine)?

used to treat allergic rhinitis Causes Anticholinergic side effects: (decreased PNS) S+S: dry mouth, tachycardia, urinary retention

Lennox-Gastaut Syndrome?

usually presents by age 5y/o Kid will have M.R. Severe seizures of varying type (atypical, absence, tonic clonic, etc.) EEG: shows "slow-spike wave pattern"

Celiac disease markers?

Anti-tissue Transglutaminase Antibody IgA levels

Breastfeeding decreases what cancer in mom?

Breast and Ovarian cancer

Sudden-onset respiratory distress in a toddler (fine before, playing with toys etc) = foreign body aspiration. Best intervention?

Bronchoscopy is indicated to remove the foreign body

First line tx. of Absence seizures?

1. Ethosuximide TQ: hallmark of Absence seizures: 3-Hz spike usually <20 seconds

1st line treatment of Absence seizures:

1. Ethosuximide #1 2. Valproic acid Hallmark: EEG shows 3 Hz wave discharges during episodes

First line for Migraine headaches?

1. NSAIDS, Acetomenophen 2. Sumitriptan and 100% oxygen (if NSAIDS don't work)

Removal of swallowed coin?

1. if patient swallowed coin, but is asymptomatic, can observe for 24 hours 2. if patient swallowed coin, but is coughing, vomiting, dysphagia, etc, must remove with Flexible Endoscopy

When can cow's milk be allowed?

12 months (before 12 months, cow's milk causes iron deficiency anemia.)

1 week old boy presents to ER with vomiting, poor feeding. BP is 40/24. He has sunken eyes, depressed Anterior fontanelle, and dry mucous membranes. Decreased skin turgor. What is increased in patient?

17-hydroxyprogesterone Pt has 21-OH deficiency, CAH. Low Aldosterone causes Salt wasting, and low Cortisol causes hypoglycemia, and hypotension.

Treatment of OCD?

1st line: SSRIs and CBT TQ: only give Clomipramine in treatment refractory cases!

When to vaccinate for Measles?

2 doses of vaccine 1st at age 1 2nd at age 4 Note: MMR is a live attenuated vaccine

Pt with CF is most likely to have deficiency in which coagulation factor?

2, 7, 9, 10, C, and S Key: Pts with CF develop pancreatic insufficiency, causing decreased Lipase. Lack of Lipase causes fat malabsorption, lack of fat soluble Vitamins A, D, E, K

When to give Rotavirus vaccine?

2-8 months old only, to avoid risk of severe dehydration from gastroenteritis. Cannot admin. past 2-8 months window!

DiGeorge Syndrome

22q11.2 deletion MOA: defective development of pharyngeal pouches Conotruncal cardiac defects Abnormal facies Thymic aplasia/hypoplasia Cleft palate Hypocalcemia

15 y/o girl has amenorrhea. Never menstruated. Also has hard time smelling stuff. Exam shows short stature, no pubic or axillary hair. Low FSH and low LH. Most likely karyotype?

46XX TQ: Kid has Kallman syndrome. (Hypogonadotropic hypogonadism), occurs bc of defect in GnRH. Low GnRH, low FSH, LH, and Estrogen. Failure of secondary sexual characteristics, hair, menses, and breast development. Also can't smell good. Key: Pts with Kallman syndrome still have normal 46XX/46XY karyotypes, and internal genitalia.

When to introduce pureed foods?

6 months

Apgar score <7?

<7 requires further eval. and resuscitation (Ex: Positive-pressure ventilation)

How long does Kawasaki fever need to last?

>5 days (CRASH Burn)

2 y/o kid comes in with easy bruising, nosebleeds, decreased activity over past week. He had an URT infection that was treated with antibiotics 2 weeks ago. He has petechiae over his extremities, markedly hypocellular bone marrow, decreased megakaryocytes, and decreased erythroid and myeloid cell lines. Dx?

Acquired Aplastic Anemia suspect in kids with pancytopenia, following drug intake, exposure to toxins, and viral infections. Results from injury to bone marrow. Bone marrow biopsy is essential to make dx. : shows profound HYPOcellularity with fatty infiltration.

Cause if AIN

Acute Interstitial Nephritis occurs from history of: medication use, Eosinophilia, abundant WBCs

Transient synovitis most commonly affects?

Acute pain, with restricted ROM of hip joint. No rash!

Treatment of infants born to moms with Hep B infection?

Administer both the Hep B immune globulin (passive immunization) and vaccine (active immunization), within 12 hours of birth

3y/o boy brought to ER with intermittent abdominal pain. Had gastroenteritis several weeks ago. In ER, has nonbilious, nonbloody N/V, and +fecal occult blood. Tx?

Air enema Kid has intessusuption--->telescoping of ileum into cecum due to prior gastroenteritis causes a "lead point" at ileocecal junction. MOA: Ileum telescopes into cecum--> compresses blood vessels, causing ischemia--->rectal bleeding with "red currant jelly" stoole. Treatment of "lead point/target sign" is with prompt reduction with air enema.

11 month old girl comes to ER with rash. Rash started on face, and has now spread to entire body. Has fever, cough, rhinorrhea, and pink eye. Exam shows a blanching, maculopapular erythematous rash from face to body, sparing palms and soles. Best way of preventing spread of rash to others in ER?

Airborne precautions TQ: Measles presents with: Cough, coryza, conjunctivitis, and rash that starts on face, and spreads to body. Best way to prevent spread is airborne precautions (droplet isolation), and to have healthcare workers wear N95 face mask.

Post-exposure prophylaxis of Varicella in healthy child >1y/o?

All non-immune, asymptomatic, healthy patients >1y/o with Varicella exposure should get the Varicella Vaccine

When to test for Chlamydia/Gonorrhea?

All sexually active women <24y/o MUST be screened for Chlamydia/Gonorrhea, using Nucleic Acid Amplification testing (NAAT) = BEST bc very sensitive and specific.

Allergic conjunctivitis

Allergic conjunctivitis is an IgE-mediated HSN reaction to allergens S+S: BILATERAL watery/clear DC, itchy, red eyes

Tx of Anyphalactic shock?

Anaphylaxis occurs due to severe allergic reaction. Foods are most common trigger (Ex: allergy to sesame seed oil). Tx: IM Epi

Anemia of prematurity

Anemia of prematurity is the most common cause of anemia in infants. Occurs due to diminished EPO levels, shortened RBC lifespan, and blood loss. Pretty Asymptomatic. Labs may show decreased H+H, but normocytic, normochromic RBCs.

13 day old infant has runny nose, cough, and fussiness. She has a sibling in day care, where other kids have been ill. Temp is 100, and infant is crying with bilateral wheezes and crackles. What is a complication of her illness?

Apnea TQ: "bilateral wheezes" is clue that baby has Bronchiolitis (due to RSV). TQ: Neonates with RSV are at risk for apnea and respiratory failure.

Most common complications of Mumps?

Aseptic meningitis and Orchitis

6 y/o female with Down syndrome is brought in to office. Family claims her behavior has changed over past few weeks. She seems dizzy, and has developed urinary incontinence. She is hypotonic, but hyperreflexic with a Positive Babinski reflex. Has an Ataxic gait. Dx?

Atlantoaxial instability TQ: Should suspect AA instability in any Down's pt with UMN S+S (C1 and C2) Tx: surgical fusion of C1 to C2.

6 month old girl has rash on face, arms, chest. Erythematous, scaly lesions. Dx?

Atopic Dermatitis (Eczema) Hallmark: infant with itching, scaling, crusting lesions on extensor surfaces, trunk, cheek, etc. Tx: Topical emolliments

3y/o girl presents with sepsis (hypotensive, tachycardic) and requires IV access for fluid resuscitation. Despite numerous attempts, it is not possible to start a peripheral IV line. Next best step?

Attempt Intraosseous (IO) cannulation IO can be placed rapidly, by drilling into the proximal tibia. Should attempt IO when emergency access is needed and peripheral IV access is hard. IO requires less skills/practice too.

Mode of inheritance for SCA?

Aut Recessive

Marfans syndrome mutation?

Autosomal dominant disorder, mutation in Fibrillin-1 gene

Inheritance of HCM?

Autosommal Dominant

Defect in Osteogenesis imperfecta?

Autosommal dominant connective tissue disorder, caused by mutation in Type 1 Collagen (COL1A1) gene S+S: frequent fractures, blue sclera, short stature, hearing loss.

Legg-Calve Perthes

Avascular necrosis of hip

19 day old girl brought to ER for difficulty feeding. Was feeding and voiding fine until 2 days ago. Since then, has been much sleepier, and not feeding. Temp is 95.2, BP is 78/52, and pulse is 150. RR is 62. Has full fontanelle. Next best step?

Bacterial cultures and antibiotics TQ: Baby has neonatal sepsis. Clues (hypotension, tachy, tachypneic, full fontanelle, and low temp.) Neonatal sepsis can present with either hypothermia or fever. Neonates must get "Full Sepsis Workup" 1. CBC 2. Blood culture 3. Lumbar puncture 4. UA 5. Urine culture 6. And then receive Empiric antibiotics (Ampicillin and Gentamycin) after cultures are obtained.

Osteoid Osteoma

Benign bone-forming tumor, presents as pain worse at night. Femur is most affected. TQ: Pain improves with NSAIDs!! (Ex: pain is improved with Ibuprofen)

3 week old boy has a 1 week history of Jaundice, pale stooles. Was exclusively breast feeding. Now has Jaundice and Hepatomegaly, with increase in Direct Conj. Bilirubin. Cause?

Biliary atresia occurs when there's obliteration of extrahepatic biliary duct, causing malformation of bile duct and Gallbladder. Leads to buildup of Conjugated (Direct) Bili. S+S: pale stooles, jaundice, hepatomegaly. Dx: U.S., check for High Conj. Bili. Tx: Kasai procedure

16 y/o boy has right knee swelling with pain and stiffness. Knee exam shows palpable warmth, mild effusion. Aspiration of knee yields yellow-colored fluid. Recent travel to Maine on Soccer camp trip. Dx?

Borrelia burgdorferi infection Acute monoarticular arthritis from Lyme disease. Lyme arthritis is most common late manifestation. Inflammatory monoartiular, asymmetric arthritis. Common in knee. Tx of Lyme Dx: Oral Doxy

3 y/o boy has headaches, blurry vision, early morning N/V. Skin shows freckles on face, axilla, and HYPERpigmented macules covering back. Decreased vision. Next best step?

Brain and orbital MRI TQ: NF1 causes HYPERpigmented cafe-au-lait spots, and tumors in both CNS and PNS. Need MRI of brain and orbit for neurofibromas/optic gliomas.

15 month old boy brought to office for 4 episodes of otitis media, 2 episodes of lobar pneumonia. P.E. shows bulging, erythematous tympanic membrane on right side. Labs: Low IgG, A, M, E. Dx?

Bruton's Agammaglobulinemia TQ: Child with recurrent sinopulmonary infections, low serum immunoglobulins, and few B lymphocytes has X-linked Agammaglobulinemia/Bruton's Cause: defect in tyrosine kinase in B cells Hallmark: recurrent sinopulmonary infections (otitis media)

C1 inhibitor deficiency

C1 inhibitor deficiency causes: Hereditary Angioedema S+S: recurrent edema, especially of face, limb, genitals, associated with stress, dental work, trauma. Risk: Laryngeal edema, causes laryngospasm and airway obstruction Note: C1 inhibitor is necessary to inhibit Bradykinin Tx: give C1 inhibitor concentrate

#1MCC of CF pneumonia?

CF patients get recurrent pneumonias--->eventually leading to irreversible bronchiectasis. Kids with CF pneumo: S. aureus Adults with CF pneumo: Pseudomonas

Chronic granulomatous disease

CGD causes recurrent cutaneous abscesses, with Catalse-positive organisms (S. aureus, Serratia, etc) Cause: gene defect in NADPH oxidase enzyme pathway, leads to low NADPH oxidase, low H2O2, and impaired intracellular killing in phagocytes.

4 y/o boy complains of worsening ear pain. Started 2 weeks ago, and he completed antibiotics for otitis media. He awakes in middle of night with headaches, but feels better after vomiting in morning. Temp. is 100.4. He has a bulging, opaque left tympanic membrane, and his Left Mastoid is tender, swollen, and red. Next best step?

CT scan of brain TQ: Nocturnal headaches, with morning vomiting is red flag for intracranial pathology. Bacteria spreading from ear to mastoid can cause life-threatening brain abscess. Dx: made with CT scan to look for hallmark "Ring-enhancing lesion of brain abscess"

Tx of constipation in toddlers?

Can treat with laxative therapy (Polyethylene glycol) to soften stools

5 y/o boy fell with toothbrush in mouth in the morning. Complains of right-sided hemiplegia, right arm and leg weakness. Cause?

Carotid artery dissection TQ: falling with object in mouth (toothbrush, pencil, etc.) can cause dissection of internal carotid artery. Patients will present with symptoms of ischemic stroke (hemiparesis, facial droop, aphasia)

Treatment of cat bite with what Antibiotic?

Cat bites increased risk of infection with Pasturella multicida Tx: Amoxicillin/Clavulanate

Impetigo

Cause: S. aureus or Strep. pyogenes painful honey-crusted lesions of mouth

Centor criteria for?

Centor criteria us used to diagnose Strep throat in Adults: 1. fever 2. Tender anterior cervical lymphandenopathy 3. Tonsillar exudates 4. Absence of cough Tx: Penicillin or Amoxicillin

Most common posterior fossa tumor in kids?

Cerebellar Astrocytoma

5 y/o boy brought to office for "clumsiness." Had normal gross motor development before, but now frequently runs into doors or corners in house. Also begun to drink lots of water and pee a lot. Loss of bitemporal vision bilaterally. Dx?

Craniopharyngioma Craniopharyngiomas are derived from remnants of Rathke's pouch, in: suprasellar region adjacent to Optic Chiasm. Can lead to Bitemporal Hemianopsia, which can cause patient to run into stuff. Hallmark: MRI/CT: shows calcifications

What do Craniopharyngiomas cause?

Craniopharyngiomas put pressure on optic chiasm, causing Bitemporal Hemianopsia

2 y/o boy brought to ER for noisy breathing. Has rhinorrhea, and congestion and cough for 2 days. Cough is worse today, and he has a high-pitched noise during inspiration. He has a harsh, dry cough. Dx?

Croup TQ: Clue: question stem is describing a "harsh cough" (aka seal-like barky cough), caused by Parainfluenza virus (Croup) S+S of Croup (Laryngotracheitis): Parainfluenza virus--->"barky" cough, stridor, and hoarse voice +Steeple sign

What would be decreased in kid with DKA?

Decreased total body potassium DKA S+S: polyuria, polydipsia, abdominal pain, and fatigue. Pt will be dehydrated and rapidly breathing (Kussmaul breathing), to compensate for increased A.G. Met. Acidosis TQ: Bc the serum glucose exceeds threshold of kidneys to reabsorb it, causes glucosuria and osmotic diuresis. Diuresis causes net renal loss of Potassium with depletion of total body potassium stores Tx: 1. aggressive IV fluids 2. Insulin 3. K+ 4. Bicarb ( for metabolic acidosis)

1 hour old girl born to mom with PPROM delivered via C-section. Has tachypnea, nasal flaring, intercostal retractions, but clear breath sounds. CXR shows increased lung volumes, and fluid in interlobar fissures. Cause?

Delayed resorption and clearance of alveolar fluid TQ: baby has Transient Tachypnea of Newborn. Causes delayed resorption and clearance of alveolar fluid. Common in infants born via C-section. Hallmark: neonates with respiratory distress and "fluid in interlobar fissures."

1st line for 7 y/o peds pt with nocturnal enuresis?

Desmopressin (ADH)

4 month old has lethargy, poor feeding, fatigue, and pallor. Has webbed neck, cleft palate, and pale mucosa. MCV is 104. Hb is 4, Reticulocyte count is 0.4. Platelets are 300,000 Leukocytes are 7,500. Dx?

Diamond Blackfan Anemia TQ: congenital, PURE RBC aplasia Causes Low Hb, Low RBC, low reticulocyte count, and causes high MCV (macrocytic anemia) S+S: webbed neck, cleft palate, multiple thumbs

3 y/o boy has painful swelling in Right groin. Had 4 previous cutaneous abscesses and a lung abscess due to S. aureus, all of which needed drainage. Maternal uncle died in childhood from recurrent infections. How to confirm diagnosis?

Dihydrorhodamine test TQ: Kid has Chronic Granulomatous Disease mutation prevents phagocytic killing with oxidative bursts (with H2O2), and impairs intracellular killing by phagocytes. Causes recurrent infections, with Catalase+ bacteria. Diagnosis is confirmed with Neutrophil function testing (Dihydrorhodamine), which checks for presence of oxidative bursts.

Girl given Amoxicillin for sore throat, but develops maculopapular rash over entire body. Dx?

EBV infection TQ: Infectious mono, caused by EBV. If Amoxicillin/Ampicillin is given, break out into a maculopapular rash.

1 month old has a harsh, III/IV holosystolic murmur, over the left lower sternal border. Next best step?

Echocardiogram TQ: Kid has a VSD (most common cause of congenital heart disease) VSD murmur: Harsh, holosystolic murmur, heard at left lower sternal border. All murmurs require Echo. VSD usually closes on its own, by 2 y/o.

4 y/o male has meningitis S+S, but Gram stain shows no organisms. Most likely cause of infection?

Echovirus TQ: Viral (aseptic) meningitis, will show high WBC count, with normal protein, and normal glucose. #1MCC of viral meningitis: Echovirus and Coxsackie

Focal adnexal tenderness, with lower quadrant pain, and a history of STD. Concern for?

Ectopic pregnancy

10 y/o boy comes in with severe pain in left knee for past few days. Left knee hurts so bad, can't even play with friends. Knee is swollen, tender, and red. X-ray of knee reveals distal femur has "central lytic lesions, onion skinning, and a moth-eaten appearance." Dx?

Ewing's Sacroma TQ: Ewing's sarcoma is a highly malignant tumor of distal femur, common in young boys. Very aggressive, and mets early. Hallmark: "onion skinning" periosteal reaction, "moth eaten (mottled appearance), and extension into soft tissue.

Friedrich's Ataxia

FA is an Aut. Recessive condition, associated with excessive amounts of trinucletotide repeats (GAA), causing excess accumulation of Frataxin protein S+S: High Frataxin accumulates in brain, heart, pancreas 1. Neurological dysfunction with "wide-based gait, and pes cavus" 2. Hypertrophic cardiomyopathy 3. Diabetes Mellitus 3. Kyphoscoliosis S+S: dysarthria, "wide-based gait," limb ataxia, loss of vibration, position sense, and hallmark: pes cavus (high-arched foot)

14 y/o girl has not started menstruating. No breast development, no pubic or axillary hair. Ultrasound shows presence of uterus. Next best step?

FSH level TQ: Primary amenorrhea is absence of menses in girls >13y/o, with no secondary sexual characteristics First, do Pelvic U.S. to detect uterus Next, measure FSH levels 1. Low FSH means problems is in CNS (low GnRH, or low FSH) 2. High FSH means problem in ovarian (low Estrogen)

S+S of HIV infection

FTT lymphadenopathy opportunistic infections (Pneumocystis jirovecii) Oral thrush Low CD4+ count Dx: PCR

Risk factor for fetal shoulder dystocia?

Fetal hyperglycemia TQ: Maternal hyperglycemia (GDM) can cause fetal hyperglycemia, and hyperinsulinema can lead to fetal Macrosomia, which increases risk of shoulder dystocia. Shoulder dystocia can cause brachial nerve palsies and clavicle fractures

Orbital cellulitis

Fever pain with EOM proptosis Tx: IV antibiotics and surgery

Fibroadenoma

Fibroadenomas are very common in adolescents, presents as a solitary breast mass. Mass is "mobile, rubbery, and well-circumscribed." TQ: Fibroadenomas are located in the "outer quadrant of the Right breast." Premenstrual tenderness is common.

3 y/o girl, adopted from Nigeria, has sudden onset weakness. Unable to climb out of bed, unable to bear weight on her right leg. Has difficulty articulating, and has tachycardia. P.E. shows weakness, hypotonia, and hyperreflexia of right arm and leg. Which test should be performed?

Hb electrophoresis TQ: Sickle cell disease is the most common cause of pediatric stroke.

8 y/o boy has multiple staring episodes that started 3 weeks ago. He tilts his head to the right, and does not respond to name or touch. His mom says it looks like "he is chewing on something." Each episode lasts 2-3 minutes, and then he seems confused for 20 minutes after. Dx?

Focal seizure TQ: This patient has a Complex partial/focal seizure. Will cause LOC/post-ictal state. Can also involve "tongue chewing" and head turning.

Ataxia, scoliosis, hammer toes, Concentric Hypertrophic Cardiomyopathy

Friedreich's Ataxia

Full term vs. preemie supplementation to breast milk?

Full term infant can be exclusively breast fed till 6 months of age, before starting Vit D and Iron supplements Premature babies require Vit. D and Iron started at birth.

When to give Buspirone?

GAD, treat with Buspirone

2 week old neonate has poor feeding, persistant N/V. Appears lethargic, irritable, jaundiced. Liver and spleen are enlarged. Has bilateral cataracts. Deficiency of what?

Galactosemia, deficiency of Galactose-1-phosphate uridyl transferase TQ: pts with Galactosemia have FTT, bilateral cataracts, jaundice, hypoglycemia. Must eliminate Galactose from diet.

Only absolute C.I. to breastfeeding

Galactosemmia otherwise moms should exclusively breastfeed till 6 months.

Best way to confirm dx. of DMD?

Genetic testing DMD is X-linked recessive (Boys) TQ: Serum Creatinine phosphokinase and Aldolase levels are elevated in DMD TQ: Genetic testing is gold standard for dx

12y/o girl with anaphylaxis given IM Epi, but 10 minutes later, continues having hives, wheezing, and emesis. Tx?

Give another dose of IM Epi. TQ: IM Epi is first line treatment for anaphylaxis. Additional doses required for refractory S+S. Anaphylaxis MOA: 1. vasodilation--->systemic hypotension 2. tachy 3. Bronchospasm-->wheezing 4. uticarial rash, pruritis, flushing, 5. N/V an IgE mediated HSN reaction (Type 1)

Scarlet Fever

Group A Strep. pyogenes Fever, pharyngitis, strawberry tongue, sandpaper rash (especially along axillae, groin), which can desquamate Usually follows Strep. pharyngitis Tx. Penicillin

When does HCM increase in murmur intensity?

HCM is Autosomal Dominant, common in African Americans. Can cause "sudden cardiac arrest." Increased murmur intensity with: Decrease LV size, by decreasing preload 1. Valsalva 2. Standing Decrease in murmur intensity, occurs with increased LV size 1. Squatting 2. Leg Raise 3. Hand Grip

Schistocytes associated with?

HUS 1. Hemolytic anemia 2. thrombocytopenia 3. Acute kidney injury

What is considered Hypotonic Saline?

Half normal saline (.45%) and 5% Dextrose

4 y/o girl has itchy private parts. Scratching at bedtime, not during day. Vulva is erythematous, with no vaginal discharge. Perianal area is excoriated and erythematous. Dx?

Helminth infection TQ: Pt's "nighttime only itching" is hallmark for Enterobius vermicularis (pinworm) helminth infection Tx: Albendazole and/or Pyrantel pamoate

8y/o male with history of eczema, develops purple lesions over buttocks and thighs and behind knees. Other S+S most likely to have?

Hematuria Pt has HSP: IgA-mediated vasculitis, presenting with abdominal pain, purpuric rash on LEX. Can cause renal disease and hematuria.

Hallmarks of HUS?

Hemolytic anemia, thrombocytopenia, and acute kidney injury. Presentation occurs after resolution of bloody diarrhea caused by Shiga toxin (E.coli 0157:H7, or Shigella).

16 y/o boy with Hemophilia complains of limited ROM in right knee and pain and swelling for past 6 months. Pain has gradually worsened, and he's had pain like this before. Cause?

Hemosiderin deposition and fibrosis TQ: Pts with Hemophilia develop "Hemophilic arthropathy," due to recurrent hemarthrosis. MOA: Recurrent hemarhthrosis allows for Hemosiderin/Iron deposition in joints leading to synovitis and fibrosis within joint. Best prevention: Early prophylactic treatment with Factor concentrates

Which childhood vaccine could reduce the lifetime risk of developing cancer?

Hepatitis B Vaccine, will decrease incidence of hepatocellular cancer

Breast Milk Jaundice

High levels of Beta-Glucuronidase in breast milk cause deconjugation of CB-->UCB.

Transient synovitis

Hip pain in child, usually after a mild viral illness. Synovial inflammation leads to pain, decreased ROM, and limp. On P.E. patient is AFEBRILE. Has normal WBC count, and normal inflammatory markers. Tx: Just rest and NSAIDs

Anorectal manometry

Hirschsprung disease

10 month old boy has not yet begun to stand. Was born at 31 weeks gestation. Bilateral lower extremities are hypertonic, patellar reflexes are brisk, and both feet have Equinovarous deformities. Mild hypertonia of upper extremities also. Cause?

History of prematurity Cerebral palsy most commonly occurs due to prematurity. Causes uncoordinated, limited voluntary movements, with nonprogressive motor dysfunction. Premature birth<32 weeks is greatest risk factor. Buzzword: Equinovarus deformity

SCD RBCs during a vasooclusive crisis would look like?

Howell-Jolly Bodies normal nuclear remnants of RBCs that are normally removed by a functional spleen.

22 month old girl brought to office for abdominal pain, and keeps drawing up her legs when it occurs. Episodes last 15-20 minutes. Had 3 prior URT infections this winter, and gastroenteritis 2 weeks ago. She has a palpable cylindrical mass on Right side of abdomen. Most likely trigger?

Hypertrophied Peyers patches TQ: Most cases of Intussusception occur in kids <2y/o due to prior viral illness. Causes hypertrophy of Peyers patches in lymphoid rich ileum--->lead point for telescoping.

Hyposthenuria?

Hyposthenuria, or inability of kidneys to concentrate urine, can occur in patients with Sickle Cell Anemia. S+S: polyuria, low urine specific gravity, and normal serum sodium.

MOA of Epi in Anaphylaxis?

IM Epi works of Beta-2 receptors and Alpha-1 receptors. 1. allows bronchial smooth muscle relaxation (for bronchospasms/wheezing) 2. vasoconstricts (for hypotension)

Pregnant mom, brings her 3 y/o son to ER after he threw up coffee ground emeses, and complains of abdominal pain. He is irritable, and lethargic. BP is 80/50. X-ray shows radioopaque tablets in stomach. Next best step?

IV Deferoxamine TQ: Kid has Iron poisoning after swallowing Iron tablets! Iron is radioopaque on imaging, and causes abdominal pain, coffee ground vomit, hypovolemic shock, and metabolic acidosis. Tx: Immediate IV fluid resuscitation (for hypovolemic shock), and IV Deferoxamine (antidote as Iron chelator)

Antidote for Methemoglobinemia?

IV Methylene blue

6 y/o Pt with CF, has +Influenza. He is started on Oseltamivir. What other medication should he be given?

IV Vancomycin TQ: In kids with CF, #1MCC of bacterial pneumonia is S.aureus, especially with superimposed Influenza infection. Tx: IV Vancomycin

C.I. to giving Rotavirus vaccine?

If kid has hx. of Intussusuption TQ: Rotavirus is most common cause of gastroenteritis in infants/kids worldwide. Causes watery osmotic diarrhea. Highly contagious, transmitted via fecal-oral route. When to admin. vaccine: 2-6 months Risk: it can cause intussception

When to admin. Tetanus booster?

If last Tetanus shot was >5yrs ago.

Next step if BOTH parents refuse consent to life-saving tx?

If patient is Stable (Ex: chemotherapy for ALL in a stable patient), then need to seek court order approval before administering necessary but nonurgent tx If patient is unstable, can skip court order and directly treat.

Henoch-Schonlein purpura

IgA-mediated small vessel vasculitis

Meconium ileus is located in?

Ileum associated with CF no "squirt sign"

Best next step in 1 day old baby with PDA that is diffusely cyanotic?

Immediate admin. of Prostaglandin E1 TQ: Prompt PGE1 admin. can be life-saving and should not be delayed. After stabilization, then you can order an Echo to look for the specific defect.

Tx. for ruptured ectopic preg?

Immediate laparoscopy

When/how to vaccinate a 1 day old premature baby, born at 33 weeks, at 4 pounds? Has Varicella and Rubella IgG positive?

Immunizations should be given according to chronological age Key: Premature babies are at high risk for infections from vaccine-preventable diseases Vaccines for all medically stable preterm infants should be given by chronological age (not gestational age) TQ: All stable preterm infants should get the first dose of Hep B at birth Second dose of Hep B, Rotavrius Dipheria Acellular pertussis HiB Step. pneumococcal Inactivated polio vaccines = all given at 2 months

When can you proceed with a procedure, despite the parent not giving consent?

In Emergency situations Ex: 1. mechanical ventilation for compromised airway 2. Urgent blood transfusions 3. Emergency surgery

2 month old has crescendo-decrescendo systolic ejection murmurs at left upper sternal border, and single second heart sound. Brought to ER after becoming cyanotic, when his lips turned blue during feed. Patient placed in knee-chest position, and helps. How?

Increased systemic vascular resistance TQ: Pt has Tetraology of Fallot. (Pulmonary stenosis causing crescendo-decrescendo murmur, with single S2) Key: Due to the RVOT obstruction, if the pulmonary vascular resistance (on right side) exceeds the systemic circulation (on left side), deoxygenated blood will shunt fromm right to left and cause cyanosis. The knee to chest position will increase systemic vascular resistance, allowing blood to push from LV to RV (through VSD) into pulmonary artery and gets oxygenated

Best diagnostic test for child with increasing head circumference?

Increasing head circumference and increased ICP in kid should be evaluated with a CT scan of the brain. Tx: of hydrocephalus= VP shunt is placed from the ventricle to the peritoneum, which allows CSF to drain.

Anorexic girl given nasogastric feeding tube. On second day of admission, complains of severe SOB, and V-tachycardia. BMI is 14. Surge of what hormone led to this?

Insulin TQ: Girl is experiencing Refeeding Syndrome, which is a fatal complication of nutritional rehab in anorexia. MOA: Increased carbohydrate intake--->stimulates increase blood glucose--->stimulates Insulin--->Insulin stimulates cellular uptake of Phosphorous, K+, and Mg2+--->leads to prolonged QRS/Torsades/V-tachy

4 day old girl brought to ER with N/V, diarrhea, and abdominal pain. Grandma found pills scattered on kitchen floor. She is hypotensive, tachycardic, and has Hematemesis. "Small opacities are found in stomach and duodenum" pH: 7.3 Bicarb: 16 PaCO2: 29 What did she ingest?

Iron TQ: Iron is corrosive to GI mucosa, and causes severe abdominal pain, N/V, diarrhea, and bloody vomit. (within 1 hour of ingestion) Pts develop hypotensive shock with metabolic acidosis TQ: Hallmark: Iron tablets are radiopaque on X-ray Tx: IV Deferoxamine

When would you see an "elevated red blood cell distribution width?"

Iron deficiency anemia TQ: Iron deficiency is the most common nutritional deficiency in kids, caused by excessive intake of Cow's milk. Causes Iron deficiency (Microcytic) anemia. Hallmark: Increased RBC distribution width

#1MCC of anemia in kids?

Iron deficiency anemia. Often can occur with excessive intake of cow's milk. S+S: pallor, pica, fatigue.

9 month old baby has lethargy and tachypnea. Had severe diarrhea and fever 4 days ago. Only one wet diaper in 24 hours. He is tachycardic, tachypnic, and hypotensive. Na: 165. Treat with what kind of saline?

Isotonic Saline (0.9%) Pt has Hypovolemic Hypernatremia (secondary to GI loss and dehydration) When treating patient with Hypernatremia, must SLOWLY decrease Sodium, to avoid cerebral edema. Can only give IV fluid bolus of Isotonic saline (normal saline/lactated Ringers at 0.9%)

2 y/o girl complains of left knee pain, and fever which started 2 months ago. At time of onset, several kids at daycare were sick, but nobody had joint pain. She has daily fevers, and erythematous macular rash over chest. P.E. shows swelling, erythema, and warmth of left knee. She can walk, but with slight limp. Elevated ESR. Dx?

JIA TQ: JIA is an auti-inflammatory dx. in kids. Hallmark: long-standing fever, arthritis of atleast 1 joint, and a pink macular rash***

12 month old boy with severe vomiting/diarrhea since past three days. Is lethargic, with sunken eyes, dry mucus membranes, and capillary refill of 3-4 seconds. Next best step?

Key: pt has S+S of severe dehydration (cool, clammy skin, delayed capillary refill (>3 seconds), cracked lips, dry mucous membranes, sunken eyes, sunken fontanelle, hypotensive, tachycardic, lethargic, low urine output. Treatment of mild/moderate dehydration: just oral rehydration Treatment of severe dehydration: immediate IV fluids to restore perfusion and prevent end-organ damage. What to use: Isotonic crystalloid (20mL/kg of normal saline IV)

Septic arthritis

Kid will be ill appearing, FEBRILE, non-weight bearing. Elevated inflammatory markers. Must order a blood culture (will be positive), Tx: Order blood culture, arthrocentesis, and IV antibiotics

6 y/o boy has leg pain. Pain has been constant for 2 weeks, and is worse at night. P.E. shows a soft, non-fluctuant, tender mass over Right anterior distal thigh. Also, patient has a rash over his chest, trunk, and groin. Skeletal survey shows lytic distal femoral diaphyseal lesions. Dx?

Langerhans cell histiocytosis TQ: causes histiocytosis (macrophage infiltration in tissues), which proliferate and infiltrate organs. Can cause bone pain, and also invade skin (causing eczematous rash) Bone lesions are common in skull, leg, etc. X-ray (shows "punched out" lytic lesions

3 y/o girl comes to ER with severe oral pain. Had similar oral pain last year. Medical history includes sinus infections and numerous episodes of cellulitis. Skin cultures from prior infections show S. aureus and Strep. pyogenes. She has periodontal inflammation and necrosis. Labs: 90% Neutrophils. Dx?

Leukocyte Adhesion Deficiency delayed umbilical cord separation, recurrent bacterial infections, severe periodonitis, and marked leukocytosis occurs in LAD MOA: defective integrins on leukocyte surface, prevents neutrophil adherence. Labs show elevated free Neutrophils.

Cause of Angelman Syndrome?

Loss of Maternal chopy of 15q11 (paternal uniparental disomy)

Cause of Prader-Willi Syndrome?

Loss of paternal copy of 15q11 (Maternal uniparental disomy)

Labs in Turner's syndrome 45XO?

Low Estrogen, causing increased GnRH, and high FSH/LH.

Treatment of infant with suspected Bacterial meningitis?

Lumbar puncture, followed by IV antibiotics Bacterial meningitis (altered mental status, lethargy, fever, nuchal rigidity, N/V) Causes: Strep. pneumo (MOPS), or N. meningitis are most common causes of meningitis in kids >1month <1month (GBS) Must do immediate LP to analyze CSF, BEFORE antibiotics (bc meds could sterilize CSF) No need for CT Tx: Ceftriaxone, Vancomycin

McCune Albright Syndrome

MAS is a rare cause of precocious puberty, due to a mutation in GNAS gene. Results in constant overproduction of pituitary hormones. Causes precocious puberty, cafe-au-lait spots, and recurrent fractures.

thymoma associated with?

MG

One of the reasons human breast milk is more beneficial than formula?

Major source of protein in human breast milk is Whey, which is more easily digested/absorbed than Casein, and helps improve gastric emptying.

Most common cause of vaginal bleeding in neonatal period?

Maternal withdrawal of Estrogen TQ: maternal estrogen crosses placenta during pregnancy, and causes growth of fetal endometrial lining. Following delivery, slough off causes self-limited, mucoid, vaginal bleeding

11 month old African American boy, brought in for well-child visit. Eats homemade baby food, and is breastfed. On exam, the anterior fontanel is wide open. Bony prominences of the costochondral junctions are noted bilateraly. +Genu Varum. Cause?

Nutritional deficiency TQ: Nutritional Rickets occurs if infant is only breastfed, and does not have enough Vit D intake (through formula or fortified baby food, or sun) Tx: Must supplement with 400 IU of Vit. D daily. Note: Kids with Rickets develop Genu Varum

Trichotillomania is comorbid with?

OCD

Osteogenesis Imperfecta

OI is an Aut. Dominant Connective Tissue disorder, caused by mutation to Type 1 (bone) collagen. Type 1 collagen: bone, sclerae, skin, teeth. MOA: defective Type 1 Collagen causes frequent fractures from minor trauma, as well as "dentinogenesis imperfecta" (blue-gray opalescent teeth), and weak enamel. Joint hypermobility (increased ligamentous laxity), Scleral thinning (blue sclerae), hearing loss, short stature

Breastfeeding Jaundice

Occurs in 1st week of life Cause: insufficient intake of breast milk, causing decreased Bilirubin elimination. Pt will have high UCB, jaundice, dehydration, decreased urine output/delayed stooling.

Tx. of choice for 7y/o kid who has Lyme disease?

Oral Amoxicillin TQ: Should avoid #1DOC in adults (Doxy) for Lyme disease in kids/preggos, bc Doxy (a Tetracycline) can slow bone growth and lead to teeth discoloration. TQ: Must use Oral Amoxicillin for kids and preggos with Lyme disease

13 day old boy has mild eyelid swelling, conjunctival injection, and a scant amount of mucopurulent discharge. Next best step?

Oral Erythromycin TQ: Bc baby only has "scant amount" of DC from eyes, he has: Chlamydial conjunctivitis (causes watery/scant mucopurulent DC) Pts need Oral Erythromycin tablets, as topical Erythromycin is not potent enough.

Most readily available Emergency contraception?

Oral Levonorgestrel (Plan B), is most readily available Emergency OCP, and works by: Delaying Ovulation Note: MOST effective OCP is Copper IUD, which works by creating an inflammatory response that is toxic to sperm and ova, thereby preventing fertilization.

4 day old boy brought to clinic for fever for past 5 days. Has a productive cough, with yellow-green mucus. Chest X-ray shows opacity over lower left lung, with minimal layering of fluid on lateral decubitus film. Next best step?

Oral antibiotics Bacterial pneumonia (yellow productive cough, with opaque consolidation) should be treated with oral antibiotics.

Most common predisposing factor to orbital cellulitis?

Orbital cellulitis most commonly occurs from Bacterial Sinusitis Pain (EOM)/opthalmoplegia Proptosis Fever Dx: Head CT Tx: IV antibiotics and surgical drainage of abscess

7 y/o boy presents with left thigh pain and limp. Exam shows limited range of motion of left hip, and atrophy of thigh. Cause?

Osteonecrosis TQ: Legg-Calve-Perthes is an Idiopathic osteonecrosis of the Femoral Epiphysis. Presents in young kids with progressive limp and thigh atrophy. X-ray will show "fragmented femoral head"

14 y/o girl has BP of 140/90 in right arm, and 90/40 in LEX. She is in the 5th percentile for height, and has not gotten her period yet. What is she at risk for?

Osteoporotic fractures TQ: Patient has Turner's syndrome (45XO) Suspect in any patient with: short stature, amenorrhea, and coarctation of aorta Patients have streaked ovaries, leading to Estrogen deficiency. Estrogen is needed to prevent osteoclast bone resorption. Pts should get Estrogen supplements to promote normal sexual maturation, and decrease osteoporsis.

Most common cause of Rabies in US?

Painless Bat bite all patients with direct exposure to bats require rabies prophylaxis Postexposure prophylaxis includes: Rabies immune globulin and Rabies Vaccine

Which tumor is Parinaud syndrome associated with?

Parinaud syndrome causes eyelid retraction, resulting in preference for downward gaze, with limited upward gaze. Also causes bilateral eyelid retraction. Associated with Pineal gland tumors.

2 month old girl has right-sided flattening of back of head. Prefers to Tilt head to Left, and Turn head to Right (contracted Left SCM). P.E. shows flattening of the Right occiput with Anterior displacement of Right ear and forehead. There is a palpable, firm mass that doesn't transilluminate in inferior part of left neck. Underlying cause of dx?

Postural deformity of neck TQ: Infant with neck mass and head tilt towards contracted side, with chin deviation away, has Torticollis TQ: common to have a "well-circumscribed mass that doesn't transilluminate in inferior SCM."

5 y/o girl has dark pubic and axillary hair. She is obese. Also has mild facial acne. Genitalia is normal, bone age is normal. Most likely dx?

Premature adrenarche TQ: Premature adrenarche is caused by early activation of adrenal androgens, and is common in obese children. S+S: precious dev. of coarse pubic and axillary hair, body odor, acne. Normal genitals, normal bone age.

Recommendation for parents of child with Freidrich's Ataxia?

Prenantal genetic counseling is available if they desire future pregnancies TQ: F.A. is an autosommal recessive condition, and subsequent risk of another child being affected is 25%. Genetic counseling is available for future kids.

4 y/o girl has Bordatella pertussis infection. Prescribed a course of Macrolide antibiotics. Lives with her parents and sister. Most appropritate way to limit spread to household contacts?

Prescribe Macrolide antibiotics to all household contacts. TQ: B. Pertussis is highly contagious. Spreads through respiratory droplets. 1st line tx: Macrolides (Azithromycin, Erythromycin, Clarithromycin). Should get regardless of age, immunization status, and S+S.

Primary polydypsia

Primary (psychogenic polydypsia) causes excessive thirst and excessive water intake Labs: dilute serum, with low Sodium (hyponatremia)

Turner's syndrome has what kind of amenorrhea?

Primary amenorrhea (absent menses before age 15) Confirm Dx. of Turner's via Karyotype Analysis (45 XO)

parents divorced. Kid steps on rusty nail and needs tetanus vaccine. Mother consents, but father refuses and wants herbal options. Next step?

Proceed with giving tetanus vaccine TQ: informed consent from one parent/guardian is considered enough to give proposed therapy.

4 y/o male has episodes of nocturnal enuresis.

Provide reassurance Key: most kids develop toilet training from 2-4 years old. But bed wetting is normal until kid is 5y/o. 1st line treatment for bedwetting after 5 y/o= 1. Enuresis alarm therapy 2. Desmopressin (ADH) 3. Imipramine (for refractory cases)

2 month old baby frequently regurgitates small amount of formula after each feed. Parents are concerned. Rest of exam is normal. Next best step?

Provide reassurance TQ: GERD is common in infants due to shorter esophagus, and underdevelopment of LES. Just reassure the parents, and advise to hold baby in Upright position for 20-30 minutes after feeds.

#1MCC of pneumonia in adults with CF?

Psuedomonas

9 y/o African American boy presents with fever, and irritability. BP is 80/60. Has been hospitalized several times due to abdominal pain. Did not get routine vaccines bc mom was afraid of Autism. Pt dies after admission. Cause?

Pt should have been vaccinated with a conjugate capsular polysaccharide. Key: In patients with SCA, must be vaccinated with Pneumococcal vaccine PLUS Penicillin prophylaxis to prevent Pneumococcal Sepsis.

7 y/o boy has sore throat, swollen tonsils, covered in thin white exudates. Small, tender anterior cervical lymph nodes are presented. Next best step?

Rapid Stretococcal antigen testing TQ: Group A Strep. pyogenes pharyngitis (Strep throat) causes; fever, tonsillar exudates, and anterior lymphadenopathy. Must confirm with Rapid Strep Antigen Test

4 y/o adopted girl from mom who abused cocaine/alcohol. Adopted 6 months ago. Does not play with other children, doesn't listen to adopted mom, cries randomly. Doesn't like being hugged or picked up. "Nothing I do seems to comfort her." Dx?

Reactive attachment disorder TQ: occurs in kids with history of early abuse/neglect. Withdrawn emotionally/socially, lack of response to comfort.

2 y/o boy comes to ER for convulsions. Has runny nose and red eyes yesterday. Woke up this morning with rhythmic jerking movements of arms/legs. Episode lasted 3 minutes. Now is awake and playful. Next step?

Reassurance Key: Febrile seizures do not cause brain injury. Harmless, so can go home.

Newborn girl delivered to mom with GDM. Baby was 10 pounds so had to be vacuum delivered. She is crying constantly, and exam shows crepitus with palpation of left clavicle. Tx?

Reassurance and gentle handling Babies large for gestational age, are at high risk for birth injuries like Clavicle fractures. Clavicle injuries heal in about 5-7 days on own, without complications.

1 day old boy has a swollen scrotum, and when light is shone behind the scrotum, it transilluminates. Next best step?

Reassurance and observation TQ: Newborn Hydroceles are painLESS scrotal swelling that does transilluminate on exam. Observe and reassure, most cases go away within 1 year. Hydrocele, fluid collection in tunica vaginalis (sac surrounding testes), bc of patent processus vaginalis. Noncommunicating hydrocele, occurs when there's fluid within tunica vaginalis, but the processus vaginalis closes, trapping the fluid.

Causes of Intussusception?

Recent viral illness (Rotavirus) Lead points (Meckel's diverticulum) HSP

Hirschsprung Disease is located in?

Rectosigmoid junction +Squirt sign S+S: poor feeding, abdominal distension, failure to pass meconium, +explosive expulsion of stoole on rectal exam ("Squirt sign") Contrast enema can show "Transition zone. Use rectal suction biopsy to diagnose

Best way to avoid firearm injures in suicidal teen?

Remove firearms from home!

23 month old girl has pain while urinating, blood in urine, and urine smells bad. Has Left CVA tenderness, and +Nitrates and +Leukocyte Esterase. Urine culture shows +E. coli. In addition to completing a course of antibiotics, what else needs to be done?

Renal and Bladder U.S. TQ: Kids with their first febrile UTI should be treated with a 1-2 week course of antibiotics, and a Renal and Bladder US needs to be done to check for anatomical abnormalities that could cause recurrent UTIs. If abnormalities found on US, then you can order a VCUG

5 y/o boy was diagnosed with Strep. pharyngitis 2 weeks ago, and was treated with 10 day course of Amoxicillin. Completed full course. Two days ago, he developed fever (102), seemed fatigued, and complained of right knee pain. Asked to be picked up rather than walk. His knee is swollen, and tender to touch. Pain with active ROM and weight bearing. Labs: Increased WBC, CRP/ESR. Next best step?

Right knee arthrocentesis TQ: Pt has Septic arthritis (suspect in kids with recent URT, sith acute fever, joint pain, and elevated WBC, ESR/CRP. Tx: Arthrocentesis is both diagnostic and therapeutic

9 month old boy status post-orchioplexy. What is he still at risk for?

Risk of testicular cancer remains increased TQ: Crytpoorchidism should be corrected before 1 y/o in order to prevent testicular torsion and infertility. Although risk of Testicular cancer is decreased with Orchioplexy, it overall remains elevated compared to general population.

Treatment of Tourette's?

Risperidone TQ: Treatment options for Tourette's include 1. habit reversal therapy 2. Antipsychotics (Risperidone works best)

Roseola (HHV-6)

Roseola caused by HHV6/7 High fever, suddenly stops and causes a diffuse maculopapular rash. NOT vesicular

Roseola

Roseola is caused by HHV-7 causes a high fever, that suddenly stops, and then leads to a Morbilliform rash

16 y/o girl has fever, joint pain, and rash that started on face, and rapidly spread down her body. Also has pain in fingers and wrists. Immunization status unknown. P.E. shows a blanching, maculopapular rash on face, chest, back, extremities, that spares palms and soles. Positive Posterior auricular and Suboccipital lymphadenopathy. Dx?

Rubella TQ: Rubella causes a maculopapular rash that starts on face and very rapidly spreads down. +Posterior auricular and Suboccipital lymphadenopathy. Girls also get arthralgias/arthritis

SCA osteomyleitits cause?

S. aureus

3 y/o boy has Right Neck Swelling. Had lump on right neck yesterday, which has increased in size, and is erythematous and tender. Had a mild URT infection last week. Has 5cm anterior cervical lymph node, mobile, warm, tender. Organisms?

S. aureus TQ: Acute, unilateral lymphadenitis in kids is due to S. aureus. L. node is tender, warm, erythematous.

5 y/o boy brought to office for fever (101) and left-sided neck swelling for one day. P.E. shows a red, tender, fluctuant anterior cervical mass about 2 cm in diameter in left Anterior lymph node. Microbe?

S. aureus TQ: S. aureus and Strep. pyogenes are most common causes of acute, unilateral lymphadenitis in kids. Submandibular nodes are most commonly infected. S+S: enlarged, red, very tender neck mass. Tx: Clindamycin helps treat against both MRSA and Strep. pyogenes

Cause of osteomylitis is SCA?

Salmonella

Vit C deficiency?

Scurvy (lack of Ascorbic acid) S+S: impaired collagen synthesis, damaged connective tissue. Bleeding gums, petechiae, impaired wound healing. Coiled (springy looking) hair.

What is a "subcutaneous emphysema?"

Severe coughing paroxysms can cause air to leak from chest wall-->into subcutaneous tissue (due to increased intralveolar pressure). This results in Subcut. emphysema and potential pneumothorax. Must do immediate CXR to rule out pneumothorax

2 month old presents to ER after a tonic-clonic seizure. Exam shows bilateral retinal hemorrhages. Cause?

Shearing of the Subdural veins Subdural bleeding can manifest as seizures, increased head size, bulging anterior fontanelle, and altered mental status. TQ: Shaking causes retinal hemorrhages. Bilateral retinal hemorrhages must be evaluated on CT for a Subdural Hematoma.

What kind of bleeding occurs in Renal Papillary Necrosis?

Sickle Cell Trait can cause PAINLESS hematuria, due to Renal Papillary Ischemia and Necrosis

Benign lymph nodes

Small, soft, mobile, nontender <2cm Tx: just observe

Next best step for pt diagnosed with GBS?

Spirometry TQ: Must monitor PFTs for respiratory paralysis in GBS! Labs: Increased protein, normal glucose, normal WBCs Tx: Plasmapheresis, IVIG, monitor PFTs. Do NOT give steroids.

Splenic sequestration labs in SCA pt?

Splenic sequestration will cause splenomegaly, with low platelet count, and high Reticulocyte count (Spleen traps Sickled RBCs and platelets inside)

12 y/o boy is brought in due to several months of back pain. Also has bed wetting recently. P.E. shows a "palpable step-off" at the lumbosacral area. Negative straight leg raise test. Perianal sensation is decreased to pinprick sensation, but has normal anal reflex. Dx?

Spondylolisthesis TQ: Development problems associated with "forward slip" of one vertebrae over another (L5 over S1) S+S: adolescents with back pain, neurological probs (bowel/bladder incontinence), and "Palpable step-off," at lumbosacral angle.

Purpose of squatting in Tetralogy of Fallot?

Squatting increases afterload felt by LV--->aorta, which causes blood to rush from LV--->RV--->oulmonary artery, improving cyanosis.

+Trendelenburg Sign means weakness of?

Stand on Right foot--->Left Hip drop means: you have weakness of Right Gluteus medius and minimus (innervated by Superior Gluteal Nerve)

6 y/o girl has had 10 day history of thick nasal discharge, nasal congestion, and cough. Yellow, purulent mucus is dripping from posterior nasopharynx. Nasal turbinates are red, swollen. Bilateral expiratory wheezes present. Organism?

Strep. pneumo TQ: Strep. pneumo is most common cause of Acute Bacterial Rhinosinusitis

3y/o boy with SCA, presents with BP of 78/40, pulse of 140, lethargic and pallor. Organism?

Strep. pneumonia Key: Pt developed sepsis (hypotensive, tachy). SCA pts develop functional asplenia due to recurrent splenic infarction. Highest risk of infection from encapsulated bacteria. Despite vaccines, Strep. pneumo remains, by far, the MOST COMMON cause of sepsis in SCA, due to "non-vaccine serotypes." To prevent: must vaccinate AND prophylactically treat with Penicillin.

Causes of non-bullous impetigo?

Strep. pyogenes, and Staph. aureus

Superficial hemangioma

Superficial hemangioma (aka Strawberry hemangioma), is a benign capillary tumor in kids. Tends to grow rapidly, and resolve spontaneously.

Obese male complains of Right groin pain, knee pain, and limping. He is in 90th percentile for weight. Hip motion is restricted, and right foot points outwards. Tx?

Surgical pinning of Femoral Head Obese males have increased risk of Slipped Capital Femoral Epiphysis Should be promptly treated with surgical pinning of slipped epiphysis where it lies in order to avoid Avascular Necrosis of Femoral Head

Ewing Sarcoma

Swelling, severe pain, deformity. Extensive involvement of affected bone.

9y/o kid has oozing skin lesions on legs. Came back from camping trip 4 days ago. Has well-demarcated tan line above the knee, and scattered erythema with vesicles in a linear arrangement below the knees. Some lesions are wet, and others are thin, crusted. MOA?

T-cell mediated reaction TQ: Kid has a Type 4 (delayed) HSN reaction, caused by T-cells (he has allergic contact dermatitis, to poison oak, nickel, poison ivy, etc)

Pinealoma

TQ: Parinaud syndrome occurs in patients with a Pinealoma gland mass. S+S: limited upward gaze, bilateral eyelid retraction, and headache, N/V due to obstructive hydrocephalus.

2 y/o boy brought to ER for bleeding. Parents discovered a substantial amount of Maroon-colored stool in diaper. Boy is cooperative, but pale. +fecal occult blood. Which imaging modality would confirm dx?

Tech-99m pertechnetate scan TQ: Meckel's diverticulum is most common congenital small intestine anomaly. Results from incomplete obliteration of Vitelline duct. Contain gastric and pancreatic tissue TQ: causes PAINLESS hematochezia

3 month old male rushed to ER for cyanosis. Turned blue while feeding. Auscultation reveals "single S2" and a harsh, crescendo-decrescendo systolic murmur in left sternal border. Dx?

Tetraology of Fallot! IHOP TQ: "single S2" occurs bc of pulmonary stenosis, so only sound is closure of Aortic valve. TQ: "crescendo-decrescendo murmur" in left sternal border occurs due to stenosis of pulmonary artery

Tx of Gonococcal conjunctivitis?

Topical Erythromycin cream (good prophylaxis for Gonoccocal conjunctivitis) S+S: significant/more severe eyelid swelling, and PROFUSE purulent DC

Wernicke's encephalopathy

Thiamine (Vit B1 deficiency) causing classic traid of: 1: Encephalopathy 2. Ataxia 3. Oculomotor dysfunction Common in alcoholics Tx: Thiamine

Most common cause of congenital hypothyroidism?

Thyroid dysgenesis is most common cause of ongenital hypothryoidism worldwide Labs: Low T3/T4, high TSH Tx: Prompt recognition and Thyroid Hormone replacement (with Levothyroxine T4), in order to prevent permanent neurodevelopmental injury

18 month old boy presents with sleepiness. Has frequent episodes of crying inconsolably, and then "totally normal" between episodes. Has had multiple episodes of emesis, and is passing loose, dark, "sticky" stoole, with blood. Next best step?

Ultrasound-guided contrast enema TQ: Kid has Intussusception. Pediatric emergency, where bowel telescopes into itself. Buzz: emesis, "red, currant jelly stoole", lethargy. Episodes of crying, then normal. Key: Must use an U.S. guided air contrast enema to diagnose Intussusception

Best way to detect VUR?

VCUG

1 day old boy has a small face and jaw. There is overlapping of the fingers bilaterally, along with convexity of bilateral soles. Murmur at left lower sternal border. Which cardiovascular abnormalities is present?

VSD TQ: Pt has Edwards Syndrome (Trisomy 18) S+S: 1.micrognathia/microcephaly 2. Rocker-bottom feet 3. overlapping fingers 4. absent palmar creases HYTQ: Edwards pts have VSD ("holosystolic murmur at left lower sternal border.")

Side effect of VUR?

VUR can cause recurrent/chronic pyelonephritis. Complications: parenchymal scarring, HTN, and renal failure. Definitive diagnosis: Voiding cystourethrogram.

3y/o boy developed fever 2 days ago. Now has a maculopapular rash, which appeared on his trunk and face and became VESICULAR. Vesicles have spread to arms and legs. Normal mucous membranes. Infection?

VZV-->Varicella Zoster Virus chickenpox Hallmark: fever, followed by a diffuse maculopapular rash with "crops of vesicles."

Preventative health measure for 4y/o boy?

Visual acuity testing must assess vision at every well-child visit. Starts at 4y/o, using 1. Snellan chart 2. Red reflex testing (for retinoblastoma) 3. corneal light reflex testing

Pellagra

Vit B3 (Niacin) deficiency S+S: dermatitis (sunburn looking), diarrhea, dementia, death. TQ: also presents with beefy red tongue (glossitis)

Patient developed high grade fever, and rash, which spread from face-->trunk--->extremities. Prior to onset of rash, has nonproductive cough, teary eyes, and congestion. P.E. shows maculopapular rash all over body, which spares palms and soles. Which Vitamin would help decrease morbidity?

Vitamin A Pt has measles (fever, with a "morbilliform rash" with cough, coryza, conjunctivitis.) Rash starts on face and travels downwards. Vit. A helps produce more antibody-producing cells and regenerates epithelial cells.

3 y/o boy has dry eyes, and photophobia. Difficulty adapting to darkness. Very picky eater, and mostly eats canned foods, without fresh vegetables or milk. Has dry scaly skin, follicular hyperkeratosis on extensor surfaces. Dx?

Vitamin A deficiency TQ: causes night blindness (loss of rods), photophobia, dry, scaly skin, and dry eyes, and hyperkeratosis.

Defect in Wiskott Aldrich Syndrome?

WAS: is X-linked recessive defect in WAS gene--->causes impaired "cytoskeleton regulation" in leukocytes and platelets S+S: 1. Eczema 2. Thrombocytopenia 3. lots of recurrent infections

Mom bathing her 3y/o son finds an abdominal "swelling." Recently treated with antibiotics for Strep. pharyngitis. Firm, nontender mass is palpable in Left abdomen. Child has hematuria. Dx?

Wilms Tumor (NEPHroblastoma) Most common pediatric renal malignancy, presents as asymptomatic, Unilateral abdominal mass, with hematuria.

Hallmark of NF2?

bilateral acoustic neuromas, and cataracts

Preseptal cellulitis

break in skin allows Staph/Strep to enter preseptal space Tx: just oral antibiotics

Alcohol use disorder

frequently present with insomnia, anxiety, and depressive S+S.

Side effect of Phenytoin?

gingival hyperplasia SJS/TEN

Common complication of bacterial meningitis?

hearing loss

1 y/o African American girl has HbA 60% HbS 40% HbF 0% most common complication of her condition?

hematuria TQ: Pts with Sick Cell Trait can lead normal, healthy lives. PAINLESS heamturia is most common complication. (occurs bc of sickling in kidney)

Tinea versicolor

hypopigmented macules on trunk, face, extremities.

HCM gets better with?

increased preload (taking deep breath in, HANDGRIP, squatting)

6y/o male with eyelid swelling after mosquito bite. Developed swelling and redness, fever. On eye exam, has limited ADDuction. Most concerning S+S?

ophthalmoplegia TQ: Kid has Orbital cellulitis bacterial infection of orbital septum, leading to 1. pain with EOM/ophthalmoplegia 2. Proptosis 3. fever Tx: IV antibiotics and surgical drainage. Otherwise can travel head and cause cavernous sinus thrombosis.

Tx. of breastfeeding jaundice

optimize lactation and increase frequency of breastfeeding.

16 y/o boy brought to ER after falling off bike and hitting his head on ground. Witnesses say he lost consciousness for about 1 minute, but had no seizures. Boy vomited twice in ambulance, and has mild headache. GCS is 15. Next best step?

order a Noncontrast CT scan of head TQ: Pts who have mild traumatic brain injury (brief loss of consciousness, headache, N/V)= concussion Should get a non-contrast CT, and observation for 4-6 hours to rule out anything bad.

Where is Vit K derived from?

our body derives Vit K from diet, gut flora synthesis, and Liver, and breast milk

Primary dysmenorrhea

pelvic pain and cramping during first few days of menses. Normal physical exam. Cause: just an increased release of Prostaglandins from Endometrial sloughing during menses. Tx: First line is NSAIDs

Nevus flammeus

port wine stain, that does not regress

Risk factors for NRDS?

prematurity, and maternal diabetes mellitus

Cyclical vomiting syndrome

recurrent, self-limiting episodes of N/V in a predictable pattern (Ex: beginning of every month). Family history of migraines is often present. Tx: Antiemetics (Ondansteron) and Anti-migraine