PEDIATRICS Shelf study- BRS/pretest/lange/uworld

Pataasin ang iyong marka sa homework at exams ngayon gamit ang Quizwiz!

miliaria rubra 1. pathogenesis + clinical presentation 2. tx

1 disrupted sweat ducts --> sweating/occlusion--> small erythematous pruritic papules or vescles on intertriginous areas 2. avoid occlusive clotion

cryptococcus 1. pathogenesis + clinical presentation 2. tx

1. yeast in soil --> inhalation--> asymptomatic in immunocompetent, immunocompromised: CNS meningitis, disseminated infection of bones/joints/skin 2. systemic antifungal

1. testicular tumor frequency 2. treatment

1. yolk sac (60%)>teratomas> seminomas/embryonal carcinomas 2. radical orchiectomy/node dissection

Birth length increases by 50% at

12 months

Triple birth weight by

12 months

Expected height increase: 13 - 24 months

12.5 cm/year

Expected weight gain: 6 - 12 months

10g/day

charcots arthritis

joint destruction from neurological loss --- ex. diabetic neuropathy, syringomyelia, spine injury, B12 deficiency, tabes dorsalis

most common cause of hypoglycemis in children age 1-6 yeasr

ketotic hypoglycemia

components of the adaptive immune system

T cells, B cells, immunoglobulins

how does blood get to the body of an infant with severe coarctation of the aorta

blood from the pulmonary artery goes past the coarctation through the ductus arteriosus (this is a right to left shunt)

what does the Apt test detect

maternal/fetal hemoglobin in a bloody specimen such as vomit or melena~~ identifies the source of the blood--- treating specimen with alkali turns mother hemoglobin to hematin and fetal hemoglobin is unchanged

evaluation of jaundice in a newborn

maternal/infant Rh types + ABO groups + coombs total, direct, indirect bilirubin Hct, reticulocyte count CBC (to screen for sepsis)

lithium effect on the kidney

nephrotic syndrome AND RTA

1. neuroblastoma origin 2. location 3. genetics

neural crest cell tumor that arises anywhere along the sympathetic ganglia chain and in the adrenal medulla 2. abdomen or pelvis, posterior mediatinum 3. unbalanced translocation between 1p and 17q, and 14q / 22q abnormalities

Can developmental milestones be skipped?

no, one almost always follows the next

What other disease does IEM have a similar clinical presentation?

sepsis

sickle cell disease 1. RBC lifespan 2. retic count 3. WBC count 4. platelet cell count *5. peripheral smear

1. 10-50 days 2. 5-15% 3. 12-20k 4. increased >500k 5. sickles, targets, howell jolly bodies

1. how is breathing evaluated during CPR 2. what to do if the patient isn't breathing

1. look listen, feel method = look for chest movement, listen for exhaled air, feel for exhaled airflow 2. rescue breathing

folic acid deficieny anemia 1. pathogenesis

1. low fruits/vegetables, goat milk**, celiac disease, infectious enteritis, crohns, anticonvulsants, OCP --> dec. folate levels --> megaloblastic (macrocytic)anemia --> sx. of anemia, FTT, chronic diarrhea, irritability

Mycoplasma pnemoniae 1. clinical presentation 2. dx + CXR 3. tx

1. low grade fever, chills, non-productive cough, pharyngitis, malaise, headache, widespread rales on physical exam **EXAMINATION FINDINGS ARE WORSE THAN THE HISTORY 2. positive COLD AGGLUTININS, serum IgM titers for mycoplasma CXR shows bilateral diffuse infiltrates 3. erythromycin or azithromycin

ASD ostium primum 1. features 2. what other heart defect associated 3. which genetic disease associtaed with

1. lower portion of the atrial septum, 2. +/-cleft or deviation in the anterior mitral valve leaflet also causes mitral regurg (MCC congenital heart defect in downs syndrome 3. DOWNS SYNDROME~ most common congenital heart disease in these patients

chronic lymphocytic thyroiditis (hashimotos) 1. pathogenesis 2. clinical presentation 3. dx/tx

1. lymphocytic infiltration of thyroid + anti -TPO/thyroglobulin antibodies--> fibrosis, atrophy/hyperplasia 2. transient HYPERTHYROIDISM then hypothyroidism, pebbly* goiter, short stature 3. neonatal screen (TSH), high TSH, low T4, antithyroid antibodies (anti-TPO) tx = synthroid when patient becomes hypothyroid

Extra-pulmonary TB disease

(1) Cervical lymphadenitis (scrofula), the most common form of extrapulmonary TB disease in children (2) Meningitis (3) Abdominal involvement (ileitis) (4) Skin and joint involvement

What are the health risks of smoking?

(1) Coronary artery disease and stroke (2) Cancers of the lungs, mouth, esophagus, stomach, larynx, and urinary tract (3) Chronic lung disease and asthma (4) Peptic ulcer disease (5) Pregnancy complications, such as stillbirth, low birth weight, and higher-than-normal infant mortality

Stephen johnsons syndrome 1. pathogenesis + clinical presentation 2. tx

1 drugs (phenytoin, barbiturates, sulfonamides, penicillin), m.pneumoniae, HSV1 --> high fever/cough/malaise--> erythema multiforme (targetoid lesions), lesions/blisters/necrosis + two mucosal surfaces involved 2. steroids, IVIG, burn care, stop drug

Inborn Errors of Metabolism 1. when do they typically present 2. how does mitochondrial disorders differ form most IEMs

1. severe neonatal illness at times of stress, chronic or progressive symptoms 2. chronic and progressive course

1. 3 B hydroxysteroid dehydrogenase deficiency clinical presentation 2. lab diagnosis

1. severe salt wasting crisis, glucocorticoid deficiency, and ambiguous genetalia 2. elevated 17 hydroxyprogesterone

adolescent type 1 DM pathogenesis

1. sex steroids and growth hormone are antagoinistic to insulin action

Osteogenesis imperfecta 1. patho 2. clinical presentation

1. type 1 collagen mutation 2. blue sclera, HEARING LOSS, fragile bones, genu valgum, scoliosis/kyphsis, osteoporosis, osteopenia, dentinogenesis imperfecta (II/III/IV), easy bruising

allergic conjunctivitis 1. pathogenesis + clinical presentation 2. tx

1. type 1 hypersensitivity reaction accompanies seasonal allergic rhinitis--> itching + watery discharge 2. cromolyn eye drops,topical antihistamines

autoimmune hepatitis 1. pathogenesis (type1/type 2) 2. clinical presentation 3. dx 4. treatment

1. type 1: ANA or anti-smooth muscle antibodies type 2: anti-liver kidney microsome or anti- liver cytosol type 1 antibody 2. usually females before puberty, --> mimics viral hepatitis+fatigue, anorexia, arthritis, rash, nephritis, vasculitis 3. elevated serum transaminases, hypergammaglobulinemia, circulating autoantibodies, liver bipsy 4. corticosteroids (acute), then immunosuppressents (azathioprine, 6-mercaptopurine

what fractures are suspicious for child abuse

FOUND WITH BONE SURVEY metaphyseal fractures posterior or first rib fractures multiple fractures at varied ages of healing complex skull fractures scapular, sternal, and spinous process fractures *** fracture that dont fit childs DEVELOPMENTAL ABILITIES ---- ex. spiral fracture of the lower extremities in a child who is not ambulatory

what does steroid resistant minimal change disease develop into

FSGS

Early symptoms of HIV in an infant

FTT, thrombocytopenia, recurrent infections (otitis, pneumonia, sinusiits), lymphadenopathy, parotitis, recurrent thrush, loss of dev. milestones, zoster/varicella

clinical presentation of febrile seizures (simple vs complex)

6 months - 6 years of age, FEVER >102.2 simple = <15 minute generalized seizure complex = >15 minutes, or has focal features, or recurs within 24 hours

physiologic anemia of infancy

6-8 weeks of age: erythropoiesis temporarily stops after birth and physiologic anemia reaches its nadir at 6-8 weeks

Causes of mental retardation

Genetic causes -Chromosome abnormalities (e.g., Down syndrome, fragile X syndrome) -Inborn errors of metabolism (e.g., Hurler syndrome) -Single gene abnormalities (e.g., tuberous sclerosis) Prenatal and perinatal problems -Fetal malnutrition -Placental insufficiency -Maternal drug and alcohol use -Brain malformations (e.g., hydrocephalus) -Perinatal hypoxia or asphyxia -Infection (e.g., herpes simplex, rubella, cytomegalovirus, toxoplasmosis) Environmental problems -Psychosocial deprivation -Parental mental illness Postnatal acquired insults -Infection (e.g., meningitis, encephalitis) -Head trauma -Near drowning

Contents of a well child visit

H-DOPIA • H- history - age specific trigger questions • D- developmental surveillance: age spec. ques, questionairres, school performance • O- obs. of parent/child interaction • P- physical examination - focuses on growth (i.e. length/height, weight, BMI, head circumference) • I - immunizations • A - anticipatory guidance (i.e. discussion of safety issues) and Additional screening tests (e.g. lead level depending on age)

what treatment for children born to HBsAg positive mothers

HBIG and and HBsAg recombinant vaccine in the first day of life

Acronymn for adolescent psychosocial history

HEADSS home education/employment activities drugs sexual activity suicide/depression

nephritic vs nephrotic

HERAN - hypertension, edema, acute sedement, reduced GFR, (RBC/WBC casts), non-nephrotic proteinurina <50mg/kg/day HELLP - hypertension, edema, lipiduria, hyperlipidemia, proteinuria >50mg/kg/day

what infectoius agents cause neutropenia

HIV, EBV, CMV, hep A/B, influenza A, parvo B19 typhus, rocky mountain spotted fever, malaria

HLA haplotypes (chromosome 6) associated with Type 1 diabetes

HLA DR3/DR4

ankylosing spondylitis + clinical association

HLAB27 male predomiant axial skeleton and lower extremity joints + enthesis ~ associated with pauciarticular JRA in MALES*****

what are the risk factors for child abuse that increase the frequency of abuse **SUSPECTED CHILD ABUSE MUST BE REPORTED

<4 years of age <1 year of age mental retardation chronic illness

at what level of bilirubin does kernicterus occur

>18mg/dl

Familial short stature

>2 SD below the average, with short MPH but with a NORMAL BONE AGE, a normal onset of puberty, and a minimum growth of 2 inches (or 5 cm) per year.

Constitutional short stature

>2 SD below the mean with history of delayed puberty in either or both parents, a delayed bone age and late onset of puberty, and a minimum growth of 2 inches (or 5 cm) per year.

apneic episodes in preterm infants (NOT periodic breathing)

>20s pauses due to undeveloped respiratory center

late neonatal hypoparathyroidism

>4 days of age, due to maternal hyperparathyroidsim, temporary

microscopic hematuria proteinuria

>6 RBCs per high powered field >100mg/m2/day (by urine dipstick)

Microcephaly-head examination

Head circumference below the 10th percentile •may be familial but may also be caused by structural brain malformations, chromosomal and malformation syndromes, congenital infections (e.g., cytomegalovirus, toxoplasmosis), or fetal alcohol syndrome

Growth disturbances

Defined as growth outside of the usual pattern. Two common types: • Failure to thrive (FTT) • Head growth abnormalities

Schwachman-Diamond syndrome

an autosomal recessive disorder characterized by: a. pancreatic exocrine insufficiency b. failure to thrive (FTT) c. short stature, d. neutropenia, and sometimes pancytopenia

systemic causes of headache

anemia, hypoglycmeia, depression, hypretension, CO poisoning

what drugs cause neutropenia

antibiotics, anticonvulsants, aspirin, radiation, chemotherapy

what must a patient who has rhuematic fever prophylax against

antimicrobial prophylaxis especially before minor dental procedures

phenothiazine

antipsychotic/antihistaminic class drugs include chlorpromazine (dopamine antagonist), promethazine (antiemetic, sedetive)

treatment of HIV positive children

antiretrovirals prophylaxis for oppurtunistic infections immunizations (EXCEPT LIVE VARICELLA) MONITOR VIRAL LOAD ANNUAL OPHTHALMOLOGIC EXAMINATION to assess CMV retinitis

what substances are seen in the urine from tubular damage

biconcave RBCs, glucosuria, aminoaciduria, BETA 2 MICROGLOBULIN

what other heart anomaly is associated with coarctation of the aorta

bicuspid aortic valve***

what must be investigated in a black vs white child with polydactyly

black child - nothing, 10x more common in black children white child - look for heart defects, inc. incidence of heart defects

open comedones closed compedones

blackheads whiteheads

what is the screening test for lead poisoning

blood lead level

what are the iatrogenic complications of dialysis

blood loss dialysis related aluminum toxicity folic acid deficiency

why does right to left cardiac shunt/congenital heart disease lead to increased risk of brain abscess/infection

blood that passes through the shunt avoids the lungs and its macrophage filtering mechanism

what does a low retic count in the context of anemia indicate

bone marrow failure or diminished hematopoiesis (red cell aplasias, pancytopenia, malignancy)

compression fracture (aka torus aka buckle) + treatment

bony cortex buckles under compressive force usually at the metaphysis tx. with splinting

Clinical presentation of primary adrenal insufficieny

cortisol def signs - hypotension, inc. pigmentation, weakness, anorexia aldosterone def signs - hyponatremia, hyperkalemia*, FTT, salt craving

clinical presentation of esophageal obstruction

cough, drooling, choking, avoidance of liquids

EBV mononucleosis complications

cranial nerve palsies encephalitis upper airway obstruction (from pharyngitis) splenic rupture** (no heavy lifting/contact sports) nasopharyngeal carcinoma burkitts lymphoma lymphoproliferative disease

What kind of tumor must be considered in any child older than 5 years of age who is not growing 2 inches/year

craniopharyngioma ~~ because it will decrease the production of growth hormone

clinical pres + treatment for infant clavicle fracture

crepitus over clavicle, and decreased movement of the ipsilateral armf tx = nothing, reassurance

what other conditions can present like epiglottitis

croup, bacterial tracheitits, retropharyngeal abscess

what parasitic infections seen in HIV positive patients

cryptosporidium, toxoplasmosis, isospora

viral meningitis dx

culture takes 10-14 days, PCR for EBV CMV HSV enteroviruses

job-buckley syndrome

defect in phagocytic chemotaxis- hypergammaglobulin E, PRURITIC DERMATITIS, eczema, EOSINOPHILIA, severe RECURRENT staph infections

what two factors in VSD determine how much blood goes from left to right

degree of pulmonary vascular hypertension size of the VSD

clinical presentation of hypothyroidism

delayed bone age/suboptimal growth velocity (<5cm/year [2in]) goiter myxedema (puffy/dry/orange skin)

complications of sickle cell dieseae

delayed growth, cor pulmonale, gallstones, poor wound healing, avascular necrosis of the femoral/humoral heads

what history suggests organic constipation

delayed meconium passage, onset of constipation in infancy, history of pelvic surgery, encopresis before 3 years of age, inability to toilet trian

hepatitis D infection

deltavirus that requires HBsAg for replication, cuases progression of hepatitis B or causes fulminant liver failure

biotin deficiency/biotinidase deficiency clinical presentation

dermatitis, alopecia, ataxia, hypotonia, seizures, deafness, immunodeficiency, metabolic acidosis

klippel feil syndrome

developmental failure of vertebral segmentation--> fusion of vertebrae--> congenital torticollis, GU anomalies, CHD, sprengels deformity

Clinical presentation of hypogycemia

•neonate may be asymptomatic or may present with: •diaphoresis •jitteriness •feeding problems •tachycardia •hypothermia •hypotonia •seizures, and, rarely •myocardial infarction.

diagnosis of cushings disease

dexamethasone suppression test, if cannot suppress the adrenal there is an ACTH tumor ~~~ high dose dex suppresses pituitary tumor ~~~ high dose dex doesn't suppress ectopic ACTH producing tumor

patient with exudative pharyngitis is treated with amoxicillin for suspected group A strep infection 1 week later develop pruritic macularpapular rash. Why?

did not have group A strep they had EBV mononoculeosis which has this reaction when treated with amoxicillin

what is the important clinical finding for twin transfusion syndrome

difference in hematocrit >15mg/dl between two infants donar = oligohydrominos, anemia, hypovolemi recipient = polyhydraminos, plethora, with evidence of shock

hypoglycemia in a premature infant born to a normal mother

diminished glycogen and fat stores --> post partum hypoglycemia

partial seizures

discharge of only one hemisphere, seizures are predominantly motor, sensory, psychomotor simple = no loss of consciousness complex = consciousness is impaired

basic metabolic panel (CHEM7)

electroloytes - Na, K, CO2, chloride kidney - BUN and creatinine glucose and calcium

complete metabolic panel (CHEM12)

electrolytes - Na, K, CO2, chloride kidney - BUN, creatinine *proteins- Albumin, total protein *liver tests - ALP, AST,ALT, Bilirubin glucose and calcium

what labs should be ordered for urolithiasis

electrolytes, BUN, creatinine, calcium, phosphorus, parathyroid hormone, uric acid, venous blood gas urine culture plain radiograph/renal ultrasound

what causes glucose intolerance/peripheral resistance in renal failure

elevated glucagon and growth hormone

1. lab diagnosis of lead poisoning 2. treatment of lead poisoning

elevated serum lead level elevated erythrocyte protoporphyrin 2. dimercaprol, british anti-lewisite, calcium disidium ethylenediaminetetraacetic acid

1. diptheria clinical presentation 2. tx

gray adherent tonsillary membrnae , low grade fever --> cardiac failure and neurologic complications 2. macrolide or IV penicillin + antitoxin

renal tubular acidosis

inability of the kidney to maintain normal acid base balance

What is malabsorbtion

inadequate absorbtion of nutrients that is characterized by abdominal distension and impaired growth

thyroid dyshormonogenesis

inborn errors of thyroid hormone synthesis~ a cause of congenital hypothyroidism

hyper IgM

high IgM, all other immunoglobulins are low (compare to brutons*) + neutropenia ~ recurrent sinopulmonary infections, PCP pneumonia , poor resposne to immunizations

destructive therapies

high dose salicylic acid, podophyllin, 5-FU, cryotherapy, electrotherapy, laser therapy~~ for warts of molluscum contagiosum

what should be done if there is no antivenin available for a patient bit by a poisonous snake

immobilization, tetanus prophylaxis, local wound care and transportation to a facility that has anti-venin

what is the most common cause of communicating hydrocephalous

interventricular hemorrhage (subarachnoid hemorrhage) leading to destruction of the arachnoid villi and cisterns blocking flow of CSF

treatment of allergic rhinitis

intranasal steroids (SE = local irritation, no systemic effects on HPA axis) Antihistamines - diphenhydramine ,cetirizine, fexofenadine, loratadine cromylyn sodium psuedoephedrine - insomnia, nervousness, rebound rhinitis,

treatment of status epilepticus

intravenous anticonvulsants, short acting benzodiazepines followed by phenobarbitol or phenytoin

Spastic diplegia

involves the lower extremities more than the upper extremities or face.

what treatable underlying condition can be predispose to breath holding spells/pallid spells

iron deficiency anema treat by giving oral ferrous sulfate and iron rich cereal

Haemophilus influenza type B disease

meningitis, epiglottitis, sepsis

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS)

patients develop the acute onset of obsessive-compulsive symptoms or a tic disorder after streptococcal infection. Antibiotic therapy prevents this complication.

mumps clinical presentation + complications

parotitis**** complications = meningoencephalitis (during or after parotitis), orchitis (post pubertal), epididymitis, pancreatitis, abscess/osteomyelitis of the jaw

GERD

pathological state in which reflux causes GI or pulmonary symptoms

oculocephalic manuver (coma eval)

patients eye turn back to midline after turning the head to the side , if brainstem damage has occured, head movement does not cause eye movement = (negative dolls head)

what patients are at high risk for renal artery emboli

patients with umbilical catheters

what toxicity associated with vincristine

peripheral neuropathy, constipation, jaw pain, SIADH

pyridoxine deficiency clinical presentation

peripheral neuropathy, irritability, depression, dermatitis, stomatitis, elevated homocystine

what is the cause of peripheral "acrocyanosis"

peripheral vasocontstriction from cold temperature

components of the innate immune system

phagocytes, NK cells, TLRs, manose binding protein, alternate complement path

retropharyngeal abscess clinical presentation

pharyngitis, fever, severe sore throat, fluctuant bulge in posterior pharynx suppurative infection of the posterior pharyngeal wall by staph, GAS, or oral anaerobes

why does SGA neonatal hypoglycemia and polycythemia occur in mothers who have gestational hypertension or toxemia

placental infarction/toxemia decreaes uteroplacental blood flow leading to dec. glycogen storage--> compensatory hypoglycemia and polycythemia

what are the complications of staph pneumonia (most commonly seen in children <1 year of age)

pleural effusion, empyema, pyopneumothorax

what is the possible etiologies for chest pain in a sickle cell pt

pneumonia, thromboemboli, sepsis~~ risk of rapid progression

loud snoring and suspected sleep apnea, what is the work up/treatment

polysomnography CPAP/BIPAP

post pericardiotomy syndrome

reactive pericarditis/pericardial effusion after surgery for congentital heart disease in an infant

what is fever

rectal temp >100.4

clinical presentatino + treatment of foreign body

recurrent pneumonias in a healthy child, decreased inspiratory and expiratory sounds Dx = rigid bronchoscopy (only way to dx) tx = rigid bronchoscopy

what is the role of corticosteroids given with the first dose of antibiotics for suspected meningitis

reduces the incidence of hearing loss in HIB meningitis

PTH effects

releases Ca2+/phos from the bones, reabsorbing calcium/HCO3- at kidneys secretion of phosphate secretion at the kidneys stimulates 1-alpha-hydroxylase at kidney to inc. conversion of 25- 1,25 OH vitamin D

what are the steps of early neonatal care for an uncomplicated pregnancy

removal of airway secretions drying and wrapping in blanket silver nitrate eye drops/vitamin K supplementation

most common case of ESRD in children

renal scarring secondary to reflux dx. with VCUG

mallory weiss tears

repeated vomiting/retching --> streaked blood in the vomit ~ occasionally results in massive hemorrhage

bronchopulmonary dysplasia

results from intubation --> persistant oxygen demands, respiratory distress, and abnormal cxr, airway hyperresponsiveness, PULMONARY EDEMA REQUIRING DIURETIC TREATMENT

classic cause of toxic shock syndrome

retained tampons

shaken baby syndrome / shaken impact syndrome clinical findings

retinal hemorrhages, subdural hematomas, metaphyseal fractures, brain injury

1. risk factors for brain abscess in a child

right to left intracardiac shunts/cyanotic heart disease, otitis media, mastitis,

childhood hypoparathyroidism

ring chromosome 16 or 18, autoimmune,digeorge

MCC diarrhea in children between age 6 months to 2 years

rotavirus

Amniotic band syndrome (amnion rupture sequence)

rupture of amniotic sac, small strands of amnion wrap around the fetus

what is the modern (vaccine era) cause of epiglottitis

s.pneumo, GAS, moraxella

bacterimia in an infant causes

s.pneumo, h.flu, meningococcus

keratolytics -

salicylates, urea, alpha hydroxy acids, retinoic acid~~ hyperkeratosis

RTA type III

same as type 2 but presents as wasting in infancy, same treatment

which synromes have congenital glaucoma

sturge weber, neurofibromatosis, marfans

what focal lesions of the brain can cause coma

subarrachnoid hemorrhage, multiple infarcts, thalamic infarcts, subdural hematoma, non-accidental trauma, cerebellar hemorrhage, post infectious encephalitis

nursemaids elbow + tx

subluxation of radial head from upward force on the arm (radial head is slender shape in toddlers) ex. pulling a toddler upward by the hand to make them stand *toddler presents holding the elbow flexed tx = flexing the elbow and supinating the hand

hepatitis A clinical presentation

sudden onset fever, N/V, tenderness of the liver, jaundice, AST elevation <3 weeks,

treatment of crohns disease/ulcerative colitis

sulfasalazine (maintenance), corticosteroids(acute exacerbations), immunosuppresants, metronidazole (perianal crohns) total proctocolectomy - ulcerative colitis recurrence after bowel resection is high for crohns disease TPN during flare ups

migraine treatment

sumatriptan - 5HT agonist propranolol - prophylaxis

Scalp edema or cephalohemotoma

superiosteal hemorrhage of the cranium after traumatic delivery, DOES NOT CROSS SUTURES

inactivated polio (salk) 1. advantages/disadvantages 2. timing

• only polio vaccine recommended in the US 1. no vaccine associated polio, no secondary (herd) immunity 2. given at 2 and 4 months, boosters at 6-18 months and at 4-6 years

Concrete thinking

• preschool and early elementary school years • interpreting things literally

Magical thinking

• preschool toddler years • normal state of mind when a child assumes that inanimate objects are alive and have feelings.

Newborn color

•Color is pink a few hours after birth •Acrocyanosis and cutis marmorata are frequent intermittent signs of the vasomotor instability characteristic of some infants.

Bone age < chronologic age

•Constitutional short stature •Hypothyroidism •Hypercortisolism •Growth hormone deficiency •Chronic diseases

Which 3 contraceptives have the lowest failure rate?

•Depo-Provera •IUD •OCP

Management of lead poisoning

•Education to decrease exposure •Chelation therapy (EDTA and Meso-2,3-dimercaptosuccimic acid (DMSA succimer)

Bone age = chronologic age

•Familial short stature •IUGR •Turner sydnrome •Skeletal dysplasias

Turner syndrome short stature

•Female patients may present with lack of puberty and poor growth velocity •Tx with growth hormone

Intestinal Obstruction

•Functional or mechanical •and if mechanical, may be acquired or congenital

Virilized female

•Genetic XX •Female pseudohermaphrodite with ambiguous genitalia and no gonads palpable

Three most common STDs in the united states

•HSV •HPV •Chlamydia trachomatis **HAVING 1 STD INC. LIKELIHOOD OF HAVING ANOTHER STD

What other diseases is autoimmune oophoritis associated with?

•Hashimotos thyroiditis •Addisons disease,

Macrocephaly

•Head circumference > 95% for age. •Unlike microcephaly, the size of the head in patients with macrocephaly does not necessarily reflect brain size. Evaluation includes: •measurement of parental head circumferences •careful physical examination that includes observation for split cranial sutures, bulging anterior fontanelle, irritability, or vomiting, all of which may suggest elevated intracranial pressure. •Head ultrasound or CT scan is performed to rule out hydrocephalus, if suggested by physical examination. •Genetic evaluation may be useful if a genetic syndrome is suspected.

Microcephaly

•Head circumference is 2-3 standard deviations below the mean for age. • Incidence is 1-2/1000 children. •Etiologies: a) Congenital microcephaly (b) Acquired microcephaly

Preterm delivery

•birth before 37 weeks •seen in 7% of all births in the US •incidence is higher in lower socioeconoomic populations, and in women who lack prenatal care

What cysts are seen on the lateral neck?

•branchial cleft cysts •cystic hygromas

Cri du chat syndrome

•caused by a partial deletion of the short arm of chromosome 5 •slow growth, microcephaly, mental retardation, hypertelorism, downslanting palpebral fissures, and a characteristic catlike cry.

Hydrometrocolpos

•caused by an imperforate hymen with retention of vaginal secretions •It presents as a small cyst between the labia at the time of birth or as a lower midline abdominal mass during childhood.

What information gained from the history of an adolescent (or any patient) must be reported

•sexual/physical abuse •suicidal/homicidal intention

Epstein pearls

•small, white epidermoid-mucoid cysts found on the hard palate •usually disappear within a few weeks

Craniotabes

•soft areas of the skull with a "Ping-Pong ball" feel •They may occur in the parietal bones and are not related to rickets •They usually disappear within weeks or months

Cephalohematoma

•subperiosteal hemorrhages secondary to birth trauma •confined and limited by the cranial sutures •usually involving the parietal or occipital bones.

Vernix caseosa

•thick, white, creamy material found in term infants •it covers large areas of the skin in preterm infants •It is usually absent in post-term infants

What can NEC led to?

•thrombocytopenia •disseminated intravascular coagulation •death. Late complications: •Intestinal obstruction (e.g., adhesions, strictures) •Nutritional deficiencies (e.g., malabsorption, short gut syndrome) •Cholestasis

TAR syndrome

•thrombocytopenia-absent radii (TAR)

Midline clefts or masses

•thyroglossal duct cyst •goiter (2' to maternal antithyroid medication or transplacental passage of long-acting TSH antibodies) •omphalocele •cleft lip/palate

Ears examination

•to assess maturity. •By term, the ears are firm and have assumed their characteristic shape. •The ears should also be inspected for preauricular tags or sinuses and for appropriate shape and location.

Enuresis

•urinary incontinence beyond the age when the child is developmentally capable of continence. Classification •Nocturnal (occurs only during sleep) versus diurnal (daytime) •Primary (never been consistently dry) versus secondary (at least 6 months of prior consecutive dryness)

what are the common complications of maternal diabetes on an infant

cardiac malformations (TGA, VSD) lumbosacral agenesis neural tube/renal defects duodental atresia/small left colon holoprosencephaly/anencephaly

what is the temporal sequence between genital ulcer and inguinal lymphadenopathy in chlamydia vs h.ducreyi

chlamydia- ulcer disappears then inguinal adenoaphty forms h.ducreyi - ulcer and inguinal adenopathy occur at the same time

what is the treatment for rheumatic fever chorea

chlorpromazine or barbiturates NOT antibiotics or salicylates

when does sickle cell symptoms begin

at 6 months of age when HbF declines

tacrolimus ointment used to treat

atopic dermatits

what drugs can cause coma

atropine, scopalamine, benzodiazepines, barbiturates, ethanol, lithium, opiates, TCA

When to suspect mitochondrial disorders?

atypical presentation THREE OR MORE ORGAN SYSTEMS

what is first step in evaluation of a child with a speech delay ie. dec. babbeling, or not meeting language milestones

audiology evaluation

alopecia areata 1. pathogenesis + clinical presentation 2. tx

autoimmune lymphoccyte mediated hair follicle damage--> complete hair loss complete hair loss + pitting of nails 2., hair regrowth wihtin 1 year accelerated with steroids or minooxidil

warrdenberg syndrome

autosomal dominant- iris abnormalities - sectoral heterochromia/heterochromia poliosis (forelock of white hair) wide set eyes,broad nasal root, hearing loss

Treacher Collins syndrome (mandibulofacial dystosis)

autosomal dominant- downslanting eyes, micrgnathia, missing face bones, conductive hearing loss, downslanting palpebral fissures

glanzmanns thrombasthenia

autosomal recessive defect in gpIIb/IIIa leads to decreased platelet aggregation

bernard souliers + blood smear findings

autosomal recessive disorder of gpIb platelets cannot adhere to vWF on endothelium --> severe hemorrhage ~ smear shows large platelets

cystinosis

autosomal recessive lysosomal storatge disease of cystine leads to crystalline deposits in the cornea, FANCONI SYNDROME (MCC), renal failure, FTT

what is the treatment for pertussis

azithromycin (used to be erythromycin) within 2 weeks of onset of coughing.... after this it is a toxin mediated disease

How to treat aortic dilation of Marfans to prevent complications?

beta blockers

Cognitive concepts that evolve over time

(1) Object permanence (2) Cause and effect (3) Magical thinking

Asperger syndrome

(1) Qualitative impairment in peer relationships and social interactions (2) Repetitive, restricted, and stereotyped patterns of behavior, activities, and interests (3) No clinically significant language delay. Language, in fact, may be advanced.

Russell-Silver syndrome

(1) Small for gestational age (2) Craniofacial findings include a small triangular face, prominent forehead, and down-turned mouth. Because of the small face, the head appears large, but the head circumference is normal. (3) Skeletal findings include short stature and limb asymmetry. (4) Café-au-lait spots on the skin (5) Excessive sweating

When is the optimal time for language acquisition?

first 2 years of life

what kind of CHF caused by anemia

high output CHF

what factors increase risk of vertical transmission of HIV

high viral load chorioamnionitis maternal genital infections premature birth prolonged rupture of memrbanes

organic causes of constipation

hirschprungs disease, neuroenteric dysfunction, low fiber feeding, anatomic, dehydration, celiac, hypothyroidism, cystic fibrosis, botulism, lead toxicity

bruising, petechiae, pallor in a child with cancer

infiltration of the bone marrow

aseptic meningitis

inflammation of the meninges with CSF lymphocytic pleocytosis, no protein, normal glucose

where are pediatric brain tumors located

infratentorial, and midline

bronchiolitis treatment

inhaled epinephrine

organophosphate poisoning mechanism + clinical presentation + tx

inhibits carboxylic esterase enzymes including acetylcholinesterase leading to elevated acetylcholine--> constricted pupils, bradycardia, muscle fasiculations, diaphoresis, diarrhea, salvation tx = pralidoxime and atropine

what visceral injuries cause death in child abuse

injury to the GI tract, liver, and spleen

what are the four types of VSD

inlet, trabecular, membranous, outlet ( supracristal)

psoriatic arthritis

small/large joint arthritis associated with psoriasis (caly skin plaques, nail pitting, onychoysisi)

Sotos syndrome

hereditary gigantism

why must vitamin K be supplemented for newborns

immature intestinal flora leads to low vitamin K levels

somnambulinism

sleepwalking occurs in 15% of children usually occurs during stage 4 non-REM sleep

1. cat bites secondary infetion

1. paturella multocida, bartonella henselae (Cat scratch disease)

how long is puberty

3-4 years

Birth length doubles by

Age 4

leukocoria

RB, cataract, vitreous hemorrhage

Fetal cigarette smoking

SGA, polycythemia

what are the congenital heart diseases that cuase decreased pulmonary flow

TOF pulmonary atresia tricuspid atresia

varicella post exposure prophylaxis

VZIG or acyclovir ~ perferable withing 72 hours of expsure

Common sleep problems

a. Nightmares b. Night terrors

yolk sac tumor marker

alpha feto protein

colles fracture

distal radius fracture

which antidepressant used in anorexia nervosa

imipramine

what toxins can cause coma

lead, mercury

monteggia fracture

proximal ulna with dislocation of the radial head

What kind of formula will be asymptomatic in galactosemia?

soy formula

Edema and webbing of the neck

suggests turner syndrome

WAGR syndrome

wilms tumor, aniridia, genitourinary abnormalities, mental retardation

ABCs of CPR

• Airway • Breathing • Circulation

Clinical definition of obesity

•body weight >20% than IBW •BMI > 95% for age and sex

CHARGE syndrome

(1) C—colobomas (absence or defect of ocular tissue), usually of the retina. Impaired vision is very common. (2) H—heart defects, most commonly tetralogy of Fallot (3) A—atresia of the nasal choanae (4) R—retardation of growth and cognition (5) G—genital anomalies, including genital hypoplasia (6) E—ear anomalies, including cup-shaped ears and hearing loss

Trisomy 13

(1) Neurologic findings inc: holoprosencephaly, microcephaly, seizures, and severe mental retardation. (2) Ocular findings include microphthalmia, retinal dysplasia, colobomas, and, rarely, a single eye. (3) Cleft lip and palate

Cornelia de Lange (Brachmann-de Lange) syndrome

(1) Small for gestational age and FTT (2) Craniofacial findings include single eyebrow (synophrys), long, curly eyelashes,microcephaly, thin, down-turned upper lip, and micrognathia. (3) Infantile hypertonia (4) Mental retardation (5) Small hands and feet (6) Cardiac defects (7) Behavioral findings include autistic features, lack of facial expression, and self-destructive tendencies.

Classification of cerebral palsy

(1) Spastic cerebral palsy (Affected patients have increased tone) -spastic diplegia -spastic hemiplegia -spastic quadriplegia (2) Extrapyramidal cerebral palsy

Bacterial pneumonia 1. clinical presentation 2. physical examination 3. Diagnosis 4. management

(1) Symptoms have more rapid onset and greater severity. -Fever, cough, and dyspnea typically occur without preceding upper respiratory symptoms. (2) Physical examination may demonstrate rales, tachypnea, de- creased breath sounds, and evidence of respiratory distress. (3) Diagnosis is suggested by a WBC count >20,000 cells/mm3 with a neutrophil predominance, and lobar consolidation on CXR (4) Management includes appropriate antibiotics and supportive care

Tuberculosis Screening

(PPD, Mantoux skin test) is recommended for children at risk for tuberculosis, including: a. Contacts of persons with confirmed or suspected infectious TB b. Children in contact with high-risk groups c. Children with radiographic or clinical findings suggestive of tuberculosis d. Children who have immigrated from endemic areas, those with a history of travel to endemic areas, or contact with indigenous persons from endemic areas e. Children with HIV f. Children without specific risk factors who reside in high prevalence areas

Etiologies of macrocephaly

(a) Familial, associated with an otherwise normal physical examination and a family history of large heads (b) Overgrowth syndromes (e.g., Sotos syndrome), in which all growth parameters are enlarged (c) Metabolic storage disorders (e.g., Canavan syndrome, gangliosidoses) (d) Neurofibromatosis (e) Achondroplasia (f) Hydrocephalus (g) Space-occupying lesions (e.g., cysts, tumors)

Male physical changes during puberty

(a) Testicular enlargement begins between ages 11 and 12 years and is the first sign of puberty. (b) Facial and axillary hair growth begins approximately 2 years after the growth of pubic hair begins.

Ellis-van Creveld syndrome

(defect is 4p16) is associated with atrial septal defects.

Fragile X syndrome

* most common inherited form of mental retardation 1.X LINKED trinucleotide CGG repeats with ANTICIPATION 2. mild to severe MR, large ears, macrocephaly, thickened nasal bridge, and blue irides, large testes, behavioral findings

what are the causes of apnea in a HEALTHY TERM infant

***major pathological issue (compare to preterm) sepsis, GERD, CHD, seizures, hypoglycemia****

Viral pneumonia 1. clinical presentation 2. dx + CXR 3. management

**Viruses are the most common cause of pneumonia in all age groups** 1. URI sx (nasal congest/rhinorrhea/fever/cough/dyspnea) --> tachypnea, wheezing, rales, respiratory distress 2.WBC <20k with lymphocyte predominance CXR = interstitial infiltrates 3. supportive

clinical presentation of childhood cancer

**persistent fever, weight loss, night sweats, fatigue~~~ assc. with leukemia, lymphoma and other cancers **palpable mass **abdominal mass **mass on trunk or extremities

Growth measurement initially spared in failure to thrive

*Head circumference •weight is usually affected before length, which is usually affected before head circumference

treatment for CHF (3 drug classes + mechanisms

*cardiac glycosides - increased efficeincy of myocardial contractions and dec. tachycardia *loop diuretics - decreased intravascular volume, decreases afterload/ventricular dilation *ionotropes - dobutamine/dopamine phosphodiesterase inhibitors- amrinone, milrinone- inc. contractility

Klinefelter syndrome

*most common cause of male infertility 1. XXY 2. tall starture long extremities hypogonadism- delayed puberty gynecomastia antisocial behavior, shyness, aggression

****kid with UTI --> do a VCUG

...

SLE mneumonic pg 475

...

ddx for lower GI bleed page 307

...

page 252 has whole ddx for chest pain

...

pg 408 chart of PT/PTT/bleeding time + ddx. hemostasis

....

pg 340 hypertension in children

.....

pg 328/329 differential diagnosis for hematuria/red urine

.........

what endocrine disorder should be associated with a child who has amidline defect ~ cleft palate, hypoglycemia, and microphallus

...congenital hypopituitarism

Evaluation for: 1) Indirect hyperbilirubinemia 2) Direct hyperbilirubinemia

1) CBC, reticulocyte count, and smear (for hemolysis) are necessary. 2) hepatic ultrasound (to evaluate for choledochal cyst), serologies for viral hepatitis, and radioisotope scans of the hepatobiliary tree are necessary. Evaluation for sepsis may be indicated for both.

Differential diagnosis of speech or language delay

1) Global developmental delay or mental retardation (2) Hearing impairment (3) Environmental deprivation (4) Pervasive developmental disorders, including autism spectrum disorders

Stages in cognitive development

1) The sensorimotor period (birth to age 2 years) 2) The stage of functional play (begins at about 1 year of age) 3) The stage of imaginative play (24-30 months) 4) Concrete thinking (during preschool and early elementary years) 5) Abstract thinking (during adolescent years)

Cryptorchidism +assc conditions + major risk

1) Undescended testes 2)associated with inguinal hernia, genitourinary malformations, hypospadias, and genetic syndromes In most males with cryptorchidism, the testes descend spontaneously before 12 months of age. Cryptorchid testes that do not descend by this age are predisposed to future malignancy.

What fraction of females are sexually active by end of high school? What fraction of adolescent females become pregnant?

1)50% sexually active 2) 1/9 adolescents become sexually active

pharyngitis 1. viral causes 2. bacterial causes 3. is it possible to tell what type of organism is causing the pharyngitis based on clinical presentation

1, cox, EBV, CMV 2. s. pyogenes, arcanobacterium hemolyticum, c.diptheriae 3. NO NO NO

1. most common cause of hypretension in children age 1-10 years vs adolescents

1-10 = renal disease/coarctation of the aorta adolescents = renal disease and essential hypertension

How long after menarche are menstrual cycles irregular?

1-2 years •because of the lack of consistent ovulation.

favorable prognostic factors for ALL

1-9 years of age female white <50k wbcs hyperploidy no organ involvment/ no mediastinal mass CALLA(+) no chromosomal translocation (9;22 is bad)

Chronic lung disease (bronchopulmonary dysplasia) 1. causes 2. pathogenesis + clinical presentation

1. *** RDS in premature infants (MCC) acute lung injury(mechanical ventilation, meconium aspiration, infection), secondary lung injury (oxidants/proteases) follows acute lung injury 2. lung damage (all of the above) --> abnormal healing/parenchymal remodeling --> diminished oxygenation, hypercarbia, intermittent episodes of tachypnea/weakness, frequent respiratory tract infections-- BOTH OBSTRUCTIVE AND RESTRICTIVE LUNG DISEASE

GERD 1. diagnosis

1. *barium upper gastrointestinal study shows ASPIRATION - reflux of the barium to the oropharynx is just due to transient lower esophageal sphinger realxation during the study and is not indicative of GERD *scintigraphy - technetium 99m mixed with food measrues rate of gastric emptying, and radioactive material in the lungs indicates aspiration *pH probe measurement - measure NUMBER OF TOTAL ASPIRATION EPISODES *endoscopy/biopsy * bronchoscopy with lavage to detect aspiration

1. physical exam findings of acyanotic congenital heart disease 2. complications

1. *increased right ventricular mpulse *systolic ejection murmur (through pulmonary artery) at the mid/upper left sternalborder *fixed split S2 - due to excessive pulmonary blood flow 2. right heart failure, pulmonary hypertension, atrial dysrhythmias,

1. physical exam findings of shock 2. historical features of shock

1. +/- abnormal blood pressure (normal early), tachycardia**, tachypnea**, altered mental status, capillary refil is slow, cool/mottled* extremities, bounding peripheral pulses (early) 2.vomiting/diarrhea, trauma/hemorrhage, febrile illness*, CHF sx, exposure to allergen, spine injury

Periods of speech development 1. prespeech period 2. naming period 3. work combining period

1. 0-10 months - musical like vowels (cooing), + mixing consonants and vowels (babbeling) 2. 10-18 months - infant understands that people have names and objects have identities 3. 18-24 months - early word combinations are telegraphic ~ combinatiosn occur 6-8 months after first words

bruises 1. red/blue, blue/purple, green, yellow/brown bruise ages *inflicted bruises tend to be on protected areas: face, neck, back, chest, abdomen, buttocks, genetalia *human bite marks 2. what feature of burns leads to suspicion of abuse

1. 0-3 days, 3-5 days, 5-8 days, 8-14 days 2. clear line of demarcation, stocking/glove distribution (submersion), Accidental burns tend to be more splashlike and irregular

Expected Increase in Head Circumference 1. 0-2 months 2. 2-6 months 3. total increase at 12 months

1. 0.5 cm/week 2. 0.25cm/week 3. 12 cm since birth

most common age for 1. choking 2. drowning 3. pedestrian injuries 4. baby walker injuries 5. accidental poisoning

1. 1 year 2. 2 years 3. 6 years 4. 6 month 5. 2 years

Cystic fibrosis 1. frequency of disease/carrier 2. length of survival

1. 1/2500 caucasians effected, 1/20 carrier in caucasians 2. average 31 years

ovarian tumors 1. what fraction are malignant 2. what fraction of testicular tumors are malignant 3. treatmnet

1. 1/3 2. 2/3 3. surgery, chemotherapy, radiation therapy

early adolescence psychosocial development 1. age 2. features

1. 10-13 years of age 2. early shift to independence from parents, pre-occupation with pubertal body changes, same sex peer relationships, abstract thinking

1. what fraction of fetuses are exposed to drugs in utero? 2. what obstetric complications? 3. clinical presentation

1. 10-15% 2. abruptio placentae, precipitous delivery, preterm labor 3. jitteriness, hyperreflexia, irritability, tremulousness, feeding intolerance, excessive wakefulness Mortality rates range from 3 to 10% Causes of mortality include perinatal asphyxia, congenital anomalies, child abuse, and sudden infant death syndrome.

measels (rubeola) 1. clinical presentation 2. complications 3. tx

1. 12 day incubation --> cough, conjunctivitis, coryza (THREE Cs ****), fever >101 Koplik spots (gray papules on erythematous base) --> erythematous maculopapular rash that STARTS ON NECK/EARS THEN EXTENDS TO CHEST/UPPER EXTREMITIES IN FIRST 24 HOURS THEN LOWER LIMBS BY 48 HOURS--> rash resolves by day 4-7 2. bacterial pneumonia (hemorrhagic), encephalomyelitis, otitis media, laryngotracheitis, 3. vitamin A, immunoglobulin for post exposure prophylaxis

middle adolescnece psychosocial developmetn 1. age 2. features

1. 14-17 years 2. inc. conflicts with parents, dec. preoccupation with pubertal changes, inc. desire to improve attractiveness, intense peer group involvement, initiation of romantic relationships, inc. abstract reasoning/risk taking

late adolescnece psychosocial development 1. age 2. features

1. 18-21 2. dev. of self as distinct from parents, more likely to seek advice from parents, comfort with body image,shared intimate relationships, abstract thought processes, abstract thought, fewer risk taking behaviros

1. timing of growth spurt in males vs females 2. timing of onset of puberty in males vs females

1. 18-24 months earlier in females 2. 6-12 months earlier (9.5 in females)

bacterial meningitis in infants/young children 1. most common age 2.risk factors 3. clinical presentation

1. 1st month of life 2. young age, immunodeficiency, complement deficiency, ventriculoperitoneal shunt, basilar skull fracture 3. MINIMAL OR ABSENT FEVER, BULGING FONTANELLE****, non-specific signs/symptoms (fussiness, poor feeding, irritabilty, lethargy, respiratory distress

PPD results when to call it a positive size of induration (not erythema) 1. how long after exposure does it take for a PPD to turn positive 2. what pts is >5mm positive 3. what pts is >10mm positive 4. what pts is >15mm positive

1. 2-12 weeks 2. children who have close contact with individuals, + chest radiograph, or immunocompromised 3. children <4 years of age, chronic medical conditions, or live in endemic, or work in institution, or healthcare 4. children older than 4 years with no risk factors

rubella (togavirus) 1. clinical presentation in children 2. complications

1. 2-3 week incubation period --> astymptomatic or fever <101,mild URI, suboccipital/ posterior auricular/cervical lymphadenopathy**, non-pruruitic, maculopapular and confluent exanthem with same progression as measels (head to toe/extremities) 2. meningoencephalitis, polyarteritis**

1.What is the average duration of growth spurt? 2. when in maturation does growth spurt occur 3. what hormones control this process 4. what fraction of adult weight/height is gained in adolescence

1. 2-3 years 2. adolescence 3. GROWTH HORMONE, also insulin/thyroid/sex contribute 4. 50% of ideal body weight, 25% of adult height

SIDS 1. peak incidence + clinical presentation 2. treatment 3. autopsy findings

1. 2-4 months, child is dead after being put to sleep at night 2. resuscitation on all patients 3. intrathoracic petechiae, puulmonary congestion ,small airway inflammation, hypoxia

digeorge sydnrome 1. pathogenesis + clinical presentation

1. 22q11 deletion--> loss of 3rd/4th pharyngeal pouches--> CATCH22- cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia

Toilet Training 1. Age for bowel control? 2. Age for bladder control?

1. 29 months of age (average of 16-48 months) 2. 32 months of age (average of 18-60 months) Methods: Stress encouragement, praise, and patience. Avoid shaming or blaming the child a. Allow multiple practice tries with praise for cooperation. b. Avoid pressure or force

Malrotation/midgut volvulous 1. epidemiology 2. associatted conditions 3. pathogenesis 4. clinical presentation + CXR

1. 2:1 male predominance 2. heterotaxy, small bowel atresia, hirschprung, intussusception 3. @ 10 weeks when the gut returns back to the abdominal cavity through the midgut~ this processes is interrupted --> LADDS BANDS that compress the duodenum, and voluvulus at the small bowel near the SMA 4. bilous vomitting in an apprantly healthy infant, +/- peritoneal signs on physical exam, bowel ischemia-->shock/ cardiovascular collapse + CURLY Q ABDOMINAL RADIOGRAPH

nephrotic syndrome 1. lab findings of nephrotic syndrome 2. electrolytes 3. ultrasound 4. tx - 5% mortality for nephrotic syndrome - in pts that are steroid resistant, or have thrombosis~~~ most common cause of death is BACTERIAL PERITONITIS + THROMBOEMBOLIC EVENTS

1. 3+/4+ proteinuria, microscopic hematuria, elevated TP/CR, RBC casts (not in MCD), elevated hematocrit (from hypoproteinemia), thrombocytosis, hypercholesterolemia, 2. metabolic acidosis (RTA) 3. enlarged kidnesy 4. supportive, 25% albumin for hypotension, edema, or pleural effusions, corticosteroids, cyclophosphamide, cyclosporine

1. what fraction of patients that have one febrile seizure will have another 2. treatment of recurrent febrile seizures (should not treat an isolated febrile seizure)

1. 30%, 2% risk of epilepsy 2. rectal diazepam, prophylaxis with valproic acid or phenobarbital

1. what age does aspiration occur at highest frequency 2. clinical presentation of laryngotracheal foreign bodies 3. bronchial foreign bodies clinical presentation 4. is CXR diagnostic for aspiration

1. 3months - 5 years 2. stridor, cough, hoareness 3. asymmetric auscultation findings, partial ball valve, wheezing, persistent pneumonia 4. only 15% of the time

absence epilepsy of childhood 1. pathogenesis + clinical presentation/age 2. EEG 3. tx -good prognosis

1. 5-9 years, AUTO DOM w/ age dependent penetrance ---> absence seizures lasting 5-10 seconds occuring frequently w/ automatisms NO LOSS OF POSTURE/INCONTINENCE/POST ICTAL STATE 2. 3Hz spike and wave discharge from both hemispheres (generalized) 3. ethosuximide or valproate

1. what is basal metabolic rate in cal/kg*day for: newborn, 1wk-6mo, 6mo-1year, 2. what is approximate caloric number needed for growth and activity for ages 1-3 3. what is typical caloric density for formula

1. 50, 65, 60 2. 100 cal/day 3. 0.67 calories/ml

Wilsons disease 1. patho + clincal pres 2. dx/tx

1. AR defect in ceruloplasmin copper excretion--> copper depositoin in the brain, eyes, heart, liver*** kaiser fleishcer rings, dystonia, dysarthria, tremors, ataxia, seizures, hepatic dizease, fanconi syndrome, changes in behavior 2. dec. ceruloplasmin**, elevated serum/urine copper, liver biopsy shows copper tx = avoid copper containing food chelation with oral penicillamine + zinc salts

celiac disease 1. age of onset 2. pathogenesis + clinical pres 3. diagnosis 4. consequences of not maintaining a gluten free diet

1. 6 months - 2 years 2. wheat, barley, rye, and oats contaminated with wheat --> autoimmune intolerance to gluten in the proximal small intenstine --> diarrhea, vomiting, bLoating, anorexia, FTT, large foul smelling stools, DERMATITIS HERPETIFORMIS**, HYPOCHROMIC MICROCYTIC ANEMIA 3. small bowel biopsy, IgA anti-endoymysial, IgA anti transglutaminase, IgG anti gliadin 4. growth failure, delayed sexual maturity

proteinuria total protein:creatinine ratio 1. normal TP/CR for infants/children

1. 6-24 months is <0.5, >2 years <0.2 is normal

1. age of onset of female puberty 2. thelarche 3. adrenarche 4. menarche

1. 7-13 years of age 2. breast development (breast buds = FIRST SIGN) 3. pubic/axillary hair development from adrenal androgens 4. onset of menstruation, mean 12.5 years (9-15 range)

post streptococcal glomerulonephritis 1. pathogenesis + clinical presentaiton -recorvery in 6-8 weeks, chronic renal failure rare 2. lab findings

1. 8-14 after nephritogenic strain of group A B-hemolytic strep skin/pharynx infection --> immune complex deposition+ complement in the glomerulus--> hematuria, proteinuria, hypertension, edema (HERAN) 2. low serum complement (low C3,low CH50, C4 normal), +ASO titer (only 50% positive after impetigo), anti-DNase B (positive after respiratory and skin infection)

Kawasaki disease -MCC acquired heard disease in the US -asians, M>F 1. what age does this occur 2. diagnositic criteria

1. 80% of cases younger than age 2. Fever >102 for > 5 days 4/5: non-exudative bilateral conjunctivitis with limbic sparing oropharyngeal changes- pharyngitis, strawberry tongue, red, cracked swollen lips cervical adenopathy polymorphous rash distal extremity changes - brawny edema/induration of hands/feet with erythema of the palms/soles late - (7-10 days) peeling around nail beds of distal extremities ***exclude other causes

toddlers fracture 1. pathogenesis + clinical presentation 2. dx/tx

1. 9 months - 3 years child trips and falls while running--> spiral fracture of the tibia 2. radiographs, put in a cast for 3-4 weeks

What fraction of high school senioirs have: 1. tried alcohol 2. tried illegal drugs 3. tried cigarettes

1. 90% 2. 50% 3. 60%

Asthma (Reactive Airway Disease) 1. pathogenesis + clinical presentation 2. what fraction of asthma patients are in remission by puberty 3. dx + CXR

1. 95% PRESENT BY 5 YEARS OF AGE chronic inflammatory --> bronchiolar smooth muscle constriction, airway mucosal edema, mucous plugging, inflammatory mediator production + HYPERRESPONSIVENESS TO STIMULI --> recurrent wheezing, cough, dyspnea, chest tightness ** symptoms are reversible spontaneously or with treatment, 2. 35% 3. therapeutic bronchodilator trial CXR shows hyperinflation, peribronchial thickening, , PFTs show dec. expiratory flow

pit viper snakes 1. pathogenesis + clinical presentation 2. treatment

1. 95% of snake bites, rattlesnake, cottonmouth, copperhead-> proteolytic enzyme venom --> swelling and eccymosis at site of bite --> paresthesias of scalp, periorbital fasiculations, weakness, diaphoresis, dizziness, METALLIC TASETE IN MOUTH, coagulopathy, thrombocytopenia, hypotenision, shock 2. crotalidae polyvaent immune Fab

Cardiac examination in neonates 1. normal heart rate 2. decreased femoral pulses 3. increased femoral pulses

1. 95-180 bpm (varies during feeding, sleep, or crying) 2. Coarctation of the aorta 3. Patent ductus arteriosus

juvenile rheumatoid arthritis - > in females, males >systemic onset/late onset 1. clinical presentation pauciarticular 2. general JRA laboratory findings

1. < or equal to 4 joints involved usually knees and hips, may be asymmetric Early - female predominant - chronic uveititis** (iris/ciliary body), +ANA, risk of blindness Late - male predominant - HLA B27 positive, hips/sacroiliac joint involvment (less uveitis), risk of spondyloarthritis** 2. microcytic hypochromic aneima (AOCD), elevated ESR/CRP/platelets, RF usually negative, ANA 75% postive in early onset pauciarticular, and 50% polyarticular

early neonatal hypocalcemia

1. <4 days old, prematuity, IUGR, asphyxia, maternal diabetes, hypomagnesemia

1. hypoglycemia definition 2. clinical presetnation

1. <40mg/dL in serum 2. lethargy, myoclonic jerks, cyanosis, apnea, seizures

1. hypocalcemia lab def 2. clinical features of hypocalcemia

1. <8 mg/dl or <2.5 mg/dl 2. tetany, carpopedal spasm, laryngospasm, paresthesias, seizures, long QT

Cystic fibrosis 1. dx/tx

1. >1 phenotypic features, increased immunoreactive trypsinogen on newborn screen, sweat chloride test (>60mmol/L), CF mutations, nasal epithelium ion transport abnormalitiy TX = antibiotics, bronchodilators, ADEK supplementatoin, oxygen, anti-inflammatory therapy, lung transplant, psychological support

what colony counts are significant in 1. suprapubic tap 2. midstream clean catch

1. >10^3 - 10^4 2. >10^5 in asymptomatic or >10^4 in symptomatic

juvenile rheumatoid arthritis 1. clinical presentation polyarticular JRA 2. general JRA laboratory findings

1. >4 joints, mild/absent extraarticular features, symmetric polyarthritis both small and large joints RF(-) - >females - early/late onset RF(+)- >females - late onseth - high risk of severe arthritis 2. microcytic hypochromic aneima (AOCD), elevated ESR/CRP/platelets, RF usually negative, ANA 75% postive in early onset pauciarticular, and 50% polyarticular

Neonatal jaundice 1. how high must bilirubin be to see jaundice 2. physiologic jaundice of the newborn 3. cause of PJN

1. >5mg/dl 2. indirect hyperbilirubinemia •in full-term,peaks @ day 3-4 (@5-16mg/dL) and resolves by the end of the first week of life, no treatment neccessary •in preterm, the peak bilirubin is at 5-7 days and will decrease by 10-20 days 3. inc. bilirubin load on hepatocytes, delayed maturation of the glucuronyltransferase enzyme

Werdnig hoffman disease 1. patho + clinical pres

1. AR destruction of anterior horn cells -->rapid motor weakness that begins in infancy--> weakness/paralysis, hyporeflexia, muslce fasciculations ~~ slower version = kugelberg welander

Gauchers disease 1. patho + clinical pres

1. AR glucocerebrosidase deficiency -- > glucocerebroside accumulation in spleen, liver, and erlenmeyer flask shaped distal femur, tissue macrophages --> leukopenia/anemia/thrombocytopenia from replacement of the marrow

Growth hormone deficiency (patho short stature) 1. clinical features 2. causes 3. evaluation 4. Management

1. A hx of prolonged neonatal jaundice, hypoglycemia, cherubic facies, central obesity, microphallus,cryptorchidism, and midline defects (e.g., cleft palate) Growth curve demonstrates poor growth velocity (<2in/yr) 2. brain tumors (e.g. craniopharyngioma), prior CNS irradiation, CNS vascular malformations, autoimmune diseases, trauma, and congenital midline defects. 3. a) Imaging studies -MRI of the head to rule out a CNS lesion -Delayed bone age b)Laboratory studies -Low IGF-1 levels -poor response on GH stimulation testing (with L-dopa-Inderal, glucagon, or clonidine) 4. daily subcutaneous injections of recombinant GH

evalulation of comatose patient

1. ABCs 2. serum glucose 3. urine toxicology screen + serum electrolytes + metabolic panel 4. lumbar puncture to rule out meningoencephalitis 5. EEG to rule out seizures

1. emergency treatment of burns on presentation 2. fist degree burn treamtnet 3. second degree burn treatment 4. third degree burn treatment

1. ABCs- endotracheal tubation for suspected inhaled hot gases, 100% oxygen/pulse OX, intravenous access + fluid ressusitation (due to rapid loss of lost through burned skin) 2. moisturizer/analgesics 3. analgesiscs, debridement of dead skin and popped bullae, dont pop unruptured bullae. 4. skin graft, hydrotherpy, escharotomy (surgical removal of constricting scar tissue)

Marfans syndrome 1. pathogenesis 2. clinical presentation 3. what other dz has similar presentation

1. AD connective tissue disorder that affects primarily the ocular, cardiovascular, and skeletal systems. -gene defect has been mapped to a region on chromosome 15 that codes for fibrillin 2. tall stature, dec. U:L SEGMENT RATIO arachnodactyly, pectus/scoliosis/kyophosis, upward subluxation of lens, aortic root dilation, MVP, A. regurg 3. homocystinuria

Apert syndrome 1. clinical presentation

1. AD craniosynostosis + syndactyly of the fingers/toes

autosomal dominant polycystic kidney disease 1. clinical presentation

1. AD inheritance --> adulthood presentation with abdominal pain, flank mass, UTI, gross or microscopic hematuria, hypertension, renal insufficiency, CEREBRAL ANEURYSMS

congenital glaucoma 1. pathogenesis + clinical presentation 2. tx

1. AD maldevelopment of the trabecular meshwork, congenital rubella, aniridia --> presssure >15mmHg--> damage to the optic nerve and increase in the size of eye (b/c eye is elastic in children), --> tearing, enlarged cornea/corneal clouding, dull red reflex 2. surgery, beta agonists, carbonic anhydrase inhibitors

tuberous sclerosis 1. pathogenesis + clinical presentation

1. AD mutation --> cerebral sclerotic tubercles --> myoclonic seizures ASH LEAF SPOTS (hypopigmented oval shaped macules seen underwoods lamp ADENOMA SEBACUM - angiofibromas on the nose/face SHAGREEN PATCH - thickened orange peel like skin INFANTILE SPASMS,intracranial calcifications, renal cysts, cardiac rhabdomyomas( #1 pediatric cardiac tumor) retinal astrocytoma/hamartoma

SLE 1. lab findings 2. treatment 3. tx for patient with thrombosis/anti-phospholipid antiboides 4. cause of mortality

1. ANA+, RF+, anti -dsDNA (specific), anti-Sm (specific), anti-phospholipid (inc. risk for thrombosis), dec. C3/C4 in active disease 2. NSAIDS for inflammation, glucocorticoids (main tx), cyclophosphamide (nephritis), MTX, azathioprine, cyclosporine 3. heparin/warfarin 4. infection, renal failure, nephritis, CNS complications

Dysfunctional uterine bleeding (DUB) 1. pathogenesis 2. what other conditions can cause DUB 3. laboratory testing 4. treatment

1. ANOVULATORY cycles--> excessivly thickened endometrium-->spontaneous prolonged uterine bleeding with weak uterine contractions 2. ectopic pregnancy, PID/cervicitis, vWD, ITP, leiomyoma/endometriosis, salicylates, OCP, steroids, IUDs, retained condoms/ tampons, trauma 3. CBC, pregnancy test, test for chlamydia, gonorrhea, bleeding time 4. OCP/progestin only contraceptives for anemia, iron supplements, dilation and curretage

type 1 diabetes melitus 1. immunoglobulins 2. clinical presentation 3. dx 4. tx

1. ANTI-ISLET ANTIBOTIES anti - insulin aanti - glutamic acid decarboxylase ~~~ ATTACK THE ISLETS**** 2. polyuria, polydipsia, nocturia 3. blood sugar >200mg/dL with polyuria, polydipsia, weight loss, nocturia. 4. various types of insulin, insulin pumps, glucagon (for rescue),

acute otitis media 1. AOM vs OME 2. pathogens 3. clinical pres 4. dx/tx

1. AOM is an acute infection of the middle ear, OME is just fluid in the middle ear without infection 2. s.pneuo, NTHI, m.catarrhalis 3. fever, ear pain, dec. hearing +/- pus drainage if tympanic membrane perforates 4. PNEUMATIC OTOSCOPY-- TYPMPANIC MEMBRANE DOES NOT MOVE INDICATING FLUID IN THE MIDDLE EAR -- erythema/loss of tympanic membrane landmarks are less reliable tx = amoxicillin, or amox-clav, or cephalosporin or macroides

Cystic fibrosis 1. pathogenesis 2. clinical presentation 3. pulmonary function test findings

1. AR CFTR mutation (del DF508 chromosome 7) --> sodium/chloride transport dysfunction--> abnormal mucus production 2. progressive pulmonary insufficiency, pancreatic insufficiency, high sweat electrolytes, meconium ileus at birth, steatorrhea, FTT, chronic cough, dyspnea, lung hyperinflation, crackles, wheezing, digital clubbing/hypoxemia 3. decreased respiratory flow rates , decreased lung volumes (LATE)

ataxia telangiectasia 1. pathogenesis + clinical presentation 2. dx/tx

1. AR Chromosome 11 mutation--> defective DNA repair, defective DNA recomb/cell cycle--> cafe aulait, vitiligo, early gray hair, CID, progressive cerebellar ataxia, oculocoutaneous telangiectasias, malignancy (lymphoma/carcinoma), 2. low IgE***, low IgA***, skin test anergy/dec. proliferation to migotgens tx = treat infections, monitor for malignancy, avoid ionizing radiation

Hurler syndrome 1. patho + clinical pres 2.dx/tx

1. AR alpha L iduronidase deficiecy - hepatosplenomegaly/kyophosis, dystosis multiplex progressively coarsened facial features, frontal bossing, prominent sagital/metopic sutures, wide nasal bride, hydrocephalous CORNEAL CLOUDING , stiff/contracted joints 2. dx = dermatan/heparin sulfates in urine + dec. alpha L iduronidase tx = bone marrow transplant

CAH 1. pathogenesis + clinical presentation 2. dx

1. AR defect in adrenal cortex --> inc. production of androgens--> anorexia, vomitting, diarrhea, dehydration at 5-15 days of life 2. elevated 17 hydroxyprogesterone

1. scaling 2. crusting 3. excoriations

1. desquamation of stratum corneum 2. dried exudate/debris 3. linear erosions of the epidermis caused by fingernail scratches

Tay sachs disease 1. patho 2. clinical pres of infantile and adult onset 3. dx/tx

1. AR hexaminidase A def --> accumulation of GM2 ganglioside 2. EARLY INFANCY-->hyperacusis, increased startle cherry red macula, macrocephaly, progressive blindness, severe developmental delay * NO HEPATOSPLENOMEGALY***--> death by age 4 > 2 years of life ataxia, dysarthria, choreoathetosis~ NO cherry red macula --> chronic debilitation 3. inc GM2 ganglioside, dec. HEX A activity

autosomal recessive polycistic kidney disease (infantile) 1. clinical presentation -always progresses to renal insufficiency needing transplantation

1. AR inheritance --> oligohydrominos/pulmonary hypoplasia, enlarged cystic kidneys, severe hypertension, ****cirrhosis/portal hypertension***

Vitamin D dependent rickets 1. pathogenesis 2. labs

1. AR mutation of 1 alpha hydroxylase 2. high PTH, low vitamin D, low ca/phos, inc. alk phos

protein C/S deficiency 1 pathogenesis + clinical pres (hetero/homo) 2. dx/tx

1. AR/AD deficiency --> homozygotes- purpural fulminans= nonthrombotic cytopenic purpura, fever, shock, rapid skin bleeding/intravascular thrombosis heterozygtes - DVT or CNS thrombosis 2. protein C/S testing, tx = heparin, FFP, warfarin, purified protein C

Homocystinuria 1. pathogenesis 2 clinical pres 3. dx 4.tx

1. AR: cystathionine synthase deficiency 2. -Marfanoid body w/o arachnodactyly -DOWNWARD lens subluxation -hypercoagulability, THROMBOEMBOLISM*** of pulmonary/systemic arteries, and cerebral vasculature -mitral/aortic regurg (no dilation compare to marfans) -scoliosis -developmental delay 3. elevated methionine in the urine, or +ve urinary cyanide nitroprusside test 4. methionine restricted diet, aspirin (thromboembolism)

psoriasis 1. pathogenesis + clinical presentation 2. nail findings 3. treatment

1. AUTO DOM immune dysregulation --> epidermal proliferation --> SILVER SCALING papules/plaques on scalp, ears, elbows, knees that KOEBERNIZE, ARTHRITIS 2. pitting, distal thickening, lifting of nail bed 3. topical corticosteroids, UV light, vitamin D analogs, 3% salicylic acid, anthralin (downregulates EGF)

schwachman diamond syndrome 1. pathognesis + clinical presentation

1. AUTO REC: DEC. NEUTORPHIL CHEMOTAXIS, CYCLIC NEUTROPENIA, PANCREATIC EXOCRINE INSUFFICIENCY, RECURRENT SOFT TISSUE INFECTIONS, CHRONIC DIARRHEA, FTT

addisons disease 1. pathogenesis

1. AUTOIMMUNE DESTRUCTION - of the adrenal cortex by lymphocytic infiltration

benign rolandic epilepsy (benign centrotemporal epilepsy) 1. pathogenesis + clinical presentation/age 2. EEG + TX *good prognosis

1. AUTOSOMAL DOMINANT 3-13 years of age --> early morning hours, with oral/buccal manifestations (moaning, grunting, pooling or saliva) --> tonic clonic seizures 2. spike and sharp wave disturbance in the mid temporal/central regions tx= valproic acid, carbamezipine

hypertropic cardiomyopaty 1. pathognesis 2. what infants have transient septal hypertrophy 3. clinical presentaiton 4. what manuevers increase the murmur of HCM

1. AUTOSOMAL DOMINANT LVH, specifically asymmetric septal hypertrophy (idiopathic hypertrophic subaortic stenosis) w/o cardiac disease --> poor left ventricular filling + dyanmic left ventricular outflow tract obstruction --> myocardial ischemia 2. infants born to diabetic mothers 3. sudden cardiac death (MCC in athletes), syncope, chest pain, exercise, harsh SYSTLIC EJECTION MURMUR AT THE APEX 4. valsalva and standing these reduce LV volume and increase the outflow tract obstruction

migraine (MCC headache in children) 1. pathogenesis + clinical presentaation

1. AUTOSOMAL DOMINANT changes in cerebral blood flow DUE TO SERATONIN (5HT) RELEASE, SUB. P--> +/- AURA -->unilateral THROBBING headache associated with N/V, visual changes, streaks of light (fortifications), photo/phonophobia, normal neurologic examination

hereditary spherocytosis -eastern europeans 1. pathogenesis + clinical presentaiton 2. dx 3. tx

1. AUTOSOMAL DOMINANT mutation in SPECTRIN --> spherical shape RBC --> splenomegaly , pallor, pigmented gallstones, aplastic crisis (assc. Parvo B19), jaundice/anemia in infants 2. spherocytes on smear, INC. OSMOTIC FRAGILITY 3. splenectomy

medullary sponge kidney 1. pathogenesis + clinical presentation

1. AUTOSOMAL DOMINANT, asymptomatic or have hematuria, UTI, or nephrolithiasis

pyruvate kinase deficiency 1. pathogenesis+ clinical presentation 2. dx 3. tx

1. AUTOSOMAL RECESSIVE pyruvate kinase deficiency --> decreased ATP, and dec. RBC survival--> pallor, jaundice, splenomegaly, kernicterus 2. polychromatic RBCs, PK activity of RBCs 3. transfusions/splenectomy

Meconium ileus 1. pathogenesis 2. dx 3. tx

1. Abnormal accumulation of intestinal secretions + deficiency of pancreatic enzymes causes inc. viscosity of meconium ⇒ occlusion of the distal ileum 2. abdominal radiograph showing distension of intestine with no air fluid levels ⇒ SOAP BUBBLES - from fine gas bubbles mixed meconium 3. enemas

Newborn chest examination

1. Accessory nipples 2. Congenital deformities (pectus carinatum and pectus excavatum) 3. Chest asymmetry (poland syndrome)

Short stature physical examination

1. Accurate height and weight plotted on a NCHS growth chart 2. Patient's upper-to-lower (U/L) body segment ratio. a. Lower segment = pubic symphysis to the heel b. Upper segment = total height minus lower segment c. Normal ratios: (1) Birth = 1.7 (2) 3 years of age = 1.3 (3) >7 years of age=1.0 d. Abnormal U/L ratio suggests disproportionate short stature 3. Thorough physical examination should include a funduscopic examination, assessment of thyroid size,evaluation for stigmata of genetic syndromes (e.g., web neck, shield chest, and short fourth metacarpals are suggestive of Turner syndrome; scoliosis screening, and Tanner staging

fanconi anemia 1. pathogenesis + clinical pres 2. dx 3. tx

1. Autosomal recessive --> marrow failure at age 7 ***presents with ecchymosis/petechiae** short stature, hypoplasia of the thumb* and radius, skin hyperpigmentation, renal abnormalities 2. pancytopenia, RBC macrocytosis, low reticulocyte count, elevated HbF, bone marrow hypocellularity 3. transfusions/platelets, BMT, corticosteroids Associated deformities include cafe-au-lait spots, microcephaly, microphthalmia, short stature, horse shoe kidneys and absent thumbs (not triphalangeal thumbs)

1. obstructive lung defects 2. restrictive lung defects

1. decreased airflow - asthma, bronchiolitis, foreign bodies, COPD 2. decreased ability to inc. lung volume - pulmonary edema, scoliosis, pulmonary fibrosis, respiratory muscle weakness

b12 anemia 1. normal physiology 2. pathogenesis + clinical pres

1. B12 + intrinsic factor (from parietal cells)--> absorption in the terminal ileum 2. strict vegan/pernicious anemia(no GIF)/crohns (term.ileum)--> inability to absorb B12-->smooth red tongue, combined system degeneration (ataxia, hyporeflexia, + babinski)

duodenal hematoma

1. BICYCLE HANDLEBAR INJURY --> right upper quadrant injury --> abdominal pain, vomiting, bowel obstruction on radiographic evaluation

Chlamydia trachomatis cervicitis 1. pathogenesis 2. clinical presentation 3. diagnosis 4. complications 5. tx

1. C. trachomatis transmitted as an STD intracellular pathogen of the endocervix 2. 50% males, 75% females asymptomatic ⇒purulent endocervical discharge; friable, edematous erythematous cervix, dysuria/urinary freq, 3. endocervical culture (gold standard), rapid antigen detection by fluorescent antibody or ELISA, PCR 4. PID, tubo-ovarian abscess (TOA), ECTOPIC PREG, fitz-hugh-curtis syndrome (perihepatitis) 5. oral doxycycline, erythro or azithro + TREAT PARTNER

Pelvic Inflammatory Disease (PID) 1. pathogensis 2. when in the menstrual cycle is PID most common 3. clinical presentation 4. tx

1. C. trachomatis, and N. gonorrhea ascending infection that spreads to uterus/fallopian tubes 2. first half (less cervical mucus) 3 .lower abdominal pain, cervical motion tenderness, adnexal tenderness + fever, leucocytosis >10k, elevated ESR/CRP, pos. culture 4. in patient - IV cefoxitin + oral doxy or IV clinda + gentamycin outpatient - 14 day oflaxacin, clindamycin, or single dose IM ceftriaxone + 14 days doxycycline

Differential diagnosis of ambiguous genitalia in the virilized female

1. CAH caused by 21-hydroxylase deficiency 2. Virilizing drug used by mother during pregnancy 3. Virilizing tumor in mother during pregnancy

1. laboratory evaluation of organic/non-organic abdominal pain 2. treatment *poor prognosis, only 50% of children will have resolution of symptoms

1. CBC, electrolytes, liver function, fecal occult blood, ova/parasites, ESR/CRP, h.pylori screening, lactose breath test 2/ normalization of childs activities, education of family, counseling MEDICATIONS ARE INEFFECTIVE

carbon monoxide poisoning 1. parthenogenesis 2. clinical presentation 3. perminant damage from CO poisoning

1. CO displaces oxygen from hemoglobin forming CARBOXYHEMOGLOBIN which does not carry oxygen--> left shifted oxygen dissociation curve , 2. CHERRY RED SKIN (due to inc. venous O2 content), retinal hemorrhages, tachycardia, tachypnea, syncope, slurred speech, cyanosis 3. memory loss, personality change, deafness, seizures

Cardiogenic shock

1. CO is limited due to cardiac dysfunction: dysrhythmias, congenital heart disease, cardiac dysfunction after surgery ~~ presents as CHF

RDS 1. Evaluation 2. Management

1. CXR - shows atelectasis with inc. density in both lungs GROUND GLASS APPEARANCE AND HYALINE MEMBRANES, AIR BRONCHOGRAMS (remaining patent airways surrounded by inc. density of pulmonary fields 2. supplemental oxygen, CPAP, mechanial ventilation (for resp. acidosis), exogeneous surfactant

Newborn General Appearance

1. Careful observation is necessary to assess spontaneous activity, passive muscle tone, respirations, and abnormal signs, such as cyanosis, intercostal muscle retractions, or meconium staining. 2. Apgar scores

Defects of multifactorial inheritance

1. Cleft lip and palate 2. Neural tube defects (NTDs) are the most common congenital anomalies of the central nervous system 3. Congenital heart disease (CHD)

talipes equinovarus (clubfoot) 1. pathogenesis + clinical presentation 2. treatment

1. DDH/myelomeningocele/myotonic dystrophy/skeletal dysplasia/genetics, abnormal positioning... multifactorial --> FIXED foot inversion/plantarflexion with no flexibility (compare to metatarsus adductus), 50% bilateral 2. casting in the first week of life, surgical if this doesnt work (MUST BE DONE EARLY****)

Effects of attention problems

1. Difficulty conforming to classroom routine 2. Social adjustment problems 3. Damage to self-esteem 4. Impaired relationships with parents and peers 5. Difficulty learning 6. Possible comorbidities: anxiety, conduct disorder, oppositional-defiant disorder, and obsessive-compulsive disorder

infectious mononucleosis 1. pathogenesis + clinical pres 2. dx 3. tx

1. EBV (most commonly), also less commonly toxo,CMV,HIV in saliva --> infection of B lymphocytes--> fever, malaise/fatigue, pharyngitis(exudative), POSTERIOR CERVICAL LYMPHADENOPAHTY, hepatosplenomegaly***, macular or scarlatiniform rash (rare) 2. monospot test identifying heterophile antibodies (agglutinates sheep RBC) IgG anti VCA, EA and EBNA IgM anti VCA with no* EBNA = acute infection atypical lympohcytes on smear, neutropenia, thrombocytopenia, inc. AST/ALT 3. supportive, corticosteroids for sever pharyngitis

hodgkins disease 1. pathogenesis + clinical presentation 2. is extranodal/SVC syndrome/airway compression common in hodgkins disease 3. dx/tx 4. complications of therapy - 60-70% survival

1. EBV infection --> infection of APCs --> REED STERNBERG CELLS- slow progressing painless, SYSTEMIC B SYMPTOMS, cervical/supraclavicular lymphoadenopathy, PROGRESSIVE COUGH (pulmonary symptoms is highly suggestive of hodgkins disease) 2. no 3. INITIAL CXR SHOWING MEDIASTINAL MASS lymph notde biopsy shows reed sternberg cells tx =chemotherapy + radiation 4. growth retardation, secondary malignancies (breast, AML, non-hodgkins), hypothyroidism, male sterility

dx. hypertrophic cardiomyopathy tx. hypertrophic cardiomyopahty

1. ECG: LVH, ST/T wave changes, LAD, deep and wide Q waves, in the inferior and lateral leads, Echocardiogram shows hypertrophy 2. B blockers, CCBs decrease LVOT obstruction surgical myomectomy antiarrythmics (to prevent ventricular arrythmias) dual chamber pacing

1. diagnosis of seizure 2. evaluation for patient with history of >1 afebrile seizures 3. evaluation of patient with febrile seizure

1. EEG, video EEG 2. serum electrolytes/neuroimaging 3. CNS infection by spinal tap, CBC, CXR, urine/blood culture

congenital myotonic dystrophy 1. dx/tx -infant mortality 40% due to respiratory problems, feeding improves with time

1. ELEVATED CPK***, hypotonia, examine the mother, DNA testing

Down syndrome (trisomy 21)

1. atlantoaxial instability***, leukemia, celiac disease, OSA, conductive hearing loss, hypothyroidism, cataracts, glaucoma, ~ epicanthal folds, upslanting palpebral fissures,protruding tongue, hypotonia, clinodactyly, palmar creases, duodenal atresia, hirschprungs

1. rheumatic fever lab findings 2. treatment - eventually cardiac damage/valvular insufficiency may require valve replacement

1. ESR/CRP elevated, leukocytoisis, ASO titers/anti-DNase,anti-hyaluronidase elevated, echocardiography shows decreased ventricular function, 2. benzathine penicillin, or oral penicillin NSAIDS (after DX) corticosteroids for severe cardiac involvement diuretics,digoxin, salt restriction for CHF haloperidol for syndenhams chorea

cushing 'syndrome'

1. EXCESS GLUCOCORTICOIDS BY BENING/MALIGNANT ADRENAL TUMORS

compartment syndrome 1. pathogenesis + clinical presentation 2. dx/tx

1. FOOSH injury (supracondylar fracture)--> pressure in the anterior fascial compartment >30-45 mmHg--> ischemia/volkmanns contracture pain with passive extension of the fingers + "the 5Ps" (pain, pallor, poikilothermia, paresthesias, pulselessness, paralysis)

1. what are the sources of floride 2. what is clinical presentation of excess fluoride 3. when is a child most succeptible to fluorosis 4. what children should get flouride supplementation

1. Fluoride supplements, fluoridated water, and fluoride toothpaste 2. Fluorosis = cosmetic white streaks/brown staining affecting permanent teeth 3. Between 2 and 4 years of age 4. -Exclusively breastfed children > 6 mo of age -Children who live in areas where tap water contains < 0.3 ppm fluoride.

pathogens causing sepsis/meningitis + tx 1. 0-1 month 2. 1-3 months 3. 3mo - 3yr 4. 3yr-adult

1. GBS, ecoli, listeria---amp + genta + cefotax + acylcovir (if cutaneous vesicles/apnea/seizures) 2. GBS, pneumo, listeria--- amp + cefotax + vanc 3. pneumo, h.flu, neisseria --- cefotax + vanc 4. penumo, neisseria --- cefotax + vanc

Breath holding spells 1. definition 2. age of onset 3. two subtypes 4. Management

1. HARMLESS INVOLUNTARY episodes in which child holds their breath long enough to alarm parent 2. 5% of children between age 6 and 18 months , disappears by 5 years of age cyanotic spells: precipitated by frustration/anger-->cyanosis-->may cause apnea/seizure (peaks @ 2 years of age) pallid spells: provoked by an unexpected event that frightens the child-->hypervasovagal response--> pale and limp 4. reassure parents, tell parents NOT to resuscitate, iron supplementation

gianotti-crosti syndrome (papular acrodermatitis 1. pathogenesis + clinical presentation 2. tx

1. HBV, EBV, CMV, coxsackie,--> red flat topped papules in acral areas

Increased ICP 1. clinical presentation 2. complications* 3. tx

1. HEADACHE (EARLY FIRST SIGN), PUPILLARY CHANGE, ALTERED MENTAL STATUS 2. transtentorial or uncal herniation into the infratentorial compartment --> bradycaria, blown pupil, contralateral hemiparesis, cushings triad (bradycardia, hypertension, irrecgular breathing) 3. hyperventilation with 100% oxygen, elevation of the head to 30-45 degrees to help with venous drainage diuretics neurosurgery

roseola infantum (exanthem subitum) 1. pathogenesis + clinical presentation 2. tx

1. HHV6/7, adeno, parvo, echo --> 3-5 days high fever--> pink papular eruption on the trunk that spreads to the extremities (fever is gone when rash comes) 2. supportive

epiglottitis 1. pathogenesis + clinical presetation 2. lab findings + CXR 3. tx

1. HITB, group A Beta hemolytic strep, s.pneumo, staph ---> inflammation/edema of the epiglottis/aryenoids, aryepiglottic folds --> high fever, muffled speech, dysphagia/drooling, tripod positioning, complete airway obstruction/respiratory arrest, CHERRY red epiglottis (seen during intubation) 2. left shift leukocytosis, blood culture, CXR appears like thumbprint on lateral neck radiograph 3. EMERGENCY, nasotracheal intubation, minimize stimulation, RIFAMPIN for HITB, 3rd gen cepahlosporins

warts 1. pathogenesis + clinical presentation 2. tx

1. HPV --> discrete flesh colored papules, condyloma acumulata = multiple external warts on genital area 2. liquid nitrogne, salicyclic acid, podophyllin, surgery

erythema multiforme minor 1. pathogenesis + clinical presentation 2. tx

1. HSV --> low grade fever, arthralgials/myalgias --> symmetric target lesions in an acral distribution + ONE mucous membrane finding (usually mouth) 2. acyclovir

primary ocular herpes 1. pathogenesis + clinical presentation 2. dx/tx

1. HSV1 exposure--> skin eruption + corneal ulcer 2. positive viral culture + systemic or topical acyclovir

microangiopathic hemolytic anemia 1. pathogenesis+ clinical presentation + dx

1. HUS, TTP, DIC, artificial heart valves, giant hemangioma --> mechanical damage to RBCs -->burr cells, target cells, iregularly shaped cells, helmet cells, thrombocytopenia

Newborn Craniofacial Examination

1. Head 2. Ears 3. Eyes 4. Nose 5. Mouth

henoch schonlein purpura 1. pathogenesis + clinical presentation 1-5% --> renal failure

1. IgA vascultiis --> non-thrombocytopenic palpable purpura on the buttocks, thighs, abdominal pain, arthritis, gross/microscopic hematuria

allergic rhinitis 1. pathogenesis + clinical presentaiton 2. seasonal rhinitis 3. perennial rhinitis 4. dx. 5. nasal smear findings

1. IgE mediated inflammatory response in the nasal mucosa to inhaled antigens 2. trees, grass, weed pollens (grass in spring, ragweed in fall) 3. indoor allergens - dust mites, animal dander 4. total IgE, allergen skin testing (no antihistamines before testing) 5. 10% eosinophils indicates rhinitis, high levels of PMNs suggests infectious not allergic

Transient tyrosinemia of the newborn 1. pathogenesis 2. clinical presentation 3. dx

1. In premature infants who receive high protein diet in first 2 weeks of life 2. poor feeding/letheragy 3. elevated serum tyrosine and phenylalanine levels 4. vitamin C, dec. protein intake *Self limited disease*

Differential diagnosis of ambiguous genitalia in undervirilized male

1. Inborn error in testosterone synthesis -inherited enzyme deficiencies resulting in low testosterone levels 2. Gonadal intersex -internal structures are a combination of both male and female structures •Mixed gonadal dysgenesis (MGD) •True hemaphroditism 3. Partial androgen insensitivity

Live vaccines 1. duration of immunity 2. contraindications 3. list live vaccines

1. LONG 2. contraindicated in patients with compromised immunity 3. MR VZ MAPSY - measels, rubella, varicella, zoster, mumps, adeno, polio (oral), yellow fever

What should be tested for in pathologic short stature?

1. Lab studies •complete CBC, ESR, T4, electrolytes •IGF-1 - an indirect test for growth hormone deficiency •chromosome analysis in girls to evaluate for Turner syndrome 2. Radiographic studies •Bone age (AP film of left hand and wrist to assess epiphyses or growth plates) •AP and lateral skull radiographs to assess pituitary gland 3. Key pearl •Patients with poor growth velocity with normal screening laboratory results but low IGF-1 and delayed bone age should have a workup for growth hormone deficiency

Newborn Neck and Clavicle Examination

1. Lateral neck 2. Midline clefts or masses 3. Neonatal torticollis 4. Edema and webbing 5. Clavicles

CVID 1. pathogenesis + clinical presentation 2. dx/tx

1. MATURE Bcell/Tcell dysfunction (normal counts) --> HYPOGAMMAGLOBULINEMIA--> respiratory infections (h.flu, m.catarrhalis, s.pneumo), GI (g.lambia, c.jejuni), rheumatoid arthitis, autoimmune thrombocyotopenia, autoimmune hemolytic aneima **malignancy 2. dec. immunoglobulin, dec. antibody function, dec. T cell mitogen proliferation tx = IVIG replacement, abx, diarrhea management

transient synovitis 1. pathogenesis + clinical presentation 2. dx/tx

1. MCC painful limp in toddlers- post infectous resposne of hip joint --> low grade fever, limp, mild irritability- similar limb positioning as septic arthritis, 2. WBC/ESR may be normal, aspirate/culture hip effusion tx = NSAID, bed rest, obs

1. hepatitis A disease 2. high risk grouups

1. MCC viral hepatitis on earth (up to 70% asymptomatic in children, more severe in older children/adults, RARELY fulminant hepatitis 2. homosexuals/bisexula, illicit drugs, clotting factor disorders reciving blood products, occupational

1. where is a human bite most likely located during a fist fight + pathogenesis 2. pathogens in human bites

1. MCP joint --> avascular facial layers -->deep infection/tendinitis 2. mixed bacterial: s. viridans, s.aureus, anarobes (bacteroides, peptostreptococcus, eikenella)

ASD ostium secundum (most common) 1. features

1. MIDDLE PORTION OF THE ATRIAL SEPTUM

parotitis 1. viral pathognes + clinical presentation 2. bacterial pathogens + clinical presentation

1. MUMPS, EBV, CMV, HIV, influenza~ BILATERAL PAROTITIS 2. s.aureus, s.pyogenes, M.tubuerculosis - unilateral parotid involvement

Genital warts 1. pathogenesis 2. clinical findings 3. dx 4. tx

1. Most common STD •HPV 6/11 cause warts •HPV 16/18 cause cervical cancer /warts •External genital warts are also termed condylomata acuminata. 2. Itching, pain, dyspareunia; visible on external genitalia; may be asymptomatic 3. warts ⇒ direct visual inspection; HPV⇒pap smear (koilocytes/ dysplasia/cervical cancer), 3% acetic acid wash during colposcopy (turns white) 4. cryotherapy, laser, topical podophyllin, trichloroacetate

Adverse effects of 1. most vaccines 2. MMR and varicella 3. sabin

1. Most vaccine side effects are mild to moderate in severity and occur within the first 24 hours after administration (e.g., local inflammation and low-grade fever). 2. fever and rash 1-2 weeks after immunization (i.e., after the incubation period of the virus). 3. vaccine related polio

transposition of the great arteries 1. physical examination 2. ECG 3. CXR 4. pathophysiology 5. tx

1. NO MURMUR (quiet precordium), single S2 (no split) 2. normal or RVH 3. small heart with narrow mediastinum**** (egg on a string), increased pulmonary vascular markings 4. systemic and pulmonary circuits are in parallel rather than series~~ OBLIGATE patent foramne ovale, ASD or VSD (determines the degree of cyanosis 5. arterial switch operation

HIV in infant 1. is an antibody titer that is positive for the first 2 years indicative of disease 2. dx of HIV in infant

1. NO THESE ARE MATERNAL ANTIBDOES 2. HIV DNA PCR, if negative at 4 months

GABHS pharyngitis 1. clinical presentation 2. dx/tx

1. NO URI symptoms, exudates on the tonsils, petichiae on the tonsils, strawberry tongue, and large tender ANTERIOR CERVICAL lymph nodes, fever, scarlatiniform rash 2. rapid strep test, or culture, penicillin VK, single dose of IM benzathine penicillin, or oral macrolides

hydrocephalus 1. pathogenesis

1. NON-COMMUNICATING (ex. aqueductal) COMMUNICATING (inc. production of CSF by tumors, or dec. absorption of CSF ex. meningitis) HYDROCEPHALOUS EX VACUO- brain atrophy leads to venricular enlargment

progesterone 1. effect in males 2. effect in females

1. NONE 2. converts endometrium to secretory endometrium

bacterial conjunctivitis 1. pathogenesis + clinical presentation 2. dx/tx

1. NTHI, s.pneumo, m. catarrhalis, s.aureus--> PURULENT discharge, +/- otitis media 2. dx = conjunctival cultures tx =topical antibiotics- sulfacetamide, polymixin B, TMP, gentamycin, tobramycin, erythromycin

hyponatremia isonatremia hypernatremia

1. Na < 130 2. Na 130-150 3. Na > 150

Dental Abnormalities

1. Neonatal and natal teeth 2. Nursing or bottle caries 3. Dental Trauma

lyme disease 1. pathogenesis 2. clinical presentation early/early disseminated/late

1. New england US borellia burgdorferi tick bite via ixodes tick 2. early = erythema chronicum migrans (target like annular rash), constitutional symptoms (headache, myaligias, fatigue, arthralgias,lymphadenopathy) early disseminated = multiple secondary erythema migrans, constitutional symptoms, FACIAL NERVE PALSY (UNI/BILATERAL), encephalitis, heart block/carditis (arrythmias), aseptic meningitis Late: arthritis

atypical HUS 1. causes -similar clinical presentation as shiga toxin HUS - higher risk of ESRD than

1. OCP, cyclosporine, tacrolimus, OKT3, inherited (AD/AR)

renal parenchymal glomeruluar damge 1. pathogenesis 2. lab findings

1. PSGN, lupus nephritis, HUS--> glomerular damage--> hematuria/proteinuria 2. hematuria/proteinuria

Short stature history

1. Perinatal history (prematurity or IUGR) 2. Chronic diseases (renal failure, CNS disease, SSD, severe asthma with frequent steroid use, IBD) 3. Chronic use of drugs (steroids, stimulants for ADHD) 4. Family history, especially parental growth and pubertal histories 5. Social Hx (neglected environments) 6. ROS (cold intolerance, constipation, headaches, etc) 7. Dental Hx (delayed dental eruption = delayed bone age)

1. osteogenic sarcoma pathogenesis -most common malignant bone tumor, peaks during puberty growth spurt 2. clinical associations 3. radiographic findings 4. sites of metastasis

1. Puberty during rapid bone growth--> tumor that forms osteoid (new bone) of the metaphysis** of long bones (proximal tibia/humerous/femur, distal femur) --> pain, swelling, soft tissue mass, no systemic symptoms*** 2. retinoblastoma, pagets disease, radiation for cancer, fibrous dysplasia 3. periosteal reaction with sunburst appearance, lytic/destructive changes 4. lungs, bone

long QT 1. diagnosis 2. treatment

1. QTC>0.44 seconds 2. beta blockers, cardiac pacing, left stellate ganglionectomy, automatic implantable defibrillator

coral snakes 1. pathogenesis + clinical presentation 2. treatment

1. RENEXT TO YELLOW, KILLA FELLOW, --> neurotoxic venum --> SEVERE SX = paresthesisas, vomiting, diplopia, weakness, fasiculations, confusion, respiratory depression 2. antivenin, local wound care, supportive care

Bronchiolitis 1. pathogenesis 2. CXR 3. complications

1. RSV (MCC), parainfluenza, adeno, rhino, infleunza, mycoplasma --> URI (fever/congestion/cough) --> LRI: inflammation of the bronchioles/bronchiolar obstruction - tachypnea, fine rales, wheezing, palpable SPLEEN AND LIVER (due to lung hyperinflation), hypoxemia, apnea 2. hyperinflation with air trapping, patchy infiltrates , atelectasis 3. APNEA, respiratory failure/death, bacterial superinfection

Varicella vaccine 1. timing

1. Recommended at 12-18 months

alloimmune hemolytic anemia 1. Rh hemolytic disease 2. tx

1. Rh(-) mother produces antibodies to Rh(+) fetus during first pregnancy ~~~ second pregnancy mothers IgG anti-Rh crosses placenta causing severe jaundice/kernicterus, anemia, hepatosplenomegaly, hydrops fetalis 2. phototherpay + RHOGAM for the mother during first pregnancy or after blood transfusion

toxic shock syndrome 1. pathogenesis/clinical presentation 2. tx

1. S.aureus (sometiems s.pyogenes) --> exotoxins + toxic shock syndrome toxin --> >101 fever, hypotension, diffuse erythroderma (sunburn like rash), desquamation + multisystem involvement (>3)--- gastrointestinal, myalgias, hyperemia, thrombocytopenia*** 2. anti-staph antibiotics, IVIG , treat the shock/nidus of infection

Candidal vulvovaginitis 1. clinical presentation 2. dx 3 tx

1. SEVERE ITCHING with white CURDLIKE discharge, vulvar/vaginal infalmation 2. fungal hyphae on wet mount, NORMALVAGINAL PH, positive yeast cultures 3. oral fluconazole, topical intravaginal anti-yeast therapies NO TREATMENT FOR PARTNERS

Fetal alcohol syndrome

1. SGA, FTT, microcephaly, smooth philtrum****, smooth thin upper lip, mental retardation, ADD/ADHD, VSD

N. gonorrhoeae cervicitis 1. pathogenesis 2. clinical presentations 3. dx 4. compliations 5. treatment

1. STD intracellular gram negative diplococcus of the endocervix 2. often asymptomatic ⇒MUCOPURULENT cervical discharge +/- vaginal bleeding, dysuria/urinary freq, dyspareunia 3. culture on THAYER MARTIN MEDIA shows intracellular gram negative diplococci, PCR, nucleic acid hybridization 4. PID, TOA, fitz hugh curtis syndrome, infertility, dissemination ⇒ asymmetric polyarthritis, papular/pustular skin lesions, meningitis, endocarditis, epsis 5. cetriaxone or ofloxacin, cefixime, ciprofloxacin + assume c.trochomatis co-infection + TREAT PARTNERS

type 2 DM 1. patho 2. clincial pres 3. tx

1. STRONG HEREDITARY COMPONENET** peripheral insulin resistance ---> progressive insulin decline 2. DKA (not serious), NKHHS, obesity, acanthosis nigricans (inc. insulin-->inc. IGF-->melanocyte prolif) 3. oral hypoglycemics, insulin

Management of hyperbilirubinemia in infants

1. Serial bilirubin assessments, observation, and reassurance are appropriate for physiologic jaundice. 2. Phototherapy, which creates water-soluble photoisomers of indirect bilirubin that are more readily excreted, may be indicated depending on the infant's gestational maturity, age, bilirubin levels, and risk factors, if present (e.g., blood group incompatibility and suspected sepsis). 3. Exchange transfusion is performed for rapidly rising bilirubin levels secondary to hemolytic disease.

Clinical features of ADHD

1. Symptoms before 7 years of age 2. Symptoms in more than one environment (e.g., school and home) 3. Impairment in functioning in school or in personal relationships 4. Symptoms of inattention, including not being able to focus during classroom instruction, difficulty with organization, and forgetfulness 5. Symptoms of hyperactivity, which may include fidgeting, acting as if driven by a motor, excessive talking, and difficulty remaining seated in the classroom 6. Symptoms of impulsivity, which may include behavior such as blurting out answers before a question is completed

female puberty 1. onset of puberty age + physical finding 2. onset of menstruation age

1. THELARCHE avg 9.5 years, development of breast buds, 2. MENARCHE - 12.5 (2-3 years after thelarche)

When should dental hygiene be initiated?

1. Tooth brushing should begin as soon as teeth erupt. • Once children are 2-3 years of age, they are often able to assist in brushing their own teeth. -A fluoride toothpaste may be used at this time 2. Dental floss should be initiated when tight contact exists between teeth. 3. Fluoride • Children who consume optimal amounts of fluoride from birth until adolescence have 50-75% less dental decay than expected.

Vaginitis 1. organisms 2. clinical findings 3. dx. 4. tx

1. Trichomonas vaginalis (STD), or bacterial or candidal (non-STD) 2. T. vaginalis is 50% asymptomatic, symptomatic odiferous yellow green discharge, petechiae covered "strawberry cervix", VULVAR INFLAMMATION/itching, dyspareunia 3. wet mount slide, T. vaginalis culture, vaginal pH <4.5 4. metranidazole for effected AND PARTNERS

pupillary size and reactivity indications 1. unilateral dilated non-reactive 2. bilateral dilated non-reactive pupils 3. bilateral constricted reactive

1. UNCAL HERNIATION 2. topical application of dilator, post ictal state, irreversible brainstem injury, epinephrine, 3. opiate ingestion, or pontine injury, coma, pilocarpine, horner syndrome, nerve gas, pesticides

hypertrophic pyloric stensois 1. epidemiology - may be associated with duodenal atresia, tracheoesophageal fistula, trisomy 18, cornelia de lange syndrome 2. pathogenesis 3. clinical presentation 4. treatment

1. UNKNOWN ETIOLOGY first born male children 4:1 female to male 2. thickening of pyloric muslce --> obstruction + PROJECTIVLE VOMITTING 3. nonbilous milky fluid vomiting, projectile vomiting, irritability, hunger, jaundice, dehydration - palpable olive, visible peristalsis, hypochloremic- hypokalemic-metabolic acidosis (from vomitting), ultrasound shows thiceked pylorou, UGI shows STRING SIGN (narrowed pyloric channel) 4. tx. dehydration, partial pyloromyotomy

AML 15-20% of childhood leukemias 1. pathogenesis 2. M1-M7 3. clinical presentation

1. UNKNOWN/down syndrome/fanconi/kostmann/neurofibromatosis/ionizing radiation 2. acute myeloblastic, acute myeloblastic some maturation, acute promyelocytic, acute myelomonocytic, acute monocytic, erythroleukemia, acute megakaryocytic 3. similar to ALL but with more CNS involvment~ fevers, HSM, gingival hypertrophy, bone pain, DIC******

1. diskitis + clinical presentation 2. dx/tx

1. URI/minor trauma --> infection of the intervertebral disk (s. aureus) --> tenderness above the affected disk, and refusal to flex the spine/ambulate 2. elevated ESR, MRI/bone scan, bed rest, anti-staphylococcal

brain tumors - most common solid dutmor - 20% of childhood cancers 1. which conditions are associated with brain tumors 2. are supra or infratentorial glial tumors more aggressive -astrocytomas 3. PNET - also ependymomas, and craniopharyngiomas

1. VHL, neurofibromatosis, tuberous scleorsis 2. supratentorial 3. medulloblastomas

brutons agammaglobulinemia 1. pathogenesis + clinical presentation 2. dx/tx

1. X linked BTK tyrosine kinase deficiency --> no development of mature B cells --> encapsulated infections, chronic enteroviral infections, no tonsils 2. dx - decrease in all**** immunoglobulin types, no B cells, T cells present with preserved function, BTK mutation tx = IVIG replacement monthly

glucose 6 phosphate DH deficinecy 1. pathogenesis+ clinical presentation 2. dx 3. tx

1. X linked G6PD deficiency (low reduced glutathione) :: fava beans, infection, sulfa/salacylates/antimalarials --> oxidative damage --> hemolysis--> abdominal pain, V/D, fever, hemoglobinuria/jaundice, HSM 2. hemoglobinuria, inc. Retics, bite cells, heinz bodies, hemighosts, low G6PD 3. transfusions

wiskott aldrich syndrome 1. pathogenesis + clinical presentation 2. dx/tx

1. X linked T cell receptor signaling/cytoskeleton organization--> CID, ECZEMA, congenital thrombocytopenia (micro platelets) --> frequent encapsulated organism infections, bleeding/hemorrhage, skin infections (from eczema) 2. CBC (small platelets/thrombocytopenia), low IgM***, elevated IgA/IgE*** defective polysaccharide antigen response, anergy with normal number of T cells, NO ANTIGEN SPECIFIC CYTOTOXIC T CELLS

chronic granulomatous disease 1. pathogenesis + clinical presentation 2. pathogens 2. dx/tx

1. X linked defect in NADPH oxidase --> imparied neutrophil oxidative metabolism --> impaired killing of CATALAE POS. BACTERIA AND FUNGI--> abcess formation with increased succeptability to lung/liver/bone/skin infection 2. s.aureus, psuedomaonas, salmonella, klebsiella, serratia, E.coli, c.albicans, aspergillus 3. nitroblue tetrazoleum test, flow cytometery tx = abscess drainage, TMPSMX/itraconazole prophylaxis, IFN-y, BMT***(curative)

wiskott aldrich syndrome 1. pathogenesis / clinical presentation 2. associated cancers

1. X linked defect in actin polymerization--> thrombocytopenia*, small platelets, eczema*, T/B cell immune defects 2. lymphoma, leukemia

Hunter syndrome 1. patho + clinical pres 2. dx/tx

1. X linked deficiency iduronate sulfatase --> hepatosplenomegaly, dystosis multiplex NO CORNEAL CLOUDING, 2. dx= dermatan sulfatates tx = none, death by age 15

factor IX deficiency 1. pathogenesis 3. treatment

1. X linked disorder, similar to VIII deficiency, PTT and PT -- 2. treat with recombinant factor IX

alports syndrome 1. pathogenesis

1. X linked dominant- defect in type IV collagen in the basement membrane --> ESRD, hypertension, hematuria, HEARING LOSS, ocular abnormalities of the lens and retina

duchennes/beckers muscular dystrophy 1. pathogenesis 2. clinical presentation

1. X linked recessive dystrophin mutation -->weakness/rupture of muscle plasma membrane --> degeneration/regeneration + infiltration with lymphocytes-->replacement of muscle fibers with fibroblasts/lipids 2. begins age 2-5 with progressive weakness starting in the legs --> loss of ability to walk by age 10 (20 for BMD), pseudohypertrophy of the calves, gowers sign (proximal hip muscle weakness), cardiomegaly/cardiac failure, mild cognitive impairement in DMD

hemophilia A 1. pathogenesis + clinical pres 2. dx 3. tx

1. X linked recessive factor VIII defieciency --< hearthrosis, DEEP soft tissue bleeding, life threatening hemorrhage 2. prolonged PTT, normal PT, normal bleeding time/platelet count/platelet function, low factor VIII activity 3. recombinant factor VIII, DDAVP (inc. release of stored VIII)

vitamin D resistant rickets 1. pathogenesis 2. labs 3. clinical pres **

1. XLD disorder, renal tubular phosphate leakage 2. normal calcium, low phosphorus, 3. bowing BUT NO TETANY***~ compare to vitamin D dependent rickets and secondary rickets

Menkes kinky hair disease 1. patho + clinical pres 2. dx

1. XLR abnormal copper transport -> low serum copper pale kinky friable hair, low serum copper, optic nerve atrophy, severe mental retardation 2. hair findings + low ceruloplasmin and copper

1. pathogenesis of acid ingestion 2. pathogenesis of alkali ingestion 3. clinical presentation

1. acids cause coagulative necrosis + superficial damage 2. liquifactive necrosis and deep penetrating damage 3. immediate burning -->dysphageia, salivation, chest pain, OBSTRUCTIVE AIRWAY EDEMA, gastric perforation, esophageal perforation with mediatinitis

stages of iron toxicity 1. 1-6 hours 2. 6-12 hours 3. 12-36 hours 4. 2-6 weeks

1. abdominal pain, vomiting, diarrhea, GI BLEEDING, shock from bleeding, fever, leukocytosis 2. resolution of stage 1 symptoms 3. metabolic acidosis, circulatory collapse, hepatic/renal failure, DIC, neurologic deterioration 4. late sequelae, pyloric or intestinal scarring with stenosis

1 developmental dysplasia of the hip - pathogenesis + clinical pres girls 6x> boys 2. dx 3. tx - complicatoins = avascular necrosis of femoral head, limb length discrepancy

1. abnormally flat acetabulum --> easy disolcation 2. postive barlow ("clunk"), positive ortolani, abnormal galeazzi sign (one knee higher than the other), asymmetric abduction of the hips 3. pavlik harness - hold head of femur against acetabulum, surgery (if pavlik fails)

1. clinical presentation of near drowning 2. what determines the degree of neurological defecit in a near drowning 3. hematologic sequelae of near drowing ~ renal failure can also occur

1. absent/irregular respirations, cough up pink/frothy material, rales/rhonchi/wheezes **pneumonia often develops within 24 hours from normal flora slow deterioration of pulmonary function 2. length/severtiy of hypoxia, pt. may be alert, agitative, combative, or comatose 3. hemolysis/DIC

chronic benign neutropneia of childhood - <4 years of age 1. pathogenesis/clinical pres 2. dx/tx

1. acquired/inherited non-cyclic neutropenia---> mild infections (otitis,sinusitis,pharyngitis, cellulitis) 2. low neutrophils, normal WBCs, bone marrow shows immature neutrophil precursors tx = nothing, resolves spontaneously

hepatitis B serology 1. HBsAg 2. IgG anti HbS 3. IgG anti HBc 4. HBeAg 5. IgG anti HBe

1. active disease 2. protective: from vaccination* or recovery from natural infection* 3. from natural infection ONLY persists life long (IgM early, IgG late) 4. rises early in active infections, useful to diagnose acute infection 5. rises late in infection

Testicular appendage torsion 1. clinical presentation 2. dx 3. tx

1. acute or gradual onset of testicular pain and tenderness most noticable in the upper testicle, BLUE DOT SIGN 2. doppler ultrasound/radionucleotide scan is NORMAL 3. rest and analgesia, resolves within 2-12 days

kawasaki disease 1. lab findings during acute/subacute/convalescent phase

1. acute phase 1-2 weeks- elevated ESR/CRP subacute phase weeks-months - dec. ESR/CRP, inc. platelets** convalescent phase weeks to years- normal lab values

viral conjunctivitis 1. pathogenesis + clinical presentation 2. tx

1. adenovirus 3 and 7 --> bilateral conjunctivitis, pharyngitis, fever--> watery conjunctival discharge, hyperemic conjunctiva, preauricular adenopathy 2. supportive treatment + NSAIDs,

epidemic keratoconjunctivitis 1. pathogenesis + clinical presentation 2. tx

1. adeovirus 8,19,37 --> petechial conjunctival hemorrhage, pseudomembrane on the conjuntiva, keratits/photophobia (hypersensitivity reaction) pre-auricular lymphadenopathy, NO FEVER OR PHARYNGITIS 2. supportive treatment

SLE females >males 8:1 1. when is peak age of onset 2. pathogenseis

1. adolecence 2. UNKNOWN, drug reactions, sun exposure, infections, hormonal changes--> widespread inflmmation of connective tissues and immune mediated vascultiis

Ewing sarcoma 1. pathogenesis + clinical presentation 2. radiographic findings 3. sites of metastasis

1. adolescence chromosomal translocation (11;21) --> undifferentiated small blue cell tumor in flat bones and diaphysis of tubular bones- AXIAL SKELETON, humerous, femur --> pain, swelling, FEVER, MALAISE, WEIGHT LOSS, leukocytosis, inc. ESR` 2. periosteal reactoin with onion skinning 3. lungs, bone, bone marrow

1. what two endocrine changes occur during puberty 2. what is adrenarche how long is puberty 3. what is true puberty

1. adrenarche and true puberty 2. onset of ADRENAL androgen synthesis, occurs 2 years before HPG axis turns on 3. LH and FSH and sex steroids increase (HPG axis is now on)

hyper tension 1. normal systolic/diastolic pressure 2. normal high blood pressures 3. hypertension 4. severe hypertension 5. malignant hypertension

1. age based blood pressures <90th percentile 2. 90-95th percentile for age 3. >95th percentile for age 4. >99th percentile for age 5. hyper tension with end organ damage

sideroblastic anemia 1. pathogenesis

1. alcohol, lead, isoniazid, chloramphenicol -->accumulation of iron in the mitochondria --> RINGED SIDEROBLASTS

Pompe's disease 1. patho + clinical pres

1. alpha glucosidase deficiency --> muscular weakness, CARDIOMEGALY, flaccid weakness, poor feeding, hepatomegaly

restrictive cardiomyopathy 1. causes 2. clinical presentation 3. treatment

1. amyloid, fabrys, gauchers, hemosiderosis, hemochromatosis 2. exercise inolerance, weakness, dyspnea + edema, hepatomeegaly, elevated CENTRAL VENOUS PRESSURE 3. diretics, beta blockers, CCBs

kyphosis - two types

1. anterior posterior curvature of the thoracic spine two types = flexible (self correctable) and scheurermanns kyphosis (non-flexible)

graves disease 1. pathogenesis 2. labs 3. tx

1. anti-TSH-R IgG --> excess thyroid hormones 2. low TSH, high T3/T4 3. PTU (also inhibits peripheral conversion of T4-T3), methimazole, thyroidectomy, radioactivce iodine

serologic testing 1. ulcerative cholitis 2. crohns disease

1. anti-neutrophil cytoplasmic antibody 2. anti- saccharomyces cerevisiae antibody

1. spina bifida 2. neural tube defect 3. myelomeningocoele + clinical findigs 4. meningocele + clinical findings 5. spina bifida occulta + clinical findings

1. any failure of bone fusion of the posterior midline of the spine 2. any failure of neural tube closure (anecephaly/meningocele) 3. herniation of spinal cord and meninges through bony cleft in lumbosacral region + fluctuant midline mass, neurologic defecits ( higher than L3 = paraplegia, lower than S3 = bowel and bladder only) 4. herniation of meninges only through bony cleft (no neural defecit) + fluctuant midline mass (CSF filled) over the spine 5. just bony cleft, no herniation through cleft + hair patch/dimple over the area of the defect

Idiopathic apnea of prematurity 1. pathogeneissi 2. clinical presentation 3. management

1. apnea that is seen in up to 85% of infants <28 weeks gestation and 25% of infants 33-34 weeks gestation 2. occurs 24 hours after birth, resolves by post gestational age 38-44 3. caffeine, theophylline (respiratory stimulants), proprioceptive stimulation, ventilation, CPAP/mechanical ventillation

relative polycythemia

1. apparant increase in RBC mass caused by decrease in plasma volume *** DEHYDRATION

1. atonic neck reflex 2. appears/dissapears

1. arms and legs extend on same side as head is turned, flex on opposite side 2. 2-4 weeks/6 months

1. parachute 2. appears/dissapears

1. arms/legs outstretch when moved head first downward 2. 8-9 mo/persisits

bowed legs (genu varum) 1. pathogenesis + clinical presentation 2. treatment

1. asymmetric bowing <2 years of age --> cowboy stance (lateral bowing), normal gait 2. observation, if does not resolve by 2 years of age consider rickets, growth plate injury, blounts disease, skeletal dysplasias, measure intercondylar distance

1. when after birth does the onset of cyanosis occur in TOF 2. treatment for tetralogy of fallot

1. at birth as soon as the PDA closes~ now blood cannot flow through the PDA to the lungs to be oxygenated (left to right shunt) 2. surgical repair at 4-8 months, modified blalock-taussig shunt (gore tex graft between subclavian and pulmonary artery)

1. what are the predisposing risk factors for asthma 2. what are the asthma "triggers"

1. atopy, family history of asthma, tobacco smoke exposure, infection, diet, pollution 2. respiratory infections, cold air, emotions, allergens, GI reflux, pollutants

Parinaud oculoglandular syndrome 1. patho

1. atypical bartonella, tularemia, tuberculosis, syphilis --> conjunctiva --> pre-auricular lymphoadenopathy + conjunctivitis

chediak higashi syndrome 1. pathogensis + clinical presentation 2. tx

1. auto recessive --> oculocutaneous albinishm, blond/brown hair with silver streaks azurophilic granules in neutrophils/dec. degranulation, peripheral neuropathy, neutropenia, thrombocytopenia ~~ recurrent infections with s.aureus 2. TMPSMX prophylaxis, daily ascorbic acid

myasthenia gravis 1. pathogenesis 2. dx/tx *60% children remission after thymectomy *symptomatic tx. for adults b/c it is self limited

1. autoimmune antibodies against Ach-R --> hypotonia, weakness, poor feeding, BILATERAL PTOSIS, increasing weakness later in the day, DIPLOPIA, preserved DTRs, 2. tensilon test - IV edrophonium (acetylcholinesterase inhibitor) transiently increases strength decremental response to low frequency repetitive nerve stimualtion AChR antibody titers TX = pyridostigmine (cholinesterase inhibitory), corticosteroids , plasmaphoresis, IVIG, thymectomy

rheumatic fever - children 5-15 year of age 1. pathogenesis 2. clinical dx/presentation

1. autoimmune complication of GABHS URI.pharyngitis (NOT IMPETIGO) 2. Jones criteria, 2 major or 1 major + 2 minor Major: migratory polyarthritis, carditis(transmural, left sided endocarditis, tachycardia) syndhams chorea (basal ganglia autoimmune erythema marginatum (non-pruritic pink/red macules that spread with centrpitally), subcutaneous nodules Minor: fever, arthralgia, leukocytosis, CRP/ESR, prolonged PR on ECG

Complement deficiencies (usually autosomal recessive) 1. C1q/C2/C4 (early classic pathway) clinical assc + tx 2. C5/C6/C8 (late classic pathway) clinical assc + tx 3. C1 esterase inhibitor clinical assc + tx 4. dx of complement deficieny

1. autoimmune disease ex. SLE + treat autoimmun dz/infections 2. meningococal/gonococcal infections + treat infectiosn 3. hereditary angioedema + fibrinolysis inhibitors (danazol), 4. abnormal CH50 indicates that there is an issue SOMEWHERE in the complement path--> specific assay to identify where

central DI causes central DI tx

1. autoimmune, trauma, craniopharyngioma, glioma, germinoma, langerhans cell histiocytosis, sarcoid/TB, vascular, genetic 2. DDAVP

hereditary telangiectasisa

1. autosomal dominant locally dilated totuous veins/capilaries of the skin/mucus membranes

Alagille syndrome

1. autosomal dominant paucity of intrahepatic ducts--> CHOLESTATIC LIVER DISEASE + EXTREME PRURITUS, unusual facial characteristics, pulmonary outflow obstruction/ToF, renal disease, postery embryotoxon, butterfly vertebrae/broad thumbs, pancreatic insufficiency, hypercholesterolemia

congenital myotonic dystrophy 1. pathogensis + clinical presentation (early and late)

1. autosomal dominant trinucleotide repeate expansion chromosome 19 with maternal transmission--> Early: polyhydraminos, poor feeding/respiration, facial diplegia, hypotonia, Late: myotonic facies (atrophic masseter/temporalis), ptosis, stiff straight smile GRIP MYOTONIA, cataracts, cardiac arrythmias, infertility

sickle cell anemia 1. pathogenesis+ clinical presentation 2. dx 3. tx

1. autosomal recessive substitution position 6 beta globin chain glutamate to valine--> stacking of HbA in low oxygen/acidotic condtions --> distorted shape/hemolysis and OCCLUSION OF SMALL VESSELS--> distal ischemia, infarction, organ dysfunction 2. fetal newborn Hgb electrophroesis

1. what causes increased risk of death from infection in patients with sickle cell disease 2. what kind of osteomyelitis in patients with sickle cell disease

1. autosplenectomy leads to risk for infection with encapsulated bacteria, HITB, s.pneumo, n.meningitidis, salmonella 2. salmonella osteomyeliits from the GI tract, and s.aureus osteomyelitis

Cat scratch disease 1. pathogenesis + clinical presentation 2. Parinauds oculoglandular syndrome 3. other complications 2. dxtx

1. bartonella henselae -->initial scratch forms papule along line of scratch--> REGIONAL LYMPHADENOPAHTY (AXILLARY/CERVICAL/INGUINAL)~ nodes are warm tender, suppurative (10%), fever occurs in 1/3 of patients 2. conjunctivitis and PREAURICULAR LYMPHOADENITIS if the infection is in the eye. 3. osteomyelitis, encephalitis, hepatitis, pneumonia, hepatic/spleen lesions 4. IgM antibody to b.henselae azithromycin/TMPSMX/ciprofloxacin for systemic disease

Wilms tumor ~ presents age 2 to 5 years 1. associated conditions 2. clnical presentation

1. beckwith wiedeman/WAGR syndrome, chromosome 11 abnormalities--> most common renal tumor of childhood 2. CHILD WITH abdominal mass***, abdominal pain, hematuria****, hypertension(pressure on renal artery), renin secretion, fever/anorexia, weight loss, hemihypertrophy, aniridia

nevocellular nevi 1. congenital nevi + risk 2. acquired nevi

1. before 6 months of age, black/brown/tan inc. risk of malignancy, giant nevi >20cm have 7% chance of forming melanomas 2. forms between age 2 and 18 years old, usually junctinoal nevi, lower risk of malignancy

nonorganic (functional) abdominal pain 1. epigastric pain 2. periumbilical pain 3. infraumbilical pain

1. belching, bloating, N/V, early satiety, (equiv to non-ulcer dyspepsia) 2. classic pres - varied in character (dull/sharp etc), confers SECONDARY GAIN, does not interfere with sleep/pleasure... 3. abdominal cramping bloating,alterations in stool (equiv to irritable bowel syndrome)

1. anterior mediastinal teratomas clinical presentation + tx 2. ovarian teratomas clinical presentation + tx

1. benign, present with symptoms of airway obstruction 2. benign, finding calcium on the abdominal radiograph near the ovaries

Disciplining child 1. when should child begin showing self control 2. before age 6 months 3. 18-3 years 4. preschool 5. >5 years *make punishment brief, followed with love/trust, direct toward BEHAVIOR not PERSON

1. between ages 3 and 4 (parent should begin providing external control at 6 months) 2. none, distraction/ redirection 3. ignoring, time out, disapproval (both verbal and nonverbal) 4. logical consequences - lose toy being used to hurt another child 5. negotiation and restriction of privileges

poisoning 1. activated charcoal + which toxins is it ineffective for

1. binds toxins, minimizes their absorbtion --- iron, lithium, alcohols, ethylene glycol, iodine, potassium, arsenic, ferrous sulfate, strong bases, cyandide, strong bases **contraindicated in caustic injections because it will interfere with endoscopy

non-hodgkins lymphoma 1. dx/tx - poor prognosis for disseminated disease, good prognosis for localized lymphoma

1. biopsy, liver enzymes, bone scan, CSF analysis tx = surgery + chemotherpay

LANGUAGE MILESTONES 1.attunes to human voices, can differentiate parents voice 2. Cooing 3. Babbling 4. Jargoning, begins using mama, dada (nonspecific) 5. 1-3 words mama and dada (specific) 6. 20-50 words 7. two word phrases / 50% of speech intelligible 8. three word sentences, 75% of speech intelligible

1. birth 2. 2-3mo - (runs of vowels), musical sounds (e.g., ooh-ooh, aah-aah) 3. 6 mo - consonants and vowels (ba ba ba) 4. 9-12 mo babbling with mixed consonants, inflection, cadence 5. 12 months 6. 18 mo 7. 18mo 8. 3 years

iron deficinecy anemia 1. pathogenesis + clinical presentaiton 2. lab findings

1. birth blood loss/cows milk/adolescent menstruation/poor diet--> low iron --> hypochromic microcytic anemia --> pallor, poor weight gain, weakness/fatigue, tachycardia tachypnea, systolic murmur, CHF, dilated cardiomyopathy, SOB, HSM spoon shaped nails, dec. ability to learn 2. low ferritin, low transferrin, high transferrin saturation, high TIBC, high free erythrocyte protoporphyrin, high reticulocytes (surprisingly)

black widow spider 1. pathogenesis + clinical presentation 2. treatment

1. black spider with red or orange hourglass marking--> female bites delivers POTENT NEUROTOXIN --> HYPERTENSION + MUSCEL CRAMPS + headaches,, dizziness, N/V, anxiety, sweating 2. local care, benzodiazepines for muscle cramping, meperideine, lacrodectus anti-venin for sever envenomation

1. Epidural hematoma 2. dx 3. tx

1. bleeding between inner table of the skull and the dura --->due to tearing of the MIDDLE MENINGEAL ARTERY 2. CT shows LENS SHAPED DENSITY*** 3. immediate drainage

Intracerebral hematoma

1. bleeding in the brain parenchyma ~ frontal/temporal lobes OPPOSITE (contracoup) to the side of injury

1. diagnosis of infectious endocarditis 2. treatment 3. what pts need prophylaxis for infectious endocarditis

1. blood culture 3x, ESR (usually elevated) unless there is polycythemia, rheumatoid factor,echocardiography identifies vegetations 2. intravenous antimicrobial therapy 4-6 weeks 3. all structural disease except secundum ASD, post operative cardiac surgery indefinatly if hemodynamic residua remain (otherwise stop at 6 months)

AML 1. diagnosis 2. treatmnet -50% remission from chemotherapy, 70% cure rate with sibling bone marrow transplant

1. blood smear showing leukemic myeloblasts, AUER RODS, bone marrow biopsy 2. intense myeloablative therapy to cause remission, BMT after remission from HLA matched donarCML

EHEC 1. clinical pres 2. dx/tx

1. bloody diarrhea, HUS (0157 H7) 2. WBCs are PRESENT in the stool, culture tx = NO NO NO ABX IF HUS IS PRESENT****THIS ENHANCES ENDOTOXIN RELEASE

shigella sonnei 1. clinical pres 2. dx/tx

1. bloody diarrhea, seizures in children (fron neruotoxin) 2. stool WBCs, stool culture is diagnostic, tx = third generation cephalosporin, fluoroquinolones

buccal cellulitis 1. clnical presentation 2. tx

1. bluish discoloration of the cheek in unimmunized child caused by HIB 2. IV 3rd gen cephalosporins (cefotax/cefuroxime), lumbar punctrue to check for meningitis

Cyanosis 1. definition 2. is it dangerous 3. causes

1. bluish discoloration of the skin/mucous membranes due to >3g/dL reduced Hgb in arterial blood, and >5g/dL Hgb in capillary blood 2. always an emergency 3. •Respiratory (e.g. pneumothorax) •5 T's of congenital heart disease •Central nervous system pathology (e.g. IVH) •Hematologic disorders (e.g. polycythemia) •Metabolic disorders (e.g. hypoglycemia, hypocalcemia, hypothyroidism, and hypothermia)

orbital floor fracture (blow out fracture) 1. pathogenesis + clinical presentation 2. tx

1. blunt trauma --> fractures in the orbital floor--> entrapment of the orbital fat apad and inferior rectus muscle --> strabismus and enophthalmos + numbness of cheek/upper teeth (infraorbital nerve damage) 2. oral antibiotics, surgical repair if diplopia >2-4 weeks

hyphema 1. pathogenesis + clinical presentation 2. tx

1. blunt trauma, diabetic neovascularization, retinal vascular diseases, juvenille xanthogranuloma--> tearing of iris vasculature--> blood in the anterior chamber--> glaucoma, staining of the cornea, optic nerve damage, rebleeding 2. bed rest 5 days

Anorexia nervosa 1. diagnostic criteria 2. physical exam findings 3. lab findings

1. body weight 15% BELOW IDEAL, ABSENCE OF 3 OR MORE CONSECUTIVE MENSTURAL CYCLES •too low caloric intake to maintain weight •delusion of being fat, obsessed with being thin •refusal to maintain body weight, fear of weight gain, excessive exercise, withdrawal from peers/family, disturbed body image 2. hypothermia, hypotension, bradycardia, malnourishment, hypoactive bowel sounds, lanugo, dehydration 3. LOW MG, LOW PHOSPHORUS, LOW CALCIUM, HIGH BUN, high LFTs anemia, leukopenia, low sex steroids, low thyroxine

1. Define attachment 2.when does separation and individuation begin? 3. when does parallel play and social play occur

1. bonding with primary caregiver (begins at birth). Developing empathy is critical during the first 3 years of life. 2. 15 months of age 3. parallel play 2 years; social play 3 years

bone age vs height age vs chronologic age in 1. constituional short stature 2. familial short stature

1. bone age/height age the same and both are less than chronological age (BOTH BONE AGE AND HEIGHT AGE ARE LOW) 2. bone age > height age, bone age = chronological age (ONLY HEIGHT AGE IS LOW)

1. what diagnostic tests must be done for suspected osteogenic sarcoma or ewings sarcoma 2. treatment for these two cancers osteogenic sarcoma more common than ewings sarcoma

1. bone scan and chest CT to evaluate for mets 2. chemotherapy and surgical removal, also can use

1. Small for gestational age definition 2. LGA definition 3. high birth weight

1. born weighing <5th percentile for gestational age as a result of IUGR 2. >90th percentile for gestational age, 3. >4000 gram baby *** LGA does not mean baby has a high birth weight (LGA may mean baby is less than 4000g)

1. precocious puberty in female 2. precocious puberty in male

1. breast development before age 7, or menarche before age 9 2. Testicular changes/penile enlargement or pubic/axillary hair before age 9

1. most common lower respiratory tract infection in the first two years of life 2. risk factors for bronchiolitis

1. bronchiolitis 2. day care, siblings, tabacco smoke exposure, lack of breast feeding, chronic lung disease, prematurity, immunodeficiency

brown recluse spider (fiddleback) 1. pathogenesis + clinical presentation 2. treatment

1. brown violin shaped marking --> bite --> painful itchy papule-->necrotic/deep lesion --> +/- fever, chills, weakness, vomiting, DIC, hemoysis, renal failure 2. no antivenin available, treat ulcer with steroids, skin grafting, dapsone, hyperbaric oxygen

development of the pulmonary system 1. when does bronchial tree develop by 2. when are the air sacs/pulmonary vasculature developed enough to support life 3. when does 90% of alveolar development occur

1. by 16 weeks 2. 26-28 weeks 3. after birth

guillian barre syndorme 1. pathogenesis + clinical presentation

1. c.jejuni**, EBV, HSV, influenza, varicella, coxsackie --> GI illness-->resolution--> CELL MEDIATED IMMUNE RESPONSE TO SCHWANN CELL MEMBRANES -> demyelination of ventral spinal roots + peripheral myelinated neurons --> ASCENDING SYMMETRIC PARALYSIS + RESPIRATORY DISTRESS, lower back pain, leg discomfort, no loss of sensory, cranial nerve involvment

poisoning skin findings from pt 1. cherry red color 2. sweaty 3. dry skin 4. urticaria 5. gray skin

1. carbon monoxide/cyanide 2. organophosphates/sympathomimetics 3. anticholinergics 4. allergic reaction 5. methemoglbinemia

LH 1. effect in males 2. effect in females

1. causes leydig to produce testosterone 2. stimulates theca cells to produce androgens stimulates corpus luteum to make progesterone, ovulatory surge

Polycythemia 1. Definition 2. Epidemiology 3. Etiology

1. central venous hematocrit > 65% 2. Polycythemia occurs in 2-4% of infants born at sea level. 3. Causes include: •increased EPO secretion secondary to placental insufficiency •increased red blood cell production by the fetus in response to hypoxemia •increased placental transfusion from delayed cord clamping

1. sudden change of mental status while treating a patient with DKA 2. electrolyte changes in DKA

1. cerebral edema drop in serum glucose>100mg/dl/hour, excess fluid administration, 2. hypoglycemia, hypocalcemia (osmotic loss/phosphorus usage, hypokalemia(causes arrythmias),

1. sickle aplastic crisis 2. hyperhemolytic crisis

1. cessation of RBC production after parvo B19 --> low HbA, low reticulocytes 2. rapid hemolysis in aptients with G6PD, dec. HbA, high reticulocytes

treament of sickle cell anemia 1. hydroxyurea+ 2. penicillin 3 trans cranial doppler/MRI - daily folate + routine imuizations

1. chemotherapeutic inc. HbF 2. prophylaxis for s.pneumo 3. to monitor for stroke

retinal hemorrhages 1. pathogenesis + clinical presentation

1. child abuse ***, birth trauma, inc. ICP, malignant hypertension, ITP, cardiopulmary resusitation

supracondylar fracture 1. pathogenesis + clinical presentation 2. dx/tx

1. child falls onto outstretched arm/elbow--> point tenderness/swelling, --> possible neurovascular injury/compartments syndrome 2. POSTERIOR FAT PAD SIGN on xray TX: DONT PASSIVLY MOVE THE ARM, casting for nondisplaced/nonangulated, surgery for displaced/angulated

School phobia - child fears leaving home/caregiver 1. clinical presentation 2. tx

1. child who misses school b/c of abdominal pain/diarrhea/fatigue that occur in the morning on departure for school, and disappear on weekends/summer 2. hx/px, return child to school, if still wants to stay home visit physician

1. which are risk factors for a poor prognosis for near drowning 2. why in general do children have better near drowning results than adults

1. children < 3 years of age, submersion time >5 minutes, ressusitation delay>10 minutes, CPR required, abnormal neurologic examination ro seizures, pH < 7 2. primitive dive reflex shunts blood to vital organs

poisonings 1. what age do most poisonings occur 2.when/why do most poisonings occur

1. children <6 years of age 2. accidental when the caregiver is distracted (usually ingestions, somtimes inhaled, cutaneous, skin/eyes, intravenous

ketotic hypoglycemia 1. clinical pres

1. children age 1-6 yrs, hypoglycemiain the morning, + ketonnuria, low insulin, due to inability to adapt to fasting state

Osgood schlatter disease 1. pathogenesis + clinical presentation 2. dx/tx

1. children who participate in sports involving jumping --> apophysitis (inflamm of a tuberosity) + 2. tenderness with extension of the knee against resistance tx = rest/stretching of the quadriceps/hamstrings

reactive reiters disease 1. clinical presentation

1. chlamydia trachomatis--> arthritis, urethritis, conjunctivitis (cant see cant pee cant climb a tree - TF)

retinoblastoma 1. pathogenesis + clinical presentation 2. tx 3. most common late complication

1. chromosome 13 mutation--> leukocoria/strabismus, retinal detachment, hyphema 2. dx = calcification within tumor seen on CT tx = enucleation, or radiation/laser/cryo 3. cancer later in life especially OSTEOGENIC SARCOMA

Medium chain acyl CoA dehydrogenase 1. pathogenesis + clinical pres 2. dx/tx

1. dec. ability to breakdown fatty acid chains---> non ketotic hypoglycemia, hyperammonemia, myopathy, cardiomyopathy 2. tandem mass spec showing inc. medium chain fatty acids, ~ tx = high carboydrate low fat diet, carnitine supplementation during acute episodes

1. what conditions is hepatocellular carcinoma associated with 2. clinical presentation 3. diagnosis 4. treatment -poor prognosis,mostly non-resectable and mets to lungs, brain, lymph nodes

1. chronic hepatits B, biliary atresia, glycogen storage diseases, alpha 1 antitrypsin deficiency, hereditary tyrosinemia 2. right upper abdominal mass, loss of appetite, and weight loss 3. elevated ALPHA FETO PROTEIN, and CT/MRI of the liver 4. surgical resection, and chemotherapy (to make tumor operable)

lyme disease 1. diagnosis 2. treatmnet

1. clinical pres + ELISA (sensitive), then western blot (sens + specific), 2. early doxycycline or amoxicillin ceftriaxone or penicillin for carditis/meningitis

Hydrocele 1. pathogenesis 2. clinical presentation 3. tx

1. collection of fluid in the tunica vaginalis 2. transillumination of scrotum reveals soft painless cystic mass 3. reassurance, or surgery if very large/painful

Categories of Apnea 1. central apnea 2. apnea secondary to airway obstruction 3. mixed apnea

1. complete cessation of chest wall movements without airflow 2. cx wall movements or respiratory effort without airflow 3. central + obstructive = most common type in infants

neuroblastoma staging

1. confined to structure of origin 2. extends beyond structure of origin but doesnt cross the midline 3. tumor extends past the midline 4. metastasis to bone/lymph nodes/ bone marrow/soft tissue 4s. localized tumor at stage 1 or two with distant mets to any organ but bone

short bowel syndrome 1. pathogenesis 2. clinical presentation 3.treatment

1. congenital - gastroschisis, volvulus, intestinal atresia necrotizing enterocolitis surgery, rohns disease, tumors, radiation --> carbohydrate/fat malabsortbion, dehydration, hyponatremia, hypokalemia, B12/bile acid malabsorbtion 2. diarrhea, malabsorbtion, failure to thirve 3. parenteral nutrition, but encourage enteral feedings so that intestine/biliary function develops, small bowel transplantation

1. carbohydrate malabsorbtion pathogenesis 2. lab diagnosis

1. congenital ENZYME DEFECT/MUCOSAL ATROPHY --> undigested sugars are osmotic force that cause water to enter the intestine increasing stool volume/peristalsis and decreasing transit time ==> colonic bacteria proliferation forming H2/CO2/acids --> diarrhea 2. reducing substance (sugars) by positive CLINITEST reaction, stool pH<5.6

chronic abdominal pain -organic= caused by disorder -inorganic= functional (more common in females) 1. causes of organic CAP

1. constipation, PUD, carbohydrate intolerance, IBD, pancreatitis, parasites, pyelonephritis, hydronephrosis, malrotation, hernia

PDA 1. physical exam findings 2. ECG fidnings 3. CXR

1. continuous machinelike murmor at the uppler left sternal border with STRONG PERIPHERAL PULSES, WIDENED PULSE PRESSURE 2. LVH, RVH if pulmonary hypertension 3. cardiomegaly with increased PVM

kawasaki disease 1. cardiovascular manifestiation 2. systemic manifestatiosn -also: arthitis, hydrops of gallbladder, anterior uveitis

1. coronary artery aneurysms in 20% of untreated pts, myocarditis, arrythmias, brachial artery aneurysms 2. aseptic meningitis, urethritis (sterile pyuria)

protein intolerance 1. pathogenesis + clinical presentation 2. enteropathy 3. enterocolitis 4. dx/tx

1. cows milk protein (MCC), soy, egg protein--> diarrhea/vomiting, colicky pain, ENTEROPATHY, ENTEROCOLITIS 2. progressive diarrhea, vomiting, irritability, abdominal pain --> anemia, protein loss, FTT 3. acute diarrhea, rectal bleeding, mucus, distension, irritability, hypoproteinemia, FTT 4. withdrawing the food, avoid the food

myocardidits (inflammation of the myocardium itself) ** major cause of sudden death in athletes 1. etiologies 2. clnical presentation 3. dx 4. tx

1. coxsachie, c.diphtheria, s.pyogenes, s.aureus, m.tuberculosis, candida, cryptococcus, t.cruzi (chagas*), SLE, rheumatic fever, sarcoidosis, kawasaki disease 2. viral or flu like illness --> dyspnea and malaise ~~ resting tachycardia, muffled heart sounds, gallop heart rhythum, tachypnea, hepatomegaly, pulmonary rales 3. ESR/CRP, CK-MB, ECG (T/ST wave changes), echocardiogram shows global ventricular dysfunction, endomyocardial biopsy 4. ionotropes, diuretics, IVIG, cardiac transplant~~ mortality 10-20%

hand foot mouth disease 1. pathogenesis + clinical presentation 2. tx

1. coxsackievirus A16--> vesicles, papules, pustules on the palms/soles/fingertips + mouth (herpangina) + non specific fever, headache, backache and vomiting 2. supportive

Subglottic disorders 1. what are they 2. describe the assc: stridor/cough/voice/dysphagia+drooling/fever/toxicity/posture

1. croup and bacterial tracheitis 2. loud/barking/hoarse voice/no dysphagia/drooling/low fever in croup, high fever in tracheitis/toxicity only with tracheitis/normal posture

Cervical cap 1. mechanism 2. advantages 3. disadvadvantages

1. cuplike diaphragm placed over cervix 2. can be left in place for 48 hours 3. need for individual fitting, inc. risk for UTIs, CERVICAL DYSPLASIA (must follow up with Pap smear)

poisoning scents from pt 1. bitter almond 2. garlic 3. acetone 4. wintergreen 5. moth balls

1. cyanide 2. arsenic/organophosphates 3. salicylates, isopropyl alcohol 4. methyl salicylate 5. camphor

corneal abrasion 1. pathogenesis + clinical presentation 2. dx/tx

1. damage/loss of the corneal epithelium--> severe pain/foreign body sensation 2.dx = flurescein staining of the cornea tx = protective shield and antibiotics

1. lipid malabsorbtion pathogenesis 2. lipid malabsorbtion diagnosis

1. dec lipase/exocrine pancreatic insufficiency/intestinal mucosal atrophy/bile acid deficiency /abetalipoproteinemia --> steatorrhea and dec. absorption of ADEK 2. fecal fat, pH, reducing substances, alpha 1 antitrypsin

21 hydroxylase def 1. pathogenesis 2. simple virilizing vs non-classic CAH3 3. lab dx of CAH 4. tx

1. dec. 21 OH leads to inc. androgens Girls have ambig genetalia, and electrolyte abnormalities 2. simple virilizing = no effect on aldosterone, non-classic = late onset with no mineralocorticoid involvement 3. incrased 17 hydroxyprogesterone 4. cortisone suppresses ACTH production, flurocortisol suppresses mineralocorticoid production

constipation encopresis *encopresis usually in males

1. dec. defecation, that is difficuly, and causes abdominal discomfort with dry hard stools 2.developmentally inappropriate release of stool: liquid stool leaks around hard retained stool mass and is released through a distended anorectal canal

Bacterial vaginosis (non-STD) 1. pathogenesis 2. clinical findings of bacterial vaginosis 3. dx 4. tx

1. decreased lactobacilli ⇒ inc. GARDNERELLA VAGINALIS, MYCOPLASMA HOMINIS, anaerobic gram negative rods 2. gray-white vaginal discharge, FISHY odor, LESS**vaginal/vulvar inflammation 3. fish smelling whiff test w/ 10% KOH, CLUE CELLS, thin gray vaginal discharge, vaginal pH <4.5%. 4. metronidazole or 2% topical clinda or 0.75% topical metranidazole *NO TREATMENT FOR PARTNERS****

PPH 1 evaluation 2. management

1. decreased pulmonary vascular markings on CXR; echocardiogram to rule out congenital heart dz 2. oxygen: most POTENT PULMONARY VASODILATOR, mechanical ventilation, high frequency ventilation, extracorporeal membrane oxygenation (ECMO), inhaled NITRIC OXIDE

clinical presentation of 1. optic glioma 2. craniopharyngioma

1. decreased vision, decreased visual field, strabismus 2. growth retardation, delayed puberty (disruption of the pituitary), visual changes, central diabetes inspidus, precocious puberty, papilledema/hydrocephalous (50%), vomitting

necrotizing fasciitis 1. pathogenesis 2. clinical presentation 3. tx

1. deep cellulitis that is potentially fatal-- 2. crepitus and hemorrhagic bullae 3. IV antibiotics and surgicla debridement

sinus venosus 1. features

1. defect high in the septum near right atrium/SVC junction, right pulmonary veins drain into the atrium/SVC instead of the left atrium

Galactosemia 1. patho 2. clinical pres 3. dx/tx 4. complications

1. defect in galactose 1 phosphate uridyl transerase --> 2. HEPATOMEGALY, HYPOGLYCEMIA vomitting, diarrhea, FTT, oil droplet cataracts, RTA 3. non-glucose reducing substance in urine by clnistix ~ galactose restricted diet 4. e. coli sepsis***, female ovarian failure

SCID 1. pathogenesis (XLR/AR) + clinical presentation 2. dx/tx

1. defective T cell and B cell function X linked - deficiency gamma chain for IL-2 receptor AR - ADA deficiency + others ---> inc. infection with oppurtunistic pathogens (c.albicans, p.carinii) 2. <1500 lymphocytes/mL, dec. # of T cells, severe hypogammaglobulinemia, depressed response to mitogens/antigens TX = abx, blood products irradiated, IVIG replacement, P.carinii, bone marrow transplant**** (curative)

diabetes inspidus 1. pathophysiology 2. central vs nephrogenic 3. labs 4. dx

1. defective/deficienct effect of ADH leads to inability to concentrate urine 2. central = no ADH, nephrogenic =ADH resistance 3. NORMAL ELECTROLYTES IF THIRST IS INTACT. urine inappropriatly dilute, urine SG <1.018 4. water deprivation test- rising serum osmolality with persistantly low urine osmolality, and good response to DDAVP = central DI if no response = nephrogenic DI

von willibrands disease 1. pathogenesis (types 1/2/3) 2. clinical presentation 3. treatment

1. defects in vWF portion of factor VIII complex --> Type1 = quantitative deficiency of vWF/VIII Type 2 = qualitative abnormalitiy in vWF Type 3 = absesne of vWF 2. mucocutaneous bleeding, epistxis, menorrhagia, dental bleeding, NO HEMARTHROSES 3. DDAVP (Type 1/2), cryoprecipitate (used during surgery, bleeding, or type III disease)

1. what determines the severity of TOF 2. what actions increase right to left shunting in TOF 3. what actions increase flow through the RVOT in TOF

1. degree of RVOT obstruction (pulmonary stenosis) 2. dec SVR = exercise, vasodilation, volume depletion inc. RVOT resistance = crying, tachycaria 3. inc. SVR = volume infusion, systemic hypertension, valsalva, bradycardia

prerenal failure 1. pathogenesis 2. lab findings

1. dehydration, hemorrhage, CHF, septic shock, hypoproteinemia --> decreased renal perfusion --> REVERSIBLE decrease in renal perfusion and dec. GFR 2. BUN/CR >20, urine SG >1.030, urine osmolarity>500, urine Na <20, FENa<1% in older children, <2.5 in neonates

Williams syndrome 1. patho 2. clinical presentaiton 3. dx

1. del chromosome 7, elastin 2. COCKTAIL PARTY PERSONALITY, elfin facies, mental retardation and loquacious personality, supravalvular aortic stenosis***, idiopathic hypercalcemia, connective tissue abnormalities e.g. hoarse voice and hernias 3. FISH probe

DiGeorge syndrome 1. pathogenesis 2. clinical presentation

1. deletion at 22q11 2. CATCH22 - cardiac (VSD/TOF), abnormal facies (small chin, short palpebral fissures, ear anomalies), thymic hypoplasia and parathyroid hypoplasia (cell mediated immunity deficiency) , cleft palate, hypocalcemia (→seizures)

Velocardiofacial syndrome 1. pathogenesis 2. clinical presentation

1. deletion at 22q11 2. cleft palate, wide prominent nose with squared nasal root, short chin, fish-shaped mouth, VSD, right sided aortic-arch, neonatal hypotonia, learning disabilities, perseverative behavior

beta thalassemia -Mediterranean demographics 1. pathogenesis + clinical presentation (based on # of deletions) 2. complications

1. deletion of beta globin genes --> -one deletion - mild asymptomatic anemia, hypochromic microcytic anemia, target cells, anisocytosis -two deletions - HSM, extramedullary hematopoiesis, thalassemia (chipmunk) facies, target cells, poikilocytes, indirect hyperbilirubinemia, high serum iron, high LDH, low HbA, high HbF 2. hemochromatosis (heart, liver, lungs, pancreas, skin) from transfusions

alpha thalassemia -asian demographics 1. pathogenesis + clinical presentation (based on # of deletions)

1. deletion(s) in alpha globin chain (total 4 genes) --> - one deletion - silent carrier, no anemia -two deletions- alpha thalassemia minor - mild anemia - three deletions- HbH disease- severe anemia at birth, elevate Hgb Barts (tetramers of beta chains, severe lifelong anemia -four deletions - only Hgb Barts, fetal hydrops - profound anemia, CHF/death

poisoning 1. acetaminophen mechanism of damage 2. 30minutes - 24 hours 3. 24-72 hours 4. 72-96 hours 5. 4 days - 2 weeks

1. depletes glutothione--> accumulation of toxic intermiates causes death of hepatocytes and hepatocellular necrosis 2. asymtomatic + N/V + diarrhea 3. hepatic transaminases rise 4. hepatic necrosis (-->transplant), jaundice, hypoglycemia, lactiic acidosis, hepatic encephalopathy, coagulopathy, renal fialure 5. resolution, progressive liver damage, death

Contraceptive injections 1. mechanism

1. depomedroxyprogesterone acetate (Depo-Provera): slow release progestin given 1x every 3 months by intramuscular injection 2. 3 months protection 3. need for injection, irregular bleeding, weight gain, no STD protection

treatment for poisonings 1. if patient has altered mental status **CALL POISON CONTROL FOR HELP*** 2. syrup of ipecac 3. gastric lavage

1. dextrose + naloxone 2. syrup of ipecac induces emesis, only useful in first 30 minutes--- does not improve clinical outcome...... 3. large bore orogastric tube suction within one hour after ingestion --- variable results as to clinical outcome

Yersinia enterocolitica 1. clinical pres 2. dx/tx

1. diarrhea + mesenteric adenitis (mimics appendicitis) 2. stool culture or mesenteric node grwos organism tx = cephalosporins

Kwashiorkor 1. pathogenesis + clinical pres

1. diet that consists of only starches --> PROTEIN DEFICIENCY --> GENERALIZED EDEMA , abdominal distension, skin pigmentation changes, thin sparse hair

cholecystitis 1. clinical presentation 2. dx.tx

1. diffuse right upper quadrant pain**, fever, anorexia, jaundice, MURPHYS SIGN*** (RUQ palpation causes pain/guarding/dec. respiratory effort) 2. abdominal ultrasound shows stones + thickened gallbladeder wall tx= fluid resuscitation, parenteral antibiotics, cholecystectomy if peritonitis is occuring

toxins/antidotes 1. digitalis glycosides 2. heavy metals (mercury, maganese, copper, gold, nickel, zinc, lead, arsenic) 3. inducers of dystonia 4. inducers of methemoglobinemia 5. iron 6. isoniazid

1. digoxin Fab fragments 2. D-penicillapmine, dimercaprol, DMSA, EDTA 3. diphenhydramine, benztropine 4. methylene blue 5. deferoxamine 6. B6

Varicocele 1. pathogenesis 2. clnical presentation 3. tx

1. dilation and tortuosity of veins in the pampiniform plexus. 2. most commonly found in the left half of the scrotum: characterized as a "a bag of worms" that increases with standing/valsalva 3. reassurance; if varicocele is painful/distended or associated with small testicle refer to urology

toxoplasmosis 1. pathogenesis + clinical presentation

1. direct contact with cat feces, eating undercooked meat, fruts/vegetables, transplacental, blood products/transplant --> Immunocompetent : asymptomatic, or MONONUCLEOSIS LIKE ILLNESS Immunocompromised/PREGNANT: encephalitis, focal brain lesions/seizures, disseminated 2. PCR, serology, finding organisms in CSF, blood tx = sulfadiazine and pyrimethamine

1. generalized seizure 2. tonic clonic seizure 3. absence seizures

1. discharge from both hemispheres - tonic, tonic clonic, clonic, myoclonic, absence, atonic 2. increased thoracic/abdominal tone --> clonic movement of arms/legs, incontinence, decreaesed consciousness, with POSTICTAL STATE* 3. staring spells that occur without loss of posture, only eye movements, lasts less than 15 seconds

otitis externa 1. pathogensis 2. pathogens 3. cliniccal presentation

1. disruption of protective mechanisms: cerumen, swimming, excessive moisture 2. pseudomonas, staph, candida 3. pain/itching/drainage 4. dx - erythema of external auditory canal tenderness while moving tragus, tx- acetic acid wash, topical antibiotics/topical corticosteroids ~ if perforated use both oral and topical abx

1. distributive shock 2. anaphylactic shock 3. neurogenic shock

1. distal pooling of blood/fluid extravasation--> caused by anaphylaxis, neurogenic, meds, toxins 2. acute angioedema of upper airway, bronchospasm, pulmonary edema, urticaria, hypotension (from extravasation) 3. 2' to spinal cord transection--> loss of sympathetic cardiovascular tone --> hypotension from pooling of blood in the vascular bed

atlantoaxial instability 1. pathogenesis 2. dx

1. down syndrome**, keletal dysplasia, klippel feil syndrome --> unstable 1st/2nd cervical vertebrae joint --> spinal cord injury --> paralysis 2. x ray, C1/C2 cervical spine fusion

pathogenesis of cerebral edema after treating DKA

1. drop in serum glucose >100mg/dL/hour, fluid administration >4 L/m2/24 hours--> osmotic shift from hypotonic intravascular space to the brain --> BRAIN EDEMA

Toxic epidermal necrolysis

1. drug reaction--> widespread epidermal necrosis--> sloughing of the skin, NIKOLSKY SIGN~ HIGH DEATH due to sepsis, dehydration, electrolyte

Porphyria 1. patho + clinical pres 2. dx/tx

1. drugs/hormone surges/poor nutrition --> skin photosensitivity, personality changes, emotional labiltiy, colicky abdominal pain.vomiting, autonomic instablity****, dark colored urine 2. increased serum prophobilinogen IV glucose, correction of electrolytes, avoidance of fasting and precipitating drugs

anaphylaxis 1. pathognesis + clinical presentation 2. treatment

1. drugs/insect venom/foods/latex/biologic agents --> antigen binds to IgE on the surface of mast cells/basophils -->release of vasoactive substances--> RAPID pruritus, urtiaria, angioedema, dyspnea/wheezing, N/V, hypotension/shock 2. EPINEPHRINE (resp/cardio), antihistamines, corticosteroids, B-agonists

1. Isosexual precocious puberty or central precocious puberty (CPP) 2. cause in girls vs boys 3. diagnosis 4. what to do in boys with a positive CPP

1. early hypothalamus activation --> early onset of gonadotropin mediated puberty~~~ looks like normal puberty just early 2. idiopathic in girls, pathologic in boys, need MRI of the head (hydrocephalus, CNS infections, cerebral palsy, hamartomas, astrocytomas/glyomas, head trauma) 3. GnRH stimulation test~ in someone with CPP the LH/FSH will dramatically rise where as in someone who is pre-pubertal there will be very little rise in LH/FSH 4. MRI of the head because this is almost always pathological in boys

What are the health effects of obesity

1. early puberty 2. hypertension/heart disease 3. hypercholesterolemia/ inc. triglycerides 4. type 2 diabetes 5. gallbladder disease 6. back pain/tibia vara 7 depression/poor body image

crohns disease 1. pathogenesis 2. clinical presentation

1. eccentric segmental transmural skin lesions, FISTULAS, sinus tracts, crypt abscess, most commonly effects TERMINAL ILEUM, but can effect anywhere from mouth to anus 2. abdominal pain, post prandial cramping, diarrhea, anorexia iron/B12/zinc/folate deficiency, PERIANAL DISEASE- skin tags, fissures, fistulas, abscesses

food allergy 1. common triggers + clinical presentation

1. egg, milk, peanut, soy, wheat, fish--> itching/swelling of the lips, tongue, throat, GI sypmtoms like N/V, diarrhea, rhinorrhea, wheezing, sneezing, atopic dermatitis, urticaria/angioedema, anaphylaxis

pulmonary stenosis 1. physical exam findings 2. ECG fidnings 3. CXR

1. ejection click, systolic ejection murmur at ULSB 2. RVH 3. prominant main pulmonary artery (post stenotic dilation)

aortic stenosis 1. physical exam findings 2. ECG fidnings 3. CXR

1. ejection click, systolic ejeection murmor that radiates to URSB/apex/suprasternal notch/carotids, thrill at the URSB and suprasternal notch 2. noraml or LVH 3. nromal or mild cardiomegaly, prominent ascending aorta from post stenotic dilation

Iron deficiency anemia 1. treatment

1. elemental iron taken with orange juice (vitamin C enhances intestinal absorbtion)

coarctation of the aorta 1. physical exam findings 2. ECG fidnings 3. CXR

1. elevated BP in right arm, reduced BP in legs, delayed femoral pulses (brachiofemoral delay), left upper back bruit***~ also associated with bicuspid valve aortic stenosis*** 2. normal or LVH 3. RIB NOTCHING (dilation of collateral chest wall vasculature), normal heart size

septic arthritis 1. dx/tx -complications = avascular necrosis/cartilage damage

1. elevated WBC/ESR/CRP, postive blood culture(50%), SYNOVIAL FUID SHOWS WBC >50K-100K, radiograph shows widened joint space Tx = EMERGENCY surgical decompression/drainage, by joint aspiration, empiric IV abx

two phases of parenteral rehydration

1. emergency phase - 20ml/kg BOLUS of normal saline or lactated ringers 2. repletion phase - acute hyponatremic or isonatremic dehydration correct over 24 hours hypernatremic dehydration correct over 48 hours ****correct chronic dehydration much more slowly

1. EBV pharyngitis clinical presentation + tx 2. coxsackie pharyngitis clinical presentation

1. enlarged POSTERIOR CERVICAL NODES, malaise, hepatosplenomegaly ~~ Tx = corticosteroids 2. painful ulcers on posterior pharynx and mouth (herpangina), and blisters on palms and soles

DMD/BMD 1. dx/tx - DMD death from resp. failure in late teens -BMD - wheelchair by ~ 20 years of age

1. enlarged calf muscles with weakness, high CK, EMG shows polyphasic muscle potentials with normal condution muscle biopsy with dystrophic pattern absent or decreased dystrophin tx = corticosteroids early in disease (mild improvement)

1. protein malabsorbtion pathogenesis 2. lab diagnosis

1. enterokkinase def/protein losing enteropathies/crohns/colitis --> dietary loss of proteins 2. fecal alpha 1 antitrypsin levels used to document enteric protein loss

1. viral causes of meningitis 2. bacterial aseptic meningitis causes 3. fungal "" 4. parasitic """

1. enteroviruses, LCM, HSV, EBV, CMV, VZV, arbo, influenza 2. tuberculosis, borrelia burgdorferia, treponema pallidum 3. cocci, crypto, histo 4. t. solium, toxo gondii

langerhans cell histiocytosis 1. diseases 2. clinical presentation -also non-specific: fever, faitgue, FTT, 3. treatment

1. eosinophilic granuloma (focal), hand schuller christian, letterer siwe (disseminated) 2. skull most commonly involved, single or multiple bony lesions, chronic draining ears , skin involvement of the diaper area and scalp, pituitary/hypothalamic (growth retardation/DI/hypogonadism/panhypopituitarism) 3. coritcosteroids, local curettage, low dose radiation

1. first degree burn 2. second degree burn (two subtypes) 3. third degree burn

1. epidermis only, red blanching, painful skin, HEALS WITHOUT SCARRING (ex. sunburn) 2. entire epidermis and part of the dermis A. superficial partial thickness = outer dermis-- blister but no scar deep partial thickness -= entire epidermis and lower dermis pale white may blister, heal with scarring 3. full thickness burn: epidermis, dermis, subcutaneous tissue, DRY WHITE LEATHERY, skin grafts are needed, INSENSITIVE TO PAIN

Supraglottic disorders extrathoracic 1. what are they 2. describe the assc: stridor/cough/voice/dysphagia+drooling/fever/toxicity/posture

1. epiglottitis, retropharyngeal abscess 2. quiet/none/muffled/present/high fever/toxic apperarance/neck extended+tripod position

perianal cellulitis 1. clincal presentation. 2. treatment

1. erythema of the anus caused by group A strep 2. cefalexin (first get cepho)

jaundice 1. in the first 24 hours 2. """ 2nd or 3rd day 3. 3rd day to 7th day

1. erythroblastosis fetalis, concealed hemorrhage, sepsis or congenitla infections 2. usually phsyiologic 3. bacterial sepsis or UTI (often lacks classic poor feeding, bulging fontanel) --> LP/blood cultures

Oral contraceptives 1. mechanism 2. advantages 3. disadvantages 4. contraindications**

1. estrogen/ progesterone combination or progesterone- inhibits ovulation (E/P), thickens cervical mucus(P) 2. dec. dysmenorrhea, protects against endometrial/ovarian cancer, improved acne, spontaneity 3. headache, weight gain, amenorrhea, breakthrough bleeding, mood changes, no STD protection 4.Absolute: pregnancy, breast/endometrial cancer, STROKE, CAD, LIVER DISEASE Relative: smoking, HTN, migraines, diabetes, sickle cell anemia, elevated triglycerides

toxins/antidotes 1. methanol/ethylene glycole 2. narcotics 3. organophosphate/carbamate pesticides 4. pit viper snake bite 5. beta blockers/calcium channel blockers 6., sulfonylureas/oral hypoglycemics

1. ethanol, fomepizole 2. naloxone 3. atropine, pralidoxime 4. antivnin, crotalidae polyvalent Fab fragments 5. glucagon 6. octreotide, glucagon

sinusitis pathogenesis 1. development of the sinuses

1. ethmoid/maxillary - 3rd/4th month of gestation, sphenoid between 3 and 5 years of age, and frontal sinuses between 7 and 10 years of age

acne vulgaris 1. pathogenesis + clinical presentation 2. tx

1. excessive coheision of cells in sebaceous follicles + sebum --> comedones --> proliferaiton of propionibacterium acnes occurs 1-2 years before puberty 2. benzoyl peroxide

Mycobacterium tuberculosis 1. exposure vs latent vs disease. 2. epidemiology

1. exposure = someone who has contact with pulmonary TB latent tuberculosis infection = asymptomatic with positive PPD, and +/- chest radiograph disease = signs and symptoms with +/- positive chest radiograph 2. immigrants, homeless, institutions, correctional facilities, immunodeficiency conditions

1. inspiratory stridor 2. expiratory wheezing 3. crackles or rales

1. extrathoracic obstruuction - ex. croup, laryngomalacia 2. intrathroactic obstruction ex. asthma/bronchiolitis 3. parenchymal disease ex. pneumonia/pulmonary edema

nasolacrimal duct obstructino 1. pathogenesis + clinical presentation 2. tx

1. failure of canalization of the lacrimal system and obstruction at Hasners valve --> watery eyes, matted eyelashes, mucus 2. spontaneously resolves, nasolacrimal massage, toical abx, NLD probing

Duodenal atresia 1. pathogenesis -associated with down syndrome, more common in males 2. clinical presentation 3. diagnosis 4. treatment

1. failure of the duodenum to recanalize at 8-10 weeks gestation, 2. polyhydraminos, scaphoid abdomen with epigastric distension, feeding intolerance, vomitting, NO BILIOUS EMESIS 3. abdominal radiograph shows DOUBLE BUBBLE 4. hydration/electrolytes, duodenoduodenostomy

1. what studies for suspected malabsorbtion 2. CBC findings in B12 or folate malabsorbtion 3. CBC findings in abetalipoproteinemia 4. CBC findings in schwachann diamond syndrome 5. low serum albumin

1. fecal fat, carbohydrates (CLINITEST reducing sugar), alpha 1 antitrypsin, CBC 2. macrocytic anemia 3. acanthocytosis 4. neutropenia 5. protein losing enteropathies

hepatits A (picornavirus) 1. pathogenesis 2. diagnosis

1. fecal oral -->2-6 week incubation--> asymptomatic jaundice 2. IgM anti-HAV then lifelon IgG anti-HAV

ascaris lumbricoides 1. pathogenesis + clinical presentation 2. tx

1. fecal oral egg ingestion --> loefflers syndrome = transietn pneumonitis, fever, cough, wheezing, eosinophilia, small bowel obstruction 2. tx = mebendazole, albendazole or pyrantel pamoate (for the whole family)

rotavirus 1. pathogenesis + clinical presentation 2. dx/tx

1. fecal oral in winter--> 1-3 day incubation --> vomiting, diarrhea, dehydration (self limited) 2. stool ELISA, tx= hydration

giardia lamblia 1. pathogenesis + clinical presentation 2. dx/tx

1. fecal oral ingestion of cysts from contaminated water in U.S./daycare centers--> 1-2 wk incubation-->small bowel --> voluminous watery, FOUL SMELLING stool, bloating, flatulence, weight loss 2. stool examination for cysts/trophozoites tx = etronidazole, furazolidone

norwalk viurs 1. pathogenesis + clinical presentation

1. fecal oral outbreaks in closed populations --> vomitting (self limited in 48-72 hours) ~ often associated with daycare, cruise ships etc

salmonella 1. clinical pres 2. dx/tx

1. fecal oral poultry/milk/eggs/lizards/turtles ---> bloody/non-bloody diarrhea + rose spots on the abdomen 2. +/- stool WBCs, culture is diagnostic tx = NOT INDICATED FOR GASTROENTERITIS IN IMMUNOCOMPETENT HOSTS BECAUSE IT INCERESES CHRONIC CARRIER RATE 3rd gen cephalosporins

enterobius vermicularis 1. pathogenesis + clinical presentation 2. tx

1. fecal oral transmission of eggs --> anal/vulvar pruruitus/insomnia, anorexia, enuresis, teeth grinding 2. tx = mebendazole, albendazole or pyrantel pamoate (for the whole family)

Appendicitis - most often age 8-12 years of age 1. pathogenesis 2. clinical presentation 3. dx

1. fecolith or lymphoid tissue cuases appendiceal distension/ischemia 2. T10 periumbilical pain + vomitting --> right lower quadrant pain @ MCBURNEYS POINT 3. leukocytosis with PMN prodominance, abdominal ultrasound/CT- TX appendectomy, antibiotics, irrigation of the peritoneal cavity if perforation occurs

α-Fetoprotein (AFP) 1. high levels 2. low levels

1. fetal neural tube defects, multiple gestation pregnancies, underestimated gestational age, ventral abdominal wall defects, fetal demise, or fetal conditions that cause edema or skin defects. 2. overestimated gestational age, trisomies 21 and 18, and intrauterine growth retardation

FUO - fever of unknown origin 1. definition 2. cause 3. lab studies 4. radiologic studies 1/4 resolve spontaneously

1. fever that lasts longer than 8 days to 3 weeks, or when prior history, physical and lab evals have failed to lead to a diagnosis 2. common illness with atypical presentation~ big table 7-2 3. CBC/blood culture, ESR or CRP, ASO, serum transaminases, urinalysis/urine culture, ANA, stool culture, tuberculosis test, HIV test 4. CXR, echocardiography, bone scan, CT and MRI (depending on the clinical clinical pres)

PCP pneumonia in an HIV+ infant (most common oppurtunistic infection in HIV infected children) 1. clinical pres 2. tx

1. fever, HYPOXIA, interstitial infiltrates 2. TMPSMX prophylaxis + TMPSMX, pentamidine, or atovaquone (ubiquinone analog)

SLE 1. clinical presentation

1. fever, weight loss, malaise malar rash, photo sensitivity, alopecia, raynauds arthrialgias WITHOUT joint deformity/erosions pericarditis, libman sacks endocarditis, congenital heart block* pleuritis, interstitial fibrosis, pulmonary hemorrhage glomerulonephritisi, nephrotic syndrome, hypretension leukocytopneia, AOCD, thrombocytopenia, coombs positive hemolytic anemia

scleroderma 1. pathogenesis + classic clinical presentation

1. fibrosis and dysfunctions of multiple organ systems - skin, heart, kidneys, lungs, GI Thickening of the skin with loss of dermal ridges

neuroblastoma 1. clinical presentation 2. diagnosis

1. firm abdominal mass(MCC), respiratory distress/anorexia, tracheal compression, horners syndrome, flushing, hypertension, headache, sweating, fever, weight loss acute cerebellar atropy - ataxia/opsoclonus/myoclonus metastasis - periorbital eccymosis/proptosis, blueberry muffin skin nodules, bone pain or limping, hepatomegaly 2. excessive urinary catecholamines - VMA, HVA, positive bone marrow/tissue biopsy CT/MRI to find mets skeletal survey or technetium 99m bone scan to find mets to bone

1. when is visual development most important 2. what can cause abnormal visual development

1. first 3-4 months of life 2. improper eye alignment (strabismus) any condition that blocks retinal stimulation

1. macule 2. patch 3. papule 4. plaque

1. flat non-palpable, cutaneous color change 2. large macule 3. elevated superficial dermal lesion 4. large/coalesced papules

1. vesicle 2. bulla 3. pustule 4. cyst 5. wheals

1. fluid filled papule 2. large vesicle 3. purulent filled papules 4. nodules with expressable material 5. cutaneous elevation with dermal edema

fracture 1. nondisplaced(non angulated) fracture 2. displaced 3. angulated 4. overriding

1. fracture ends that are near each other 2. fractured ends have shifted 3. fractured ends for m an angle 4. fractured ends override without contact

FSH 1. effect in males 2. effect in females

1. inc. spermatogenesis in the seminiferous tubules 2. stimulates ovarian follicles, stimulates ovarian granlosa cells to produce estrogen

Abnormal Vaginal Bleeding 1. dysfunctional uterine bleeding 2. polymenorrhea 3. menorrhagia 4. metorrhagia 5. menometorrhagia 6. oligomenorrhea

1. frequent irregular menstrual periods, prolonged painless bleeding 2. regular intervals <21 days 3. prolonged or excessive uterine bleeding that occurs at regular intervals 4. uterine bleeding that occurs at irregular intervals 5. prologned excessive bleeding that occurs at irregular intervals 6. uterine bleeding that occurs at regular intervals >35 days

burns 1. scalding injury **consider child abuse

1. from hot liquids

Hereditary fructose intolerance 1. patho 2, sx

1. fructose 1 phosphate aldolase B deficieny - 2. hypoglycemia, vomitting, diarrhea, FTT, seizures, avoiding fructose, sucrose, sorbitol

tinea unguium 1. pathogenesis + clinical presentation 2. tx

1. fungal infection of the nails 2. topical antifungals dont work very well, use systemic griseofulvin, terbinafine, ketoconazole

coccidioides immitus 1. pathogenesis + clinical presentation 2. tx

1. fungus found in SW united states and mexico--> asymptomatic/mild pneumonia, **africans, filipinos, pregnant, neonates, immunocompromized --> disseminated meningitis, severe pneumonia, osteomyelitis 2. mild diseaes- no tx, CNS cryptococcal infection includes systemic antifungal

Prader-Willi syndrome 1. pathogenesis 2. clinical presentation 3. complications

1. genomic imprinting caused by the absence of a region on the paternally derived chromosome 15 2. failure to thrive (hypotonia/poor feeding) in 1st year of life→obesity/hyperphagia in later childhood, short stature with small hands/feet, hypotonia, mental retardation, hypogonadism 3. FTT, obstructive sleep apnea (obesity), cardiac disease (obesity), type 2 diabetes

brain tumor CSF findings 1. AFP/B-HCG 2. HVA, VMA, polyamines

1. germ cell tumors 2. medulloblastoma

RBCs in the urine identification 1. RBC casts 2. blebbed RBCs 3. normal biconcave RBCs 4. large numbers of RBCs + dysuria

1. glomerular bleeding~ acute/active glomerulonephritis 2. RBCs originating in the glomerulus 3. lower urinary tract in origin (ie. distal to the glomerulus) 4. acute hemorrhagic cystitis

1. oral rehydration therapy mechanism 2. ORT not used when

1. glucose + electrolytes ~~ electrolyte transport is enhanced by glucose, this leads to increased uptake of solutes~~~ this mechanism is not effected during secretory diarrhea 2. severe life threatening dehydration, paralytic ileus, GI obstruction, rapid stool losses or repeated severe emesis losses

Von-gierke 1. patho + clinical pres 2. tx 3. complications 4. lab findings

1. glucose 6 phosphatase deficiency --> hepatomegaly, HYPOGLYCEMIA DURING FASTING**** metabolic acidosis, hypertriglyceridemia, enlarged kidneys 2. high carboydrate diet 3. hepatocellular carcinoma 4. hyponatremia,hypokalemia due to the fact that they are hyper lipidemic which dilutes serum ion concentrations

Amenorrhea (puberty onset, genitalia, FSH/LH) 1. turners syndrome (XO) 2. hypothalamic or pituitary failure before puberty 3. testicular femininization syndrome (46XY) 4. mayer rokitansky kuster hauser 5. PCOS 6. sheehans syndrome

1. gonadal dysgenesis/ovarian failure, delayed onset puberty, normal genetalia, high FSH/LH 2. delayed onset puberty, normal genetalia, low FSH/LH 3. X-linked androgen receptor defect (androgen insensitivity) - normal female puberty, absent uterus/blind pouch vagina/ inguinal testes, **BILATERAL INGUINAL HERNIAS elevated FSH/LH 4. congenital absense of vagina/uterus , normal pubertal development, NORMAL FSH/LH 5. normal puberty, normal genetalia, high LH:FSH raatio + obesity hirsuitism, acne 6. noraml puberty, normal genetalia, low FSH/LH

Urethritis 1. pathogensis 2. clinical presentation 3. tx

1. gonococcal or non gonoccoccal (u. urealyticum, mycoplasma genitalium, HSV, t. vaginalis, chlamydia) 2. often asymptomatic; dysuria/freq, mucopurulent discharge 3. >5 wbc per high power field on gram stain; >10wbc on first void urine, + LEUKOCYTE ESTERASE, urethral swab + culture 4. ceftriaxone + doxy (same as cervicitis)

1. hand grasp 2. appears/dissapears

1. grab anything placed in palm 2. birth/1-3 months

erysipelas 1. pathogenesis 2. complications

1. group A beta hemoloytic strep --> dermal lymphatics --> TENDER ERYTHEMATOUS SKIN WITH A DISTINCT BORDER~ usually effects the face and scalp 2. post strep glomerulonephritis,necrotizing fasciitis, bacteremia

scarlet fever 1. pathogenesis/clinical presentation

1. group A strep produces erythrogenic toxin --> fever/chills/malaise/exudative pharyngitis--> blanching erythematous scarlatiniform (sandpaper like) rash Pastias lines - petechiae at skin creases form a linear distribution --> desquamation when the infection resolves

adrenal androgens 1. effect in males 2. effect in females

1. growth of pubic hair, linear growth 2. growth of pubic hair, linear growth

Spina bifida 1. external findings 2. myelomeningocoele

1. hair tufts, lipomas, dimples, in the lumbosacral area 2. hernial protrusion of the cord and meninges through a defect in the vertebral canal

RTA type II (proximal) 1. pathogenesis + clinical presentation 2. treatment

1. heavy metals/gentamycin, fanconi syndrome --> impaired reabsorbtion of bicarb by proximal tubule --> vomiting, FTT, acidosis, muscle weakness 2. large doses of alkali

Proportionate (pathologic) short stature 1. define 2. causes ~ pre/post natal

1. height falls more than 3SD below the mean with abnormal growth velocity <2 in/year 2. Prenatal onset-- environmental exposures (e.g. EtOH), chromosomal (down/turner), genetic (russell-silver, prader willi, viral infections (CMV/rubella) Postnatal onset--malnutrition, psychosocial causes (neglect, abuse), organ disease (IBD, RTA, CF)

1. Short stature 2. normal variant short stature 3. pathologic short stature

1. height less than two standard deviations below the mean 2. below the third percentile but normal growth velocity 3. child who is below third percentile but is growing with suboptimal growth velocity *Children who grow 2 inches per year (5 cm per year) between 3 years of age and puberty usually do not have an endocrinopathy or underlying pathologic disorder.)

MPGN 1. biopsy findings - many causes, most patients develop end stage renal disease 2. treatment

1. lobular mesangial hypercellularity, thickening of the glomerular basement membrane 2. corticosteroids/ace inhibitors

enzyme defec+ clinical pres in: 1. tay sachs 2. gauchers 3. niemann pick 4. krabbes 5. fabrys 6. metachromatic leukodystrophy 7. farber disease

1. hexominidase A - 6-12mo inc. startle + cherry red spot, hypotonia, loss of milestones 2. beta glucosidase - erlenmeyer flask femur, HSM, late onset 3. sphingomyelinase- cherry red spot, HSM death by 2 or 3 4. galactocerebroside beta galactosidase 5. alpha galactosidase --> late childhood onset, acroparesthesia, painful crisis (of abdomen), cataracts 6. galactosyl 3 sulfate ceremide sulfatase - age 1-2 years onset --> peripheral neuropathy, progressive ataxia, gray macular lesions 7. ceramidase --> bone pain and joint swelling

1. high grade vs low grade tumor

1. high grade = agressive, proliferative undifferentiated cels low grade = less aggressive, more differentiated cells

VSD 1. physical exam findings 2. ECG fidnings 3. CXR

1. high pitched holosystolic murmur at LLSB, diastolic rumble due to inc. pulmonary flow 2. LVH +/- RVH if there is pulmonary hypertension 3. cardiomegaly, and increased pulmonary vascular markings

MAS 1. evaluation 2. management

1. history of meconium noted at, or before, delivery and the presence of respiratory distress. CXR shows increased lung volume with diffuse PATCHY areas of atelectasis and parenchymal infiltrates alternating with hyperinflation. -pneumothorax or pneumomediastinum may occur 2. oxygen, suctioning the trachea***(first), ECMO, mechanical ventilation

infantile botulism 1. pathogenesis 2. dx/tx *GOOD PROGNOSIS*~ complete resolution of symptoms***

1. honey/dirt -->c.botulinum spores --> 24-48 hour incubation --> inhibition Ach release --> CONSTIPATION--> weak cry/suck, loss of milestones, ophthalmoplegia, hyporeflexia, SYMMETRIC DESCENDING* PARALYSIS 2. dx = toxin /bacteria in stool, EMG shows INCREMENTAL RESPONSE DURING HIGH FREQ. STIMULATION tx = botulism Ig, NG feeding, assisted ventilation, NO ABX

1.transverse fracture 2. oblique fracture 3. spiral fracture 4. comminuted fracture

1. horizontal across the bone 2. diagonal fracture across bone 3. oblique fracture that encircles the bone associated with twisting injury/child abuse*** 4. multiple fracture fragments

necator americanus/ancylostoyma duodenale (hookworm) 1. pathogenesis + clinical presentation 2. tx

1. human feces contaminated soil --> percutaneous infection through bare foot migrate to lungs coughed up and swallowed --> rash/pruritus at site of penetration, IRON DEFICINECY ANEMIA with fatigue, abdominal pain, eosinophilia, pica, pallor, FTT 2. tx = mebendazole, albendazole or pyrantel pamoate (for the whole family)

Cyanosis evaluation 1. initial steps for evaluation of cyanosis 2. 100% oxygen test results interpretation

1. hx/pe/serum electrolytes with serum glucose/ABG/CBC/CXR 100% oxygen test 2. ABG is performed after administration of 100% oxygen (helps evaluate whether cyanosis is caused by cardiac or respiratory disease). a. Oxygen test in infants with heart disease. •with dec. pulmonary blood flow (e.g. TOF)⇒ PaO2 inc. only slightly, usually <10-15 mm Hg. •with normal or inc. pulmonary blood flow (e.g. truncus arteriosus) ⇒ PaO2 usually inc. > 15-20 mm Hg, but >150 mmHg is unusual b. Oxygen test in infants with lung disease. •PaO2 usually inc. considerably, often reaching > 150 mm Hg

renal masses 1. at birth 2. <3 years of age 3. >3 years of age

1. hydronephrosis 2. neuroblastoma (neural crest) <1 year of age 3. wilms tumor (metanephros) age 2-5

1. what metabolic/endocrine abnormalties can cause coma 2. what organs/seizure types """

1. hyper/hypoglycemia hyper/hyponatremia hyper/hypothyroidism hyper/hypocortisolism diabetic ketoacidosis 2. uremia, hepatic failure, reyes syndrome,cardiac arrest, non-convulsive status epilepticus

DKA 1. clinical definition 2. pathogenesis 3. clinical presentation 4. lab dx 5. tx

1. hyperglycemia >300, ketonuria, bicarbonate <15, pH<7.3 2. insulin deficiency--> cells starved of glucose + hyperglycemia --> glucagon/cortisol/growth hormone/epinephrine-->ketogenesis (glucagon) 3. fruity breath vomitting, polyuria, polydipsia, dehydration, abdominal pain, coma (ketones) 4. anion gap metabolic acidsis, hyperglycemia, ketonemia HYPERKALEMIA 5. isotonic saline to correct dehydration, gradual decline in osmolality, potassium REPLETION b/c cellular K+ is depleted, insulin,

Chronic lung disease 1. CXR 2. tx

1. hyperinflation, atelectasiss, linear/cystic chest densities 2. supplemental oxygen, bronchodilators, caloric intake,

vitamin toxicities 1. vitamin a 2. nictotinic acid 3. vitamin C 4. vitmain D 5. vitmain B6

1. hyperosteosis + slowed growth, hepatomegaly, inc. CSF pressure, drying of skin 2. flushing/pruritus, tachycardia, liver damage, hyperglycemia/hyperureciemia 3. kidney stones, diarrhea, cramping 4. Nausea, diarrhea,calcification of heart/kidney/BV 5. sensory neuropathy

pityriasis rosea 1. pathogenesis + clinical presentation 2. tx

1. hypersensitivity to a virus --> herald patch --> 1-2 weeks --> oval erythematous macules and papules of the trunk/legs follows skin lines (CHRISTMAS TREE), pruritic 2. antihistamines, UV light

erythema multiforme 1. pathogenesis + clinical presentation

1. hypresensitivity reaction to various stimuli (infectious/non-infectious)--> targetoid lesions *three types minor/major/stevens johnson syndrome

Acute pancreatitis *uncommon in children 1. pathogenesis 2. clinical presentation 3. dx 4. complications

1. iDIOPATHIC (25%) TRAUMA (MCC), INFECTION, congenital , cystic fibrosis, SLE --> pancreatic duct obstruction or infection-->premature activation of proteolytic enzymes leads to autodigestion of the pancreas 2. periumbilical or epigastric pain that RADIATES TO THE BACK, fever, anorexia, N/V, abdominal distension, GRAY TURNER SIGN ( blue colored flanks), CULLEN SIGN ( blue discoloration of the periumbilical area), hypotension, tachycardia 3. serum amylase, serum lipase (more specific + elevated longer), leukocytosis, hyperglycemia, hypocalcemia (precipitates) elevated transaminases, coagulopathy, abdominal pancreatitsi 4. PSEUDOCYSTS, ARDS, renal failure, shock, GI bleeding 5. supportive, TPN, antibiotics, surgery to remove necrotic tissue

Umbilical hernia 1. pathogensis 2. clinical presentation 3. treatment

1. incomplete closure of the umbilical ring 2. soft swelling beneath the skin around the umbilicus 3. only treat those that last greater than 4-5 years, or if the hernia is >2 cm

legg calve perthes age 4-9, boys >girls 1. pathogeness + clinical presentation 2. dx 3. tx

1. idiopathic avascular necorsis of the femoral head, decreased internal rotation/abduction of the hip (ELICITS PAIN) + PAIN IN THE ANTERIOR THIGH 2. radiographs show increased density of the femoral head, or "crescent sign" subchondral fracture 3. physical therpay, restrict exercise, surgery (if 50% necrosis), self resolves in children <9 years of age (>9 --> osteoarthritis)

growing pains 1. pathogenesis + clinical presentation 2. dx/tx

1. idiopathic bilateral leg pain that occurs afternoon/evening but does not interfere with play, children wake up crying in pain ages 4-12 2. analgesics/reassurance

neonatal hepatitis 1. pathogenesis 2. treatmnet

1. idiopathic hepatitis--> transient jaundice, acholic stools, cirrhosis, portal hypertension (VERY WIDE RANGE OF SEVERITY) 2. nutritional support + ADEK supplements, TPN, liver transplantation

scoliosis 1. pathogenesis + clinical pres 2. dx 3. what degree of scoliosis recquires bracing or surgery - major complications are respiratory or cardiovascular compromise

1. idiopathic or multiple causes --> lateral curvature of the spine 2. Adam's forward bending test and measurement of Cobb angle Tx = bracing prevents progression, surgery prevents progression, or treats sever curves 3. 20-40 deg = brace >40 deg or proggression of 5 deg in 4-6 months

ITP 1 pathoenesis 2. clinical pres 3. dx . tx ~ resoleves spontaneously,

1. idiopathic*, drug induced, 1-4 weeks post-viral** --> antibodies against platelets --> destruction/removal by spleen 2. abrupt cutaneous bleeding (epistaxis/gum bleeding), internal bleeding into the brain/kidneys/GI tract (Rare) 3. thrombocytopenia +LARGE STICKY PLATELETS platelets of blood smear tx = IVIG, corticosteroids, Anti-D immunoglobulin (binds Rh on RBCs allowing platelets to escape the spleen), splenectomy~~~~ no platelet transfusions

congenital cataract 1. pathogenesis + clinical presentation 2. tx

1. idiopathic, downs syndrome, marfan, alport, smith-lemli-opitz, hypoglycemia, galactosemia, CMV/rubella, truama--> amblyopia 2. early surgery

autoimmune hemolytic anemai (AIHA) 1. pathogenesis+ clinical presentation 2. dx 3. tx

1. idiopathic/viral/lymphoma/SLE/immunodeficiency --> autoantibodies against RBCs--> anemia, pallor, jaundice, hemoglobinuria, splenomegaly -->complete recovery ~ prolonged form has high mortality 2. DIRECT COOMBS TEST POSITIVE (antibodies positive, complement positive directed toward RBCs) 3. corticosteroids (most effective for acute form)

1. when should chest compressions begin 2. where is pulse assessed in an infant vs a child

1. if need is determined after two rescue breaths~~~ if the patient is in ASYSTOLE or BRADYCARDIA 2. brachial artery of infants, carotid artery of children

1. what groups are most at risk for helminth infections 2. diagnosis of helminth infections

1. imigrants, travelers, homeless 2 three stool examinations for ova and parasites, cellulose tape test for enterobius

aspergillosis 1. invasive disease clinical pres + tx 2. allergic bronchopulmonary aspergillosis clinical pres _ tx

1. immunocompromised pts --> invasive disease tx = ampho B, resect aspergilloma 2. pts with chronic lung disease (CF etc) --> wheezing, eosinophilia, pulmonary infiltrates ****HIGH levels of aspergillous IgE -- tx = corticosteroids + antifungals

non-hodgkins lymphoma - males>females 1. pathogenesis + clinical presentation 3. lymphoblastic lymphoma - clinical pres 4. small non-cleaved cell lymphoma (two types) - clinical pres 5. large cell lymphoma - clinical pres

1. immunodeficiency states/HIV/wiskott aldrich/ataxia telangiectasia/EBV --> painless lymphadenopathy, 3. T cell - similar to lymphoblasts in ALL, presents as anterior mediastinal mass, SVC syndrome, airway obstrucion 4. often presents as intussuception*********** Burkitts presents as jaw mass 5. enlargment of lymphoid tissue of tonsils, adenoids, peyers patches

1. what features during interview with parents is suspicious for abuse 2. diagnosis of abuse

1. implausable histories, histories that change or are inconsistent 2. dilated ophthalmological exam, bone skeletal survey to find old fractures

internal tibial torsion 1. pathogenesis + clinical presentation 2. tx

1. in utero positioning --> medial rotation of the tibia (MCC in-toe-ing <2 years of age), patella faces forwards, usually bilateral 2. observation

HIV 1. pathogenesis

1. in utero, intrapartum, post partum transmission from mother to chlid is ~5% by using anti-retrovirals durign pregnancy Also: sexual contact, blood products, sharing needles

definitions 1. ataxia 2. cerebellar dysfunction gait 3. weakness in gait - also encephalopathy, seizures +post ictal, vision problems, migraines, acute labyrinthitis, brainstem tumors

1. inability to coordinate muscle activity during voluntary movement 2. unsteady wide based gait, irregular steps/veering 3. spinal cord lesions, or disorders of the motor unit

Define shock

1. inadequate delivery of oxygen/metabolic substrates to meet metabolic demands of tissue +/- decreased blood pressure

1. define CHF 2. three major physiologic responses that occur as a result of congestive heart failure

1. inadequate oxygen delivery by the myocardium to meet metabolic demands of the body 2. hypoperfusion of end organs --> inc. contractility and heart rate •hypoperfusion --> kidneys retain salt and water through RAAS •SNS --> inc catecholamines which increase heart rate/contractility

GERD 1. pathogenesis in children 2. clinical presentation in infants 3. sandifer syndrome

1. inappropriate transient lower esophageeal sphincter relaxation OR gastric emptying delay --> prolonged contact of esophageal mucosa with gastric contents --> INFLAMMATION 2. emesis, FTT, sandifer syndrome, feeding refusal (irritation of eating), constant hunger* (buffering action of milk) 3. torticollis with arching of the back due to painful esophagitis

Maternal hyperglycemia 1. pathogenesis 2. clinical presentation 3. associated clinical disease

1. inc. glucose →fetal hyperinsulinemia → inc. protein synthesis, inc. lipogenesis, macrosomia 2. visceromegaly (liver, adrenals, heart), increased skeletal length with small head/face, round facies 3. congenital heart disease, small left colon syndrome→failure to pass meconium + abdominal distention

polycythemia 1. definition 2. primary polycythemia (vera) 3.appropriate polycythemia 4. inappropriate polycythemia

1. increase in relative RBCs, Hct >60% 2. malignancy of RBC precursors 3. CHF, pulmonary disease, cyanotic heart disease, high altitude living 4. tumors of the kidney, cerebellum, ovary,liver, adrenal, hydronephrosis, corticosteroids,

OTC def 1. patho 2. dx/tx

1. increased ammonia in teh blood from dec formation of citrulline --> vomiting, lethargy, coma ~ females have cyclic vomiting and intermittent ataxia 2. elevated orotic acid, elevated ornithine, decreased citrulline-- low protein diet,

effects on bone age 1. excess androgens (ex. CAH) 2. thyroid deficiency 3. glucocorticoid excess 4. inorganic FTT

1. increased bone age and early fusion of the growth plate 2. decreased bone age 3. increased boen age and rapid fusion of the growth plates 4. decreased bone age due to low pituitary hormones

1. what is the source of unconjugated bilirubin, how is it converted to conjugated bilirubin 2. what conditions decrese bilirubin excretion

1. increased heme load (polycythemia,hematoma,hemolysis) --> UDP glucuronyl transferase--> conjugated bilirubin --> excretion into the biliary system 2. hepatitis, liver fialure, biliary atresia, choledochal cyst

Primary dysmenorrhea 1. pathogenesis 2. clinical presentation 3. tx

1. increased production of prostaglandings by endometrium --> excessive contractions + systemic effects ~ pain with menstrual flow 2. spasms, N/V, diarrhea, headache, fatigue 3. prostaglandin inhibitors (NSAIDs), OCP

estradiol 1. effect in males 2. effect in females

1. increases rate of epiphyseal plate fusion 2. breast dev, midcycle luteinizing hormone, labial/vaginal/uterine dev, proliferation of endometrium, *low level = linear growth *high level = inc. epiphyseal plate fusion

Live polio (sabin) 1. advantages 2. disadvantages

1. induces host immunity + herd immunity (secondary immunity) because the vaccine is excreted in the stool 2. vaccine related polio

neutropenia 1. types of infections with mild neutropenia (1000-1500) 2. types of infections with severe neutropenia (<500)

1. infection of mucuous membranes: stomatitis, cellulitis, gingivitis 2. pneumonia, sepsis, meningitis (s.aureus, klebsiella, serratia, e.coli, pseudomonas)

pericarditis 1 casues 2. viral causes 3. bacterial causes (purulent) + complications 4. pathophysiology

1. infection, collagen vascular disease, uremia, postpericardiotomy syndrome (autoimmune reaction after the pericardium has been opened [+viral]) 2. MOST COMMON CAUSE *** coxsackie, echo, adeno, influenza, parainfluenza, EBV 3. staph aureus, and strep pneumoiae --> constrictive pericarditis** 4. inflammation of hte pericardium -->exudate/transudate-->impaired venous return/cardiac filling --> CARDIAC TAMONADE

Epidydymitis 1. pathogenesis 2. clinical presentation 3. dx 4. tx

1. inflammation of the epididymis secondary to gonorrhea or chlamydia**** 2. acute onset scrotal pain/swelling with pain/tender epididymis 3. increased wbc on urinalysis, positive urine culture, doppler ultrasound shows INCREASED FLOW, and INCREASED RADIONUCLEOTIDE SCAN 4. same as cervicitis

atopic dermatitis (eczema) - commonly runs infamilies 1. clinical presentation 2. diagnostic criteria 3. management

1. inflammatory dermatitis characterized by dry skin and lichenification, worse with extremes of temperature, pruritus, erythema, weeping, crusting-- tends to effect FLEXOR SURFACES 2. pruritus, personal/family hx of atopy, morphology/distribution, chonic dermatitis, 3. avoid triggers, low/medium corticosteroids, antihistamines, baths in warm water + skin moisturizers

Septic shock 1. pathogenesis 2. two stages

1. inflammatory response to an organism 2. hyperdynamic stage- bounding pulses*, normal or high cardiac output, warm extremities, wide pulse pressure decompensated stage - impaired mental status, cool extremities, diminished pulses

Laryngotracheobronchitis (croup) 1. definition 2. pathogenesis + clinical pres 3. CXR 4. age group effected + time of year

1. inflmmation/edema of the SUBGLOTTIC larynx, trachea, bronchi 2. Parainfluenza (MCC), RSV, rhinovirus, adeno, influenza A/B, myocoplasma -->URI for 2-3days --> inspiratory stridor (worse at night and with agitation), barking cough/hoarse voice, lasts 3-7 days, 3. AP film shows STEEPLE SIGN OF SUBGLOTTIC NARROWING 4. age 3 months = 3 months to 3 years fall and winter

most common infra/supratentorial tumor in children

1. infra = medulloblastoma supra = astrocytoma

entamoeba histolytica 1. pathogenesis + clinical presentation 2. clinical presentation 3. dx/tx

1. ingestion of cysts in contaminated food or water --> invasion of colonic mucosa--> mild colitis or sever dysentary (young children/pregnant/immunocomp) cramping, abdominal pain, tenesmus, diarrhea with blood or mucus, weight loss, hepatomegaly, chest pain/right shoulder pain, respiratory distress 2., intestinal perforation/hemorrhage, strictures, ameboma (local inflammatory mass), ABSCESS IN THE LIVER**** (also brain/lung/other) 3. trophozoiites/cysts in stool, colonoscopy/biopsy, CT scan of liver tx = metronidazole, iodoquinol (luminal amebicide)

trichuris trichiuria 1. pathogenesis + clinical presentation 2. tx

1. ingestion of eggs --> abdominal pain, tenesmus, bloody diarrhea, RECTAL PROLAPSE 2. tx = mebendazole, albendazole or pyrantel pamoate (for the whole family)

Mycobacterium tuberculosis 1. pathogenesis + clinical presentation 2. cxr 3. definitive dx

1. inhalation of small airbone droplets --> fevers, chills weight loss, cough, night sweats, cervical lymphadenitis (most common extra-pulmonary TB in children), meningitis, ileitis, bone involvement (potts diseaes), dissemination or miliary disease 2. hilar/mediastinal lymphadenopathy, ghon complex, pleural effusion/lobar involvement, cavitary disease, PPD test (will be + 2-12 weeks after exposure), 3. positive culture for M.tuberculosis, positive staining for acid fast bacilli,caseating granulomas from biopsy speciment

RTA type 1 1. pathogenesis + clinical presentation 2. treatment

1. inherited, nephrotic synd, ampho B --> inability of distal tubule to excrete acid--> comiting, FTT, acidosis, nephrocalcinosis/nephrolithiasis 2. small dosese of alkali

cutaneous larva migrans 1. pathogenesis + clinical presentation 2. tx

1. intradermal migration of dog or cat hookworms after contact with feces-> migrating serpiginous erythematous tracks on teh skin 2. self limited, or ivermectin, thiabendazole, albendazole

metatarsus adductus (in toeing) 1. pathogenesis + clinical presentation 2. treatment

1. intrauterine constraint (ie. first child primigravid uterus) --> medial curvature of the midfoot with passive dorsiflexion and flexibility preserved 2. passive motion/stretching (dont over correct), stiff foot that cannot be straightened must be cased for 3-6 weeks

out toe-ing 1. pathogenesis + clinical presentation 2. treatment

1. intrauterine constraint(first child) --> calcaneovalgus foot = flexible foot held in a lateral position 2. stretching and casting

treatment of upper GI bleeding

1. intravenous access with two large boor peripheral lines + fluid bolus of 20mg/kg***** of normal saline, octreotide(vasoconstrictor), H2/PPI for ulcers, endoscopic therpay, arteriographic embolization (for vascular malformations)

RDS 1. acute complications 2. chronic complications* 3. bronchopulmonary dysplasia dx

1. intraventricular hemorrhage, air leaks e.g. pneumothorax, sepsis, R-L shunt across PDA (cyanosis) 2. bronchopulmonaryu dysplasia- progressive pathologic changes in the immature lung affecting parenchyma/airways 3. mechanical ventilation during first 2 weeks of life, need for O2 beyond 28 days of life, respiratory compromise >28 days of life, CXR

Abnormalities of Morphogenesis: 1)malformation 2)deformation 3)disruption

1. intrinsically abnormal process forms abnormal tissue (e.g. bladder extrophy) 2. mechanical forces exerted on normal tissue result in abnormal tissue (ex. amniotic bands) 3. normal tissue becomes abnormal after subject to destructive forces (Ex. ischemia resulting in atretic organ)

definitions 1. seizure 2. epilepsy 3. status epilepticus

1. involuntary alternation of consiousness, motor activity, sensation or autonomic function caused by excessive discharge from a population of cerebral neurons (due to excessive excitatory, or deficient inhibitory activity) 2. >2 spontaneous seizures without obvious precipitating cause 3. seizure >30 minutes during which the patient doesnt regain consciousness

femoral anteversion 1. pathogenesis + clinical presentation 2. treatment

1. inward anteversion with both feet and patella being inward facing, increased internal rotation of the hips, child prefers sitting in W POSITION*** 2. observation, res. by 8 years of age

1. clinical presentation of lead poisoning 2. hematologic findings of lead poisoning 3. radiologic findings of lead poisoning

1. irritability, seizures, listlessness, colicky pain, decreased consciousness/encephalopaty, 2. peripheral smear shows MICROCYTIC ANEIMA WITH BASOPHILIC STIPPLING 3. dense metaphyseal bands on abdominal radiographs lead lines on the knees and wrists

sickle cell clinical features 1. vasocclusive crisis + tx 2. acute abdominal crisis + tx 3. stroke 4. priapism 5. acute chest syndrome 6. sequestration crisis

1. ischemia/infarction of bones, dactylitis, pain, 2x IV maintenance fluids, partial exchange transfusion, incentive spirometry 2. sickling in the mesenteric artery --> abdominal pain/distension + 2x IV maintenance fluids, partial exchange transfusion, incentive spirometry 3. dysarthrisa, hemoplegia + 2x IV maintenance fluids, partial exchange transfusion, incentive spirometry 4. painful sustained erection + 2x IV maintenance fluids, partial exchange transfusion, incentive spirometry 5. mycoplasma, chlamydia, s.pneumo --> new pulmonary infiltrate with respiratory symptoms, hypoxemi + 2x IV maintenance, oxygen, cefuroxime/azithro, incentive spirometry, partial exchange transfusion 6. rapid accumulation of blood in the spleen --> abdominal distension/pain, SOB, tachycardia, pallor, fatigue, shock

renal parenchymal tubular damage (renal failure) 1. pathogenesis 2. lab findings

1. ischemic injury from hypoperfusion/heme pigments/aminoglycosides-->damage to the tubules (ATN) 2. increased urinary B2 MICROGLOBULIN, FENa >1% or >2.5% in neonates

1. what is the most common cause of cardiac arrest in a child 2. what should be done for a child in cardiac arrest

1. lack of oxygen supply --> choking, suffocation, near drowning, lung disease etc 2. CPR

DIC 1. pathogenesis 2. clinical presentation 3. dx/tx

1. large hemangiomas, sepsis, hypothermia, malignancy, heat stroke, snake bites, burns-->clotting/consuption of procoagulant factors -->HEMORRHAGE 2. cutaenous and internal bleeding 3.dx = thrombocytopenia (prolonged BT), prolonged PT and PTT, reduced clotting factors, low fibrinogen*, low II,V,VIII, ELEVATED D-DIMERS (fibrin degradation products)+ smear shows schistocytes and helmet cells tx = fibrinogen, FFP, platelets, heparin

Intussusception *most common in males 5-9 months of age 1. pathogenesis 2. dx/tx

1. lead point (meckels diverticulum, polyp, intestinal dupication, peyers patch, lymphoma) --> telescoping of distal ileum into the colon --> BOWEL WALL EDEMA AND HORMORRHAGE/ISCHEMIA/INFARCTION 2. sudden onset episodic crampy or colicky abdominal pain, infant draws legs toward chest, vomitting/lethargy, CURRANT JELLY STOOLS, SAUSAGE SHAPED PALPABLE MASS in the right upper quadrant 3. contrast enema both to diagnose (COIL SPRING SIGN), and will also reduce the intussception - if this doesnt work can use operative redution

wilms tumor staging

1. limited to kidney + complete excision w/o rupture 2. tumor extends locally but can still be excised 3. residual tumor remains in abdomen, or spillage durign resection 4. bilateral renal involvment

testosterone 1. effect in males 2. effect in females`

1. linear growth/muscle mass, penis, scrotoum, prostate, seminal vesicle development, pubic/axillary/facial hair, deepens voice, inc. libido 2. stimulates linear growth, stimulates growth of pubic/axillary hair

1. ointment 2. cream 3. lotion

1. little or no water, max water retaining properties 2. 20-50% water, average dryness 3. > water than cream, used for minimally dry skin or large surfaces

liver disease coagulopathy 1. pathogenesis 2. clinical presentation 3. dx/tx

1. liver diseaes--> dec clotting factor synth especially vitamin K dependent factors 2. same as vitamin K def 3. dx = prolonged PT and PTT, increased fibrin degradation products, thrombocytopenia tx = vitamin K, FFP, platelets

1. what is AST a marker for 2. what is ALT a marker for 3. what is LDH a marker for 4. what is ALP a marker for 5. what is GGTP/5NT a marker for

1. liver, skeletal muscle, RBCs, cardiac tissue 2. VERY SPECIFIC LIVER MARKER**** 3. Liver, and HEPATOCELLULAR NECROSIS 4. BILIARY DISEASE, BONE (rapid growth in kids), KIDNEY, INTESTINAL DISEASE, TRAUMA 5. biliary disease (5NT is more specific)

patellofemoral syndrome 1. pathogenesis + clinical presentation 2. dx/tx

1. malalignment of the patella --> knee pain worst with activity or running 2. patella in the lateral position on "sunrise" radiograph, Tx = rest, stretching, strengthening the medial quadriceps

v. cholerae 1. clinical pres 2. dx/tx

1. massive watery diarrhea 2. history of living in endemic area or travel, culture, serology tx = fluid replacement

what materanl disease is associated with 1. neonatal high output heart failure 2. third degree heart block

1. maternal hyperthyroidism/graves 2. maternal SLE

post strep glomerulonephritis 1. renal biopsy findings 2. treatment 3. does abx treatment reduce the risk of post strep glomerulonephritis

1. mesangial cell proliferation, increased mesangial cell matrix, lumpy bumpy immunoflursent, subEPIthelial deposits on electron microscopy 2. fluid restriction, antihypertensive drugs, restriction of protein/sodium/potassium/phosphorus 3. NOPE, only dec. risk of rheumatic fever, and

Jejunal atresia 1. pathogenesis + clinical presentation 2.dx/tx

1. mesenteric vascular accident during fetal life--> obstruction/reabsorbtion of the damaged jejunum--> billlous emesis and abdominal distension 2. air fluid levels and contrast studies show atresia, "TRIPLE BUBBLE ON CXR tx = NG suctioning, and surgical resection/anastomosis of the atretic small bowel

salicylates poisoning 1. pathogenesis 2. clinical presentation 3. lab findings

1. metabolic acidosis from the weak acid, but it STIMULATES THE RESPIRATORY CENTER -> respiratory alkalosis + UNCOUPLE OXIDATIVE PHOSPHORYLATION --> lactic acidosis and enhancing ketosis 2. fever, diaphoresis, tinnitus, vomiting, headache, lethargy, restlessness, coma, seizures 3. respiratory alkalosis with an anion gap metabolic acidosis, hyperglycemia-->hypoglycemia, hypokalemia

1. what kind of bone fractures characteristic of abuse

1. metaphyseal fractures (bucket handle or corner fractures) from torsional force or violent shaking posterior/first ribs, sternum, scapula, vertebral spinous processes *MULTIPLE fractures at different stages of healing

cystercercosis 1. pathogenesis + clinical presentation 2. dx/tx

1. mexico/central america fecal oral taenia solium eggs --> encyst in muscle, subcutaneous tissue, subcutaneous nodules, 4th ventricle, meninges, spine, eyes --> SEIZURES (most common presenting symptom), hydrocephalus, stroke 2. ova and parasite stool, head CTshowing parenchymal cysts/calcifications(non-viable) tx = anti-convulsant therapy

micro/macrovascular complications of diabetes

1. micro = retinopathy, nephropathy, neuropathy 2. atherosclerosis, hypertension, heart disease, stroke

Pierre robin syndrome 1. clinical features 2. Complications

1. micrognathia, cleft lip and palate, and a large protruding tongue. 2. recurrent otitis media and upper airway obstruction that often requires tracheostomy

1. clinical presentation of aseptic meningitis 2. clinical presentation of tuberculosis meningitis

1. mild fever, headache, emesis, altered level of consciousnss, seizures 2. initially lethargy or irritability, but then progresses to cranial nerve defecits, LOC, paraplegia,death

candida 1. disease in immunocompetent 2. disease in immunocompromised

1. mild superficial infections, oral thrush, diaper dermatitis, vulvovaginal candidiasis 2. fungemia, meningitis, osteomyelitis, endophthalmitis

nephrotic syndrome 1. most common nephrotic syndrome in children 2. pathogenesis 3. clinical presentation

1. miniaml change disease (90%) 2. membranous, membranoproliferative, IgA, minimal change disease --> loss of normal charge/size barrier--> hypoproteinemia from urinary loss of proteins, hypercholesterolemia from from dec. plasma oncotic pressure, decreased lipoprotein lipase activity 3. upper respiratory tract infection --> HELLP symptoms + thrombosis (due to hypercoagulablity) + spontaneous bacterial peritonitis, pneumonia, sepis1

strabismus 1. pathogenesis + clinical presentation 2. tx

1. misalignment of the eyes, esotropia, exotropia, vertical strabismus --> suppression of the suppression of the deveiated eye -->amblyopia (<5), diplopia (>7) 2. patching, corrective lenses, surgery

1. behavioral signs of depression 2. physical signs of depression 3. psychological signs of depression

1. missing school, change in school performance, acting out, lack of interest in activities 2. abdominal pain, headaches, weight loss, overeating, insomnia, anxiety, fatigue 3. sadness, feelings of hopelessness, low self-esteem, excessive self-criticism, and feeling worthless.

molluscum contagiosum 1. pathogenesis + clinical presentation 2. tx

1. mollusci pox virus --> flesh colored papules with central umbilication ~~~ very high numbers of eruptions in pts with HIV 2. resolves without tx , can also use curettage, podophyllin, 0.9% catharidin, liquid nitrogen

neonatal ITP 1. pathogensis

1. mother has ITP and antibodies against platelets cross placenta and destroy fetal platelets, mother also has thrombocytopenia

alloimmune hemolytic anemia 1. ABO hemolytic anemia 2. tx

1. mother is blood group O, and fetus is A,B or AB mother produces IgG anti-AB which passes through placenta ~ occurs during FIRST PREGNANCY**** (direct coombs positive) 2. phototherapy

1. what is the most common cause of death in children >1 year 2. why are injuries more common in a child

1. motor vehicle accidents ~ trauma 2. because head is larger % of total body mass in a child , shorter neck w/ greater weight • pliable rib cage leads to more spleen/liver injuries • ligaments stronger than growth plates --> growth plate injuries

PKU 1. clinical pres 2. dx/tx

1. mousy or musty odor, hypotonia, eczema, hypopigmentation, 2. inc. phenylalanine, inc. phenylpyruvate and phenylacetic acid and dec tyrosine in the urine~~ phe restricted diet

ulcerative colitis 1. pathogenesis 2. clinical presentation 3. complications

1. mucosally limited and effects only the colon, starts at rectup and progresses (ulcerative proctatitis --> pancolitis) 2. rectal bleeding, nocturnal stool, cramping, tenesmus, weight loss, anemia, fever, leukocytosis/hypoalbuminemia (severe) 3. toxic megacolon --> fever, abdominal distension, septic shock, colon cancer

systemic onset JRA (Stills disease) 1. clinical presentation 2. general JRA laboratory findings 3. what fraction fully recover, what fraction go on to develop chronic destructive arthritis

1. multiple large/small joints inc. sternoclavicular Male = female, HIGH SPIKING feveres, >39, transient evanescent non-pruritic salmon colored rash, HSM, lymphadenopathy, fatigue, anorexia, weight loss, FTT, serositis (pericariditis, pleuritis), myositis, tenosynovitis, meningitis, encephalopathy 2. microcytic hypochromic aneima (AOCD), elevated ESR/CRP/platelets, RF usually negative, ANA 75% postive in early onset pauciarticular, and 50% polyarticular 3. 50/50

tension headaches 1. pathognesis 2. treatment

1. muscle contraction --> bifrontal or diffuse dull aching, rarely throbbing increases intensity during day, contraction of temporalis, masseter, trapezius, NO VOMITTING, VISUAL CHANGES, OR PARESTHESIAS 2. acetaminophen, ibuprofen, stress/anxiety reduction

Ehlers-Danlos syndrome 1. pathogenesis 2. clincal presentation 3. complications

1. mutation of type V collagen 2. hyperextensibility of joints, dislocation/ scoliosis, velvety skin, with tissue paper thin scars, mitral prolapse, aortic root dilation, fragile blood vessels*, constipation, rectal prolapse, hernias 3. aortic dissection, GI bleeding

erythema multiforme major 1. pathogenesis + clinical presentation 2. tx

1. mycoplasma pneumoniae/drugs --> low grade fever/arthralgias/myalgias--> symmetric targetoid lesions of the trunk AND extremeities + TWO mucus membrane surfaces 2. erythromycin for m.pneumoniae, stop drug

toxins/antidotes 1. acetaminophen 2. anticholinergics 3. benzodiazepines 4. black widow spider venom 5. carbon dioxide 6. coral snake 7. cyanide

1. n-acetyl cystine 2. physostigmine 3. flumazenil 4. antivenin lactrodectus mactans 5.oxygen 6. antivenin micuris fulvius 7.amyl nitrate, sodium nitritie, sodium thiosulfate, hydroxocobalamin

sinusitis 1. acute perisitant 2. acute severe 3. subacute 4. chronic

1. nasal discharge AND****cough 10-30 days, headache, malodorous breath 2. HIGH FEVER with purulent discharge >3-5 days 3. same as acute perisistant but 30-90 days 4. same as acute persistant but >90 days

Marasmus 1. pathogenesis + clinical pres

1. near starvation from PROTEIN AND NON-PROTEIN DEFICIENCY --> loss of muscle/body fat

1. diagnosis of brain tumor 2. brain tumor treatment - astrocytomas 35%-75% survival (depends on grade) -PNET - 75% survival - brainstem gliomas - poorest prognosis

1. neuroimaging MRI>CT CSF 2. surgical debulking, and radiation, chemotherapy

Motor development red flags 1. persistent fisting >3mo 2. early rolling over, early pull to stand, persistant toe walking 3. spontaneous postures such as scisoring or frog leg in hypotonic infant 4. early hand dominance before 18 months of age

1. neuromotor problems 2. spasticity 3. motor abnormalities (cerebral palsy) 4. motor weakness of the opposite upper extremity

headaches 1. primary headache 2. secondary headache

1. neuron or muscular in origin 2. increased ICP, meningeal irritation (meningitis/ subarrachnoid hemorrhage)

sickle cell trait (HbA/S) clinical presentation

1. no anemia, no crisis (EXCEPT severe hypoxemia), normal spleen, no prophylactic penicillin ****ONLY CLINICAL MANIFESTATION = INABILITY TO CONCENTRATE URINE OR HEMATURIA

Amenorrhea 1. primary amenorrhea 2. secondary amenorrhea 3. dx*** 4. what does high vs low FSH+LH indicate

1. no menstrual bleeding by age 16 with normal secondary sex characteristics, or age 14 with no secondary sex characteristics 2. absence of menses for three menstrual cycles or 6 months after regular menstrual cycles have occurred 3. PREGNANCY TEST FIRST***, TSH/T3, fasting prolactin**, FSH/LH 4. high indicates ovarian failure, low indicates hypothalmic/pituitary suppression

EPEC 1. clinical presentation 2. dx/tx

1. non-invasive watery diarrhea in preschoolers 2. absent stool WBCs stool culture tx- quinolones or sulfonamides, hydration

ETEC 1. clinical presentation 2. dx/tx

1. non-invasive watery diarrhea-- MCC travelers disarrhea 2. absent stool WBCs, culture tx = quinolones or sulfonamides, hydration

hypertension 1. clinical presentation 2. physical exam

1. non-specific signs/symptoms, irritabliity, vomitting, FTT, seizures, headaches, stroke, 2. four limb blood pressures (coarct), fundoscopic examination whows hemorrhages, papilledema, AV nicking, CHF findings, cafe au lait spots, abdominal masses

1. compensated shock 2. decompensated shock 3. irreversible shock

1. normal blood pressure, adequate tissue perfusion but maldistribution to essential organs 2. hypotension, low cardiac output, inadequate tissue perfusion 3. cell death refractory to medical treatment

blounts disease (tibia vara) 1. pathogenesis + clinical presentation 2. dx/tx

1. obese African American boys who are early walkers, overload--> medial tibia growth plate injury --> inhibited growth only on the meidal side--> PROGRESSIVE UNILATERAL BOWING, >2 years of age --> recurrence of angulation/osteoarthritis if not corrected 2. dx = metaphyseal diaphyseal angle >11 degrees tx = bracing, surgery if there is no improvemnt, or if the patient is >4 years of age

1. meconium plug 2. meconium ileus 3. what disease is meconium ileus associated with 4. when is meconium usually passed

1. obstruction of left colon and rectum by dense dehydrated meconium 2. occlusion of the distal ileum caused by inspissated (thickened &dried)meconium 2' deficiency of pancreatic enzymes leading to abnormally high protein content of intestinal secretions 3. cystic fibrosis 4. within 24 hours after birth in 90% of term infants and within 48 hours in 99%

RTA type IV 1. pathogenesis + clinical presentation 2. treatment

1. obstructive uropathy, aldosterone deficient states, diabetes --> transient acidosis + HYPERKALEMIA --> asymptomatic or FTT 2. furosemide, lower serum potassium, oral alkali

breathing pattern indications 1. hypoventilation 2. hyperventilation 3. cheyne stokes 4. apneustic 5. ataxic or agonal

1. opiate or sedatie overdose 2. metabolic acidosis (deep rapid breathing) 3. alternating apneas and hyperpneas (bilateral cortical injury) 4. pons damage (pausing at full inspiration) 5. irregular respirations with no patter - medullary injury or impending brain death

poisoning eye findings 1. miosis 2. mydriasis 3. nystagmus 4. retinal hemorrhages

1. opiates, organophosphates, phencyclidine, phenothiazines 2. amphetamines, cocaine, TCA, atropine 3. dilantin (phenytoin), phencyclidein 4. carbon monoxide/methanol

meckels diverticulum 1. pathogenesis + clinical presentation 2. diagnosis + tx

1. outpouching of the terminal ileum remnant of the vitelline duct, contains ectopic gastric mucosa that produces acid,---> ulceration and painless lower GI bleeding/melena (or lead point for intussuception) 2. nuclear medicine scan*** shows ectopic gastric mucosa tx = surgical resection

knock knees (genu valgum) 1. pathogenesis + clinical presentation 2. tx

1. over correction of genu varum--> seperation of ankles with knees together, swinging legs laterally with walking/running --> osteoarthritis if not treated 2. surgery if >10 years old

Secondary dysmenorrhea 1. pathogenesis 2. clinical presentation 3. tx

1. pain with menstrual flow secondary to endometriosis, PID, uterine polyps or fibroids, bicornuate uterus 2. same as primary 3. same as primary + treat underlying cause

rhabdomyosarcoma -MCC soft tissue sarcoma in childhood - higher risks in patients with NF1/2 1. clinical presentation 2. dx/tx

1. painless soft tissue mass, head and neck, orbital (propotosis/chemosis/CN palsies), nasopharyngeal (epistaxis/airway obstruciton), laryngeal (hoarsness) genitourinary tract (hematuria/obstruction), painless mass in the extremities 2. dx = painless enalrging mass, CT or MRI tx = complete surgical resection

clinical presentation of SVT dx of SVT tx of SVT

1. palpitations, chest pain, dyspnea, altered level of consciousness, CHF symptoms (esp. in neonates) 2. ECG, will see delta wave for WPW (slurredupstroke of QRS) with short PR interval 3. vagal manuevars (valsalva), icepack on face*, carotid massage, invert the child,orbital pressure IV adenosine synchornized cardioversion digoxin/propranolol radiofrequency catheter ablation to destroy accessory paths

ALL chemotherapy side effects 1.asparaginase and corticosteroids 2. doxorubicin 3. cyclophosphamide 4. cranial irradiation

1. pancreatitis 2. cardiomyopathy 3. cystitis 4. mental retardation, stroke, hormonal problems

symptoms ~~~ what are the signs 1. headache 2. vomitting 3. stiff neck 4. double vision 5. transient loss of vision 6. episodic severe headache 7. gait disturbance 8. dulled intellect 9. irritability

1. papilledema 2. cranial nerve palsies 3. stiff neck 4. head tilt 5. retinal hemorrhage 6. macewen's sign (skull percussion hyperresonsnace) 7. obtundation 8. unconsiousness 9. progressive hemiparesis

when does a patient with burn need hospitalization

1. partial thickness burns > 10% BSA full thickness burns >2% BSA inhalation perineum, hands, feet, burn overylying joints non accidental trauma

erythema infectiosum (fifth disease) 1. pathogenesis + clinical presentation 2. complications 3. tx

1. parvo B19 by respiratory secretions--> upper URI --> slapped cheek rash (bright red macular rash)-> lacy reticular rash on the trunk +/- arthralgias 2. aplastic crisis, prolonged anemia, fetal hydrops in pregnant women 3. none- supportive, IVIG for chronic anemia in immunosuppressed

parvo B19 pure red cell aplasia 1. pathogenesis + clinical pres 2. dx 3. tx

1. parvovirus (also EBV/CMV/HIV/chlorampheicol) --> URI symptoms/slapped cheeks fifth disease--> aplastic crisis in SS disease, ASYMPTOMATIC ANEMIA IN HEALTHY CHILDREN 2. dec Hgb, dec. retics, normal plateltes 3. sponatneous recovery in 2 weeks, RBC transfusion for SS

HSV1 /HSV2 1. pathogenesis + clinical presentation 2. dx/tx

1. passage through birth canal--> gingivostomatitis + grouped lesions on an erythematous base, meningitis, meningoencephalitis, herpetic whitlow--> dormant in the DRG -->recurrent infections 2. dx =- tzank preparation, direct fluorescent antibody testing, PCR for CSF tx = acyclovir for cutaneous HSV,

isoimmune neutropenia

1. passive transfer of anti-neutrophil antibodies from mother to fetus after maternal sensitization by antigens on fetal neutrophils ~~~ resolves in infant by 8 weeks of life

Asthma 1. assessment of disease 2.tx

1. peak expiratory flow rates, and keeping journal of the values 2. AVOID TRIGGERS, beta adrenergic agonists, systemic corticosteroids, cromyln/nedocromil sodium (prophylaxis), anticholinergics (atropine/ipratropium), leukotrienne modifiers (montelukast/zafirleukast), methylxanthines (theophylline)

louse infection 1. pathogenesis + clinical presentation 2. tx

1. pediculus humanus, phthirus pubis --> sharing of clothes, combs--> pubic lice attaches to skin and ingests blood --> itching 2. head lice- 1% permethrin shampoo _ comb, 5% permethrin/malathion for resistant lice, body lice - 12 hour application of 1% y-benzene hydrochloride lotion

urticaria (hives) 1.pathogenesis + clinical presentation 2. chronic urticaria pathogenesisi

1. penicillin, NSAIDs, eggs, nuts, latex, GABHS, EBV, venoms, heat/cold, malignancy, SLE --> circumscirbed raised evanescent areas of edema that are pruritic 2. malignancy IgG anti IgE

drug allergy 1. what common drugs cause allergy 2. clinical presentation 3. tx

1. penicillin, sulfonamides, cephalosporins, aspirin/NSAIDS, narcotics 2. urticaria, angioedema, anaphylaxis 3. antihistamines, epinephrine, corticosteroids, beta agonists

hepatitits C 1. pathogenesis 2. clinical presentation (acute/chronic) 3. diagnosis

1. perinatal vertical , transfusion, parenteral --> 2. rarely symptomatic acute infection chronic infection occurs in 80% of infected--> cirrhosis and hepatic fibrosis

hepatitis B 1. pathogenesis 2. clinical presentation (acute/chronic) 3. diagnosis

1. perinatal vertical exposure, parenteral route (IVDU, needle expossure, blood products, body secretions) --> incubtion--> sx + virus in blood,tears, saliva, semen, urine, feces, breast milk 2. acute - nonspecific systemic illnesses, or clinical heptaitits, or fulminant liver failure chronic (MCC in infants)- cirrhosis, hepatic fibroisis, protal hypertension, inc. risk for HCC

Peripheral precocious puberty (PPP) or heterosexual gonadotropin-independent puberty 1. pathogenesis + clinical presentation 2. diagnostic test

1. peripheral production of sex steroids that are not FSH/LH mediated -> Boys: gynecomastia or pubic hair with NO TESTICULAR ENLARGEMENT (because NO FSH***) Girls: virilization or breast development 2. flat GnRH stimulation test because HPGA axis is not activated

nonorganic abdominal pain 1. causes/risk factors (psychosocial/family history) 2. what past medical history of the child inc. the risk **the healthier the child appears, the more likely that the pain is functional and not organic

1. personality, birth order, life stressors, change in shcool/nanny, family stressors, familial alchoholism, ADHD, family members with functional pain syndromes 2. pregnancy problems, intrapartum problems, Cesarean section, enuresis, nightmares

CML 1. adult type CML pathogenesis 2. adult type clinical presentation 3. juvenille CML pathogenesis 4. juvenille CML clinical presentation

1. philidelphia chromosom t(9;22) BCR-Abl fusion kinase 2. fatigue, weight loss MASSIVE splenomegaly, WBC>100k*** 3. abnormalities of chromosome 7 or 8, no BCR-abl 4. chronic eczema like facial rash, suppurative lymphadenopathy, petechiae/purpura, leukocytosis <100k, anemia, thrombocytopenia

10% of uncircumcised males ultimately require circumcision for any of the following conditions:

1. phimosis 2. paraphimosis 3. balinitis

toxicara canis cati 1. pathogenesis + clinical presentation 2. tx

1. pica children, ingestion of eggs --> generalized visceral larval migrans ~eosinophilia, leukocytosis, hepatomegaly, malaise, anemia, cough, myocarditis, OCULAR LARVA MIGRANS (retinal granulomas/endophthalmitis) 2. albendazole, mebendazole, steroids for ocular involvement

tinea versicolor 1. pathogenesis + clinical presentation 2. tx

1. pityosporum orbiculare yeast --> superficial fungal infection--> scaly oval macules, which may be hypo/hyperpigmented, and become more prominent with sun exposure 2. 2.5% selenium sulfide, ketoconazole shampoo, systemic antifungals

seborrheic dermatitis 1. pathogenesis + clinical presentation 2. dx/tx

1. pityosporum ovale/unknown --> eruption of greasy red scales/crusts in areas with high sebaceous glands (scalp/face/brows/nose), cradle cap on infants, nasolabial folds, pinna, scalp, diaper areas 2. low dose topical steroids, sulfur, zinc or salicylic acid shampoos, topical antifungals

malaria 1. pathogenesis 2. dx/tx

1. plasmodium falciparum(also vivax, malariae, ovale) ~ bite from anophales mosquito --> flu like prodrome, chills, fever, vomiting, headache, abdominal pain every 48-72 hours as a result of RBC rupture and parasitemia ~ hemolytic anemia, splenomegaly, jaundic, hypoglycemia, cerebral malaria, renal failure/shock 2. giemsa stained peripheral blood- to identify plasmodium tx = chloroquine, quinine, quinidine gluconate, mefloquine, doxycycline

sinusitis 1. pathogens 2. treatment

1. pnuemo, h.flu, moraxella, ~ except chronic which may be s.aureus/anaerobes *** OFTEN SECONDARY TO PRECEDING VIRAL URI****** 2. amoxicillin, or amoxicillin/clavuloanate, or second gen cephalosporin ~~ except chronic which is broad spectrum oral, CT image, and IV abx

contact dermatitis 1. pathogenesis + clinical presentation 2. tx

1. poison ivy/nickel jewlery, lotions/cream, perfumes --> T cell mediated -->erythematous papules/vesicles @ area of contact 2. topical corticosterods/avoidance of allergen

c. difficle 1. clinical pres 2. dx/tx

1. post Abx use --> pseudomembranous colitis 2. toxin in the stool + endoscopy shows pseudomembranes tx = metranididazole or vancomycin

MAS 1. pathogenesis 2. clinical presentation of MAS

1. post term baby/distress/hypoxia⇒ meconium stained amniotic fluid ⇒ aspiration of meconium into the airways of the fetus⇒distal airways and alveoli 2. mild or moderate respiratory distress, may progress to hypoxemia/cyanosis

Campylobacter jejuni 1. clinical pres 2. dx/tx

1. poultry exosure --> bloody diarrhea 2. stool WBCs present + stool culture tx = erytrhomycin

breast tanner stages

1. preadolescent 2. elevation of breast/nipple as small projection 3. enlargement of breast 4. areaola and nipple form secondary mound above breast level 5. only nipple projects, areola recedes, adult breast size

Male tanner staging

1. preadolescent, no pubic hair + pre-pubertal testes 2. testes larger, LONG downy hair 3. testes larger darker, coarser curlier hair 4. glans develops, scrotal skin darkens, coarse curly hair over pubis symphysis 5. adult size/shape, pubic hair spreads to medial surface of thigh

female genetalia tanner stages

1. preadolescnet 2. sparse long downy hair along labia 3. darker coarser curlier 4. coarse and curly adult type hair covering symphysis pubis 5. adult type hair spread onto medial surface of thighs

1. migraine aura 2. migraine equivalent 3. ophthalmologic migraine 4. basilar artery migraine

1. preceeding change of vision, or unilateral paresthesias or weakness 2. no actual headache, but alteration of behavior, cyclic vomitting, or paroxysmal vertigo 3. unilateral ptosis or CN3 palsy + migraine 4. vertigo, tinnitus, ataxia, dysarthria precedes migraine

1. define alcoholism 2. how to screen for alcoholism

1. preoccupation with over drinking, despite adverse consequences 2. CAGE questionnaire: •have you tried to cut down •have people annoyed you by criticizing your drinking •do you feel guilty •have you ever drank first thing in the morning (eye opener)

retinopahty of prematurity 1. pathogenesis + clinical presentation 2. tx

1. preterm, hyaline membrane disease, intracranial hemmorrhage--> proliferation of retinal blood vessels exposed to oxygen --> myopia, astigmatism ,amblyopia, strabismus 2. minimize O2 delivery, treat hyaline membrane disease

secondary adrenal insufficinecy 1. define 2. electrolytes """ 3. causes

1. problem at the level of the hypothalmus or pituitary ( no CRH or ACTH) 2. serum potassium NORMAL ~ BECAUSE ALDOSTERONE AXIS IS INDEPENDENT OF HPG AXIS 3. craniopharyngioma, histiocytosis, IATROGENIC by glucocoticoid use*************

Indirect inguinal hernia 1. pathogenesis 2. clinical presentation 3. treatment

1. processus vaginalis fails to obliterate-->bowel extends through internal inguinal ring- 2. painless inguinal swelling + bowel sounds on auscuiltation of the scrotum 3. surgical repair, to prevent bowel incarceration

Hypoglycemia 1. labs 2. why do infants of diabetic moms have hypoglycemia? 3. nesidioblastosis

1. serum glucose <40 mg/dL 2. inc. glucose, inc. insulin secretion⇒ transient hypoglycemia 3. islet cell hyperplasia ⇒ persistent hypoglycemia

long QT syndrome 1. what is the major risk factor with this disease 2. causes 3. drug causes 4. clinical presentation

1. progression to torsade de pointes 2. 50% AR Jervel lange nielsen* syndrome associated with congential deafness, or Romano Ward* not associated with congenital deafness, idipathic 3. phenothyazines, tricyclics, erythromycin, terfenadine 4. syncope, seizure, palpitations, sudden cardiac arrest

biliary atresia -etiology unknown 1. pathogenesis + clinical presentation 2. associated syndrome 3. dx/tx

1. progressive fibrosclerotic disease of the extrahepatic biliary tree --> jaundice, dark urine, pale/acholic stools, hepatosplenomegaly, ascites, peripheral edema, coagulopathy --> obliteration of the bile duct/cirrhosis by 4 months of age (EASY TO CONFUSE WITH PHYSIOLOGIC JAUNDICE) 2. polysplenia syndrome - bilobed lungs, abdominal heterotaxia, situs ambiguous 3. cholangiogram with laparotomy tx = kasai portoenterostomy establishes bile flow most successful if child <50-70 days of age, cholangitis is worst complication (dec. all bile flow), liver transplant supportive care- ADEK, TPN, ursodeoxycholic acid

dermatomyositis ages 5-14 (avg. age 6), F>M 1. clinical presentation 2. dx 3. tx

1. progressive muscle weakness + constitutional, periorbital violaceous heliotrope rash (sun exposed), gottrons papules, proximal muscle weakness (gowers), neck flexor weakness, calcinosis (in muscle,, nail telangiectasias, 2. clinical pres + EMG abnormal, muscle biopsy abnormal, inc. CK/AST/ALT/LDH/aldolase 3. corticosteroids, MTX, cyclophosphamide, cyclosporine, vitamin D/calcium supplementation

heart block 1. fist degree 2. second degree 3. third degree 4. causes of heart block

1. prolonged PR interval 2. type 1 = wenckeback- progressive prolonged PR ==> dropped beats type 2 = failed AV conduction without progressive PR prolongation 3. complete block -- no conduction between the atria and ventricles 4. SLE mother, post surgical AV block (especially after closing VSD), bacterial endocaridits

1. what hemostatic conditions cause hypercoagulability 2. what disease states cause hypercoagulability

1. protein S/C deficiency, antithrombin C deficiency, factor V leiden 2. sickle cell, malignancy, inflammatory disease, liver disease, nephrotic syndrome, ulcerative colitis, dehydration, vascultiis, diabetes, homocystinuria

TAPVR 1. physical examination 2. ECG 3. CXR 4. pathophysiology 5. tx

1. pulmonary ejection murmur (inreased pulmonary flow) along left sternal border 2. RVH/RAE, 3. enlarged heart with supracardiac drainage, snowman appearance, if there is obstruction small heart, and pulmonary edema 4. systemic and pumlmonary blood mixes in the right atrium becuase pulmonary veins drain into the systemic circulation 5. pulmonary veins surgically put onto the left atrium

TAPVR 1. patho + clinincal pres

1. pulmonary veins drain to the right atrium, then some oxygenated blood makes it through to the foramen ovale/PDA to the body --> CYANOSIS in infant + DIFFUSE RETICULAR PATTERN OF LUNG FIELDS on radiograph

DTaP 1. components 2. timing 3. DTP? 4. dT?

1. purified acellullar pertussis, tetanus toxoid, diptheria toxoid 2. 2,4,6 months + boosters 12-18 months, and 4-6 years of age 3. old vaccine (DTP) - contained whole cell killed pertussis ~ replaced with DTaP b/c it had high rate of side effects 4. 1/10 dose diptheria + tetanus toxoid given as booster --given every 10 years to those greater than 7 yo

Malrotation/midgut volvulous 1. diagnosis 2. treatment

1. radiograph shows proxial intestinal distension/obstruction, upper intestinal contrast imaging <--gold standard, shows abnormal ligament of treitz, and duodenal obstruction and jejunum to the right of midline 2. resection of nonviable intestine, fluid resusitation, antibiotics

poisoning 1. what is whole bowel irrigation

1. rapid complete emptying of the GI tract using polyethylene glycol + electrolyte solution

Hepatits B vaccine 1. components 2. timing of administration

1. recombinant HbSAg 2. three shot series given in first year of life

Bulimia nervosa 1. diagnostic criteria 2. physical exam findings

1. recurrent binging, >2x a week for 3 months, purging using vomiting, laxatives, diuretics, enemas, rigorous fasting exercise or diet pills, disturbed body image 2. NORMAL WEIGHT, hypothermia, hypotension, bradycardia, sequelae of vomiting, trauma to palate/hands, loss of dental enamel, parotid swelling, swollen uvula. 3. LOW CHLORIDE, LOW POTASSIUM (vomiting or diuretics or laxatives)

vascular (renal failure) 1. pathogenesis 2. lab findings

1. renal artery embolus (umbilical artery catheter), renal vein thrombosis --> dec. kidney perfusion 2. dec. renal blood flow on nuclear scan

1. what does persistent proteinuria indicate 2. what kind of protein in glomerular proteinuria + causes 3. what kind of protein in tubular proteinuria + causes

1. renal diseaes 2. large molecular weight proteins (ALBUMIN**), seen in glomerulonephritis/nephrotic syndrome 3. small molecular weight proteins (BETA 2 MICROLOBULIN**) (from decreased reabsorbtion by damaged tubular epithelium) interstitial nephritis, ischemic renal injury (ATN), nephrotoxic drugs

urine findings 1. crystals

1. renal stone disease

immunotherapy for allergy

1. repeated exposure to antigens leads to better tolerance ~ use when other therapies are ineffective, environmental controls have failed

IgA nephropathy (bergers disease) 1. pathogenesis + clinical presentation 2. biopsy and lab diagnosis 3. treatment 20-40% go to end stage renal disease

1. respiratory infection--> recurrent hematuria 2. mesangial proliferation, increased mesangial matrix, high serum IgA 3. supportive, ACE inhibitors (for htn), steroids, immunosuppressants

varicella 1. pathogenesis + clinical presentation 2. tx

1. respiratory transmission --> 3 week incubation--> macules w/ central vesicles within 1-2 days (dew drops on a rose petal) CROPS of different age lesiosn + fever 2. antipyretics with antibacterial soaps, antihistamines, acyclovir for varicella pneumonia/encphalitis

cholestatic jaundice 1. definition

1. retention of bile within the liver, direct bilirubin >2 or 15% of total bilirubin

supraventricular tachycardia 1. AV re-entrant tachycardia 2. AV node* re-entrant tachycardia 3. wolff parkinson white syndrome

1. retrograde conduction through accessory pathway leads to SVT 2. circuit moment in the AV node 3. bypass tract through the atria and ventricles --> sudden death

sydenham chorea (St. Vitus dance) -25% of people with rheumatic fever 1. pathogenesis + clinical presentation 2. dx / tx - good prognosis, lasts from several months to 2 years

1. rheumatic fever --> autoimmune antibodies cross react with group A strep and BASAL GANGLIA CELLS --> chorea (face/hands), jerky speech, chameleon tongue, milkmaids grip, no change in gait/cognition 2. ASO/anti DNase B titers, increased T2 signal in the caudate/putamen, SPECT shows inc. perfusion of thalamus/striatum tx = haloperidol, valproic acid, phenobarbital

1. causes of viral URI 2. clinical presentation of viral URI 3. persistnet fever/symptoms >10 days indicates what 4. tx

1. rhinovirus, parainfluenza, corona, RSV 2.low grade fever,rhinorrhea, cough, sore throat lasts 7-10 days 3. bacterial superinfections 4. adequate hydration, mucolytic, cough suppressant, decongestants, NO ANTIBIOTICS

vitamin K deficiency 1. pathogenesis 2. clinical presentation 3. dx/tx

1. rifampin/isoniazid/warfarin/cephalosporins infant deficiency, pancreatic insuff, biliary obstruction --> cannot synth 2/7/19/10 protein C/S 2. bruising, oozing from punctures, bleeding into organs 3. prolonged PTT and PT, normal bleeding time (no problem with platelets)

Gastroschisis -Tx

1. right paraumbilical fissure in anterior abdominal wall, NOT TRUE HERNIA, risk of bowel damage/ischemia Tx = sterile wrapping to reduce insensible water loss

what type of injuries with 1. swimmers 2. football 3. basketball/volleyball 4. running 5. ballet 6. wrestlers 7. skiing

1. rotator cuff 2. knee and foot, head/neck 3. osgood schlatter, ankle 4. patellofemoral, muscle strains 5. delayed menarche/eating disorders 6. shoulder subluxation, pre-patellar bursisits, skin conditions 7. ulnar collateral ligament

blepharitis 1. pathogenesis + clinical presentation 2. tx

1. s. aureus --> eyelid inflammation and red eye, crusting, scaling of the eyelash base, eyelids stick together in the monring 2. eyelid hygene with baby shampoo, topical erythromycin

osteomyelitis 1. pathogenesis/clinical pres 2. dx/tx

1. s. aureus/s.pyogenes/salmonella (sickle cell)/ pseudomonas (nail in foot thorugh shoe), hematogenous (MCC)--> fever, bone pain, erythema, swelling, induration 2. elevated WBC/CRP/ESR, MRI or bone scan, NOT RADIOGRAPH(will be normal early in infection) tx = 6wks abx, monitor ESR (dec. = good response), surgery for abscess if fever/swelling persist

Fever in 3-36 month child 1. sepsis/meningitis bacterial etiologies 3-36 months What to do when: A. child appears toxic B. nontoxic appearaing and temp <102.2 C. nontoxic appearing and temp >39 (102.2)

1. s. pneumo, h.flu type B, neisseria A. hospitalization with complete sepsis evaluation B. no labs required, observe closely at home C. urine culture for males <6mo and females <2mo blood culture if WBCs>15k chest radiograph if respiratory distress, rales, or tachypnea stool culture if blood and mucus in teh stool or if >5WBCs empiric antibiotics for children whose WBCs>15k

cervical lymphadenitis 1. causes 2. reactive lymphadenitis 3. clinical presentation 4. tx

1. s.aureus (MCC UNILATERAL), s.pyogenes, MAC, m.tuburculosis, B.henselaek, EBV (BILATERAL), CMV, HIV, kawasaki disease, t.gondii, branchial cleft cyst, cystic hygroma 2. response to infections of the pharynx, teeth, soft tissues of head/neck 3. mobile, tender, with erythematous skin over the node, single or multiple nodes 4. cephalosporin or anti-staphylococcal penicillin (Dicloxacillin or cephalexin or clindamycin) + incision/drainage

staph scalded skin syndrome 1. pathogenesis 2. clinical presentation

1. s.aureus exfoliative toxin 2. fever, tender skin, bullae, sloughing of sheets of skin, Nikolsky sign (extension of bullae when pressure applied to the skin), tends to be in flexor surfaces

septic arthritis 1. pathogenesis/clinical presentation 2. which joint most commonly effected in younger and older children

1. s.aureus/s.pyogenes, n.gonorrhoeae hematogenous spread, contiguous spread, direct inoculoation--> erythema, swelling, asymmetry of soft tissue, pain with movment, hip held in flexion/abduction/external rotation to eleviate the pain 2. hip in young, knee in older

strongyloides stercoralis 1. pathogenesis + clinical presentation 2. tx

1. same lifecylce as hookworm --> transient pruritic papules at site of penetration--> loefflers pneumonitis, GI symptoms, eosinophilia 2. ivermectin, thiabetdazole, albendazole

scabies 1. pathogenesis + clinical presentation 2. tx

1. sarcoptes scabiei --> papules or veseicles in the abdomen, dorsum of hands, groin, axilla, flexor surfaces of wrist/interdigital spaces + SEVERE ITCHING + S shaped BURROWS 2. DX = examination shows mite eggs or mite feces TX = overnight permethrin lotion, or 1% lindane

intussucception 1. age of onset + clinical findings 2. tx

1. second half of first year of life, teloscoping of ileum into the colon --> fever, palpable sausage shaped mass, + CURRANT JELLY STOOL 2. barium enema

Head trauma 1. what kind of response is common after head trauma 2. why are infants more tolerable of intracranial masses/hemorrhage

1. seizure 2. they have open fontanells and cranial sutures which allows blood to accumulate in sub galeal (between the scalp and galea aponeurosa) and epidural spaces

renal parenchymal acute interstitial nephritis (renal failure) 1. pathogenesis 2. lab findings

1. semisynthetic penicillins --> damage to the interstitium 2. eosinophilia, increased urinary B2 MICROGLOBULIN,

1. how is acute life threatening causes of hypotonia ruled out 2. how is central hypotonia evaluated 3. how is peripheral hypotonia evaluated

1. sepsis work up, liver enzymes, CBC, inflammatory markers, electrolyte panel (ex. urea cycle diseases) + others 2. CT head scan, serum electrolytes (Ca/Mg/ammonia/lactate/pyruvate), chromosome stuidies (ex. prader willi) 3. serum creatine kinase, DNA tests, EMG + nerve conduction, muscle biopsy

causes of cholestatic jaundice(increase conjugated bilirubin) 1. infections - 2. metabolic derrangements 3. extrahepatic mechanical obstruction 4. intrahepatic mechanical obstruction - idiopathic, neonatal hepatits - alpha 1 antitrypisn deficinecy - TPN associated 5. what are the clinical features of cholestasis

1. sepsis, hepatitis, viral infections 2. cystic fibrosis, hypothyroidism 3. biliary atresia, bile duct stricture 4. paucity of intrahepatic bile duct, alagille syndrome 5. jaundice, acholic(light color) stools, dark urine, hepatomegaly, bleeding (dec. factors), FTT

What labs for adolescent screening (sex related)? 1. females 2. males

1. sexually active females •Cervical culture for gonorrhoea •Immunofluorescent Ab test or culture of cervical fluid, or urine ligase test for chlamydia •Serological test for syphilis •Pap smear for cervical cancer screening & HPV •Vaginal wet mount for trichomonas vaginalis 2. Sexually active males •annual syphilis serology, urinalysis for pyuria, urine ligase for c.trachomatis

Non - live vaccines 1. duration of immunity 2. contraindications 3. list of non-live vaccines

1. short (need boosters) 2. none 3. DTap (and Tdap), hepatitis A/B, inactivated polio, HIB, influenza, pneumococcal, meningococcal

Disproportionate short stature

1. short staure in patients who have short legs with an increased U/L ratio 2. consider skeletal dysplasias, rickets

cholecystitis *uncommon in children 1. what children have a higher risk for acute cholecystitis 2. pathogenesis 3. causes of acalculous cholecystitis

1. sickle cell disease, cystic fibrosis, TPN therapy 2. obstruction of cystic duct--> inc. pressure/secretio of enzymes/prostaglandings --> transmural inflammation/infection /necrosis/perforation 3. salmonella, shigella, e.coli infection, or after trauma, burns, vasculitis

tricusipid atresia 1. physical examination (w and w/o VSD) 2. ECG 3. CXR 4.mechanism 5. when is tricuspid atresia PDA and not PDA dependent

1. single S2 (only one valve) if no VSD, systolic murmur of VSD if present, NO murmur if VSD is not present 2. LAD***, LVH***, RAE, 3. small heart, decreased pulmonary vascular markings 4. plate of tissue where the tricuspid valve should be with an obligate ASD or PFO~~ blood flows from the right atrium to the left atrium to left ventricle to aorta/pulmonary artery through VSD 5. if VSD is not present, this is a PDA dependent lesion, if VSD is present this lesion does not require PDA

truncus arteriosus 1. physical examination 2. ECG 3. CXR 4. tx

1. single S2, systolic ejection murmor @ left sternal border (inc. flow across truncal valve), diastolic murmor at the apex (from excess flow across mitral valve) 2. combined ventricular hypertrophy 3. enlarged heart, increased pulmonary vascular markings, right aortic arch 4. surgical repair

selective IgA deficiency -most common immune deficiency 1.clinical presentation 2. dx/tx

1. sinusitis, pneumonia, otitis, bronchitis, chronic diarrhea/giardia infection, SLE, juvenille arthritis, celiac disease, atopic diseases ***anaphylaxis from blood products 2. serum immunoglobulin, manage infections/complications, NO IVIG

cellulitis 1. pathogenesis 2. clinical presentation

1. skin infection in the dermis caused by group A strep and S.aureus 2. erythema,warmth, tenderness, indistinct border (compare to erysipelas) 3. first gen cephalosproins, anti-staph penicillins

1. diagnosis of food allergy 2. treatment

1. skin tests, radio allergosorbant tests identify serum IgE to specific food allergens, provocative food challenge 2. avoidance, and injectable epinephrine

1. how to treat mammalian bites 2. how to treat cat, human, monkey bites 3. what bacteria in human bites + tx

1. soap and water (only 4% get infected) 2. abx prophylaxes (35-50%) 3. viridans strep, s.aureus, bacteroides, amoxicillin clavulanate, erythromycin

osteomyelitis complications

1. spread to distant joint or pneumonia chronic osteomyelitis due to a *sequestrum (necrotic focus) or *involucrum (new bone/fibrosis around infected bone)

Bacterial tracheitis 1. pathogenesis + clinical pres 2. Management

1. staph aureus (MCC), streptococcus, non typeable H. influenzae --> ACUTE inflammmation of the trachea--> toxicity, high fever, mucous and pus in the trachea 2. Antistaphylococcal antibiotics and airway support

Cystic fibrosis 1. sinopulmonary disease (infectious agents+clinical presentation) 2. GI disease 3. nutritional deficiencies 4. metabolic abnormalities 5, reproductive abnormalities

1. staph aureus, pseudomonas, burkholderia cepacia ~~ chronic cough/wheeze/sputum, nasal polys/sinusitis, bronchiectasis, hemoptysis pulmoary infiltrates, hyperinfaltion 2. meconium ileus, pancreatic insufficinecy/pancreatitis, chronic hepatits, rectal prolapse 3. hypoproteinemia, edema, ADEK deficinecies 4. hyponatremia, hypochloremia, hypokalemic metaboic alkalosis 5. obstructive azoospermia `

1. what secondary infections associated with dog bites 2. tx

1. staph, pasturella, strep 2. irrigation, suturing for wounds <12 hours old on body, <24 hours old for the face amoxicillin + clavulonic acid , tetanus prophylaxis

post renal failure (renal failure) 1. pathogenesis 2. lab findings

1. stones/tumor/ureterocoele/urethral tumor, neurogenic bladder, posterior urethral valves-->obstruction of urine flow 2. dilation of renal collecting system

amblyopia 1. pathogenesis + clinical presentation 2. dx/tx *earlier onset = worse vision loss

1. strabismus, opacification of the lens, severe refractive error, anisometropia, viterious hemorrhage --> abnormal visual development 2. dx = abnormal bruckner and hirshberg test tx = correct refractive errors, patch the normal eye ASAP

endocarditis 1. underlying conditions 2. infectious organisms 3 pathogensis

1. structural abnormalities, and post surgery (50%) 2. alpha hemolytic strep viridans, and staphylococcus are most common bacterial ~~~ also gram negatives (rare) and fungal(rare) 3. bacteria introduced into the blood and infect cardiac endothelium --> fibrin/platelets adhere forming a vegetation --> DISTAL MANIFESTATIONS

hemorrhagic conjunctivitis 1. pathogenesis + clinical presentation

1. subconjunctival hemorrhage cuased by h.flu, adeno, picronaviruses

primary irritant contact dermatitis 1. pathogenesis + clinical presentation 2. tx

1. substances/feces/urine/diaper --> inflammatory dermatitis-->erythema, papules w/o inguinal crease involvement--> secondary infection with candida ~~ classic diaper dermatitis 2. barrier creams (ZnO), keep skin clean, skin moisturzers, nystatin/clotrimazole

Testicular torsion 1. clinical presentation 2. dx 3. tx

1. sudden onset scrotal, inguinal pain, swollen testicle, ABSENT CREMASTERIC reflex on the affected side, pain relief on elevation 2. hx/pe, decreased uptake of technetium 99m pertechnetate radionucleotide scan or doppler ultrasound (shows absent pulsations) 3. surgical detorsion WITHIN 6 HOURS

acquired aplastic anemia 1. pathogenesis + clinical pres 2. dx 3. tx

1. sulfonammides,anticonvulsants, HIV/EBV/CMV, chemicals, radiation, idiopathic--> brusing, petechiae, pallor, neutropenia/infection 2. pancytopenia, low retics, hypocellular marrow 3. stop causative agent, BMT, immunosuppressants

impetigo 1. pathogenesis 2. clinical presentation 3. tx

1. superficial skin infection with s.aureus or s.pyogenes 2. honey colored crusted or bullous lesions on the face/nares VERY CONTAGIOUS 3.mupirocin (topical) or oral dicloxacillin, cephalexin, clindamycin

treatment of hepatits B

1. supportive for acute interferon alpha and antivirals for chronic

near drowning 1. definition 2. what classic causes

1. survival after asphyxia while submerged in a liquid 2. child falls in pool or water containers (toilet/bucket), adolescents assocated with drugs/alcohol ingestion

amniotocele (dacrocele) 1. pathogenesis + clinical presentation 2. tx

1. swelling of nasolacrimal sac --> bluish swelling in medial canthal area/warmth/erythema/tenderness 2. local massage, IV abx, NLD probing

1. moro reflex 2. appears/dissapears

1. symmetric abduction of arms with trunk extension followed by adduction of upper extremities 2. birth/4 months

1. three types of juvenile idiopathic arthritis 2. what does ANA positivity increase risk for in childe with juvenille idiopathic arthritis

1. systemic, pauciarticular, polyarticular 2. uveitis

ASD 1. physical exam findings 2. ECG fidnings 3. CXR

1. systolic ejection murmor, mid-left sternal border and ULSB, fixed split S2, diastolic rumble, 2. RAD, RVH, RAE 3. right atrial/ventricular enlargment, inc. pulmnoary vasculature markings

tetralogy of fallot 1. physical examination 2. ECG 3. CXR 4. 4 anatomic components + 5. mechanism of blood flow 6. tx

1. systolic ejection murmur of pulmonary stenosis*, increased right ventricular impulse(RVH) 2. RVH 3. boot shaped heart, decreaed pulmonary vascular markings, right aortic arch 4. VSD, overriding aorta, pulmonary stenosis, RVH~~~ 5. Mechanism: pulmonary outflow track obstruction causes a right to left shunting of blood across the VSD 6. squatting position, IV fluid bolus, oxygen, sedation (dec. agitation), beta blocker (dec. contractility / facilitate pulmonary flow sodium HCO3- (tx. acidemia) transfusion

1. Subdural hematoma 2. dx 3. tx

1. tearing of the BRIDGING MENINGEAL VEINS --> blood beneath the dura, more common than epidural bleed 2. head CT shows CRESCENT shaped density in subdural space that crosses suture lines 3. surgical drainage

1. why are children more prone to chest trauma 2. tension pneumothorax clinical presentation 3. tx for tension pneumothorax

1. their chest is soft and pliable 2. decreased breath sounds, hyperresonance to percussion, displaced trachea, distended neck veins 3. rapid chest decompression by needle thoracotomy

1. scars 2. ulcer 3. atrophy 4. fissure

1. thickened fibrotic dermis 2. absense of epidermis + some of the dermis 3. thinning of epidermis/dermis 4. linear cracks in the dermis

scarlet fever 1. dx/tx 2. complications***

1. throat culture or rapid strep test tx = penicillin, IM penicillin, or macrolides 2. rheumatic fever, post strep glomeruloneprhitis, post streptococcal arthritis, pediatric autoimmune neuropsychiatric disorders associated iwth streptococcal infection (OCD, tic disorders after strep infection)

thrombocytopenia-absent radius syndrome (TAR) 1. pathogenesis / clinical presentation

1. thrombocytopenia (improves by age 2-3), limb abnormalities (no radius), there is thumb present (compare to fanconi), cardiac and renal disease

ehrlichia chaffeensis 1. pathogenesis + clinical presentation 2. tx

1. tick bite --> same symptoms as rickettsia but no rash - fever, headache, myalgias, lymphadenopathy 2. dx = serology and PCR, tx = doxycycline and supportive

neonatal jaundice 1. what kind of jaundice do 50% of neonates have 2. what is the progression of neonatal jaundice 3. does degree of jaundice indicate the level of bilirubin

1. transient unconjugated hyperbilirubinemia-- due to decreased function of UDP glucuronyl 2. cranial to caudal in an otherwise healthy appearing infant 3. NO MUST STILL MEASURE DIRECT/INDIRECT LEVELS OF BILIRUBIN

functional fecal retention (constipation) MCC constipation in childhood 1. pathogenesis 2. clinical presentation

1. traumatic events (hard stool, painful diarrhea, diaper rash, abuse) --> retained stool --> increaesd hardness/mass--> constipation + complications 2. anorectal distension, encopresis, fecal halitosis, abdominal pain/distension, pelvic complications, psychosocial problems

tinea capitis 1. pathogenesis + clinical presentation 2. dx/tx

1. trichophyton tonsurans(from humans)/microsporum canis (from dogs) --> patchy hair loss with hairs that break + scales/pustules + kerion (large red boggy nodule hypersendivity reaction) + occipital/pos. cervical lymphoadenopathy 2. dx = KOH identifies fungae, woods light for m. canis tx = systemic griseofulvin, topical antifungals NOT EFFECTIVE

1. what is a tet spell 2. how to compensate during tet spell

1. trigger* such as crying (inc. RVOT resistance) --> dec. oxygenation of blood --> sudden cyanosis and decreased murmur intensity --> altered consciousness + hyperpnea 2. squatting in the knee/chest position to increase venous return to the heart and increase SVR

infantile spasms (west syndrome) 1. pathogeneis + clinical presentation/age 2. EEG findings 3. tx -poor prognosis, children develop intellectual disability

1. tuberous sclerosis***/PKU/hypoxia ischemia/intraventricular hemorrhage, meningitis, encephaltis 3-8 months of age --> brief myoclonic jerks consisting of sudden arm extensions or head/trunk flexion (jackknife or salaam seizures), clusters of 5-10 seizures over 3-5 minutes 2. hypsarrythmia pattern, with disorganized pattern of high amplitude spikes 3. ACTH***, valproic acid, vigabatrin

1. rooting reflex 2. appears/dissapears

1. turning head toward same side as stimulus on corner of the mouth 2. birth/six months

What should be seen on examination fo the umbilicus? What does only one umbilical artery suggest?

1. two arteries, one vein, NO URACHUS 2. Congenital RENAL ANOMALIES

lower GI bleeding 1.necrotizing enterocolitis 2.juvenille polyps 3. allergic colitis 4. infectious enterocolitis - meckels diverticulum -hemolytic uremic syndrome -henoch schonlein purpura -inflammatory bowel disease

1. typically in newborns - condsider in any newborn with rectal bleeding, feeding intolerance, or abdominal distension 2. MCC lower GI bleeding, painless, intermittent, streaky 3. sensitization to protein antigens in cows milk, soy milk, or breast milk 4. salmonella, shigella, campylobacter, yersinia, E.coli

1. coma 2. MCC coma <5 years old 3. MCC coma older children

1. unawareness of self/environment, unarrousable, eyes closed 2. nonaccidental trauma + near drowning 3. drug overdose, accidental head injury

HUS 1. pathogenesis + clinical pres 2. treatment 3. poor prognostic signs

1. uncooked beef, unpasurized milk, contaminated fruit juice --> vascular endothelial damage from shiga toxin (EHEC/shigella) --> diarrhea --> MAHA, thrombocytopenia (from vascular damage and thrombus formation), acute renal failure 2. NO ANTIBIOTICS - treatment of ecoli hemorrhagic colitis leads to an increase that the patient will develop HUS 3. high WBC count, prolonged oliguria, TOXIC MEGACOLON (rare)

1. apnea of infancy 2. short central apnea 3. two general causes of apnea 4. causes of obstructive apnea

1. unexplained cessation of breathing >20sec 2. <15seconds of apnea normal at all ages 3. central (no respiratory effort), or obstructive where there is respiratory effort 4. craniofacial abnromalities

1. SIDS 2. risk factors + age of SIDS

1. unexplained death in child <1 year of age 2. age 2-4 months ---> prone sleeping, soft bedding, over bundling, prematurity, low birth weight, recent illness, no breastfeeding, maternal smoking/drug abuse, infection

what are the top three causes of adolescent death

1. unintentional injuries 2. homicide 3. suicide

ALL - MCC childhood cancer, peak 2-6 years of age - long term survival 85% of patients** 1. pathogenesis 2. cell morphology/types 3. Pre-B cell subtyping

1. unknown/down syndrome, bloom syndrome/chemical agents/immunodeficiency --> lymphoblast proliferation 2. Pre B cell (80%) >T cell>B cell L1/L2/L3 ~ L1 = small cells with little cytoplasm, L3 = large cells > 1 nucleoli 3. common acute lymphocytic leukemia antigen (70% + or -)

tourettes syndrome 1. pathogenesis 2. tx

1. unknown/genetic --> motor/phonic tics(sterotypical behaviors), coprolalia, learning disabilities, ADHD, obsessive-compulsion 2. pimozide**, clonidine (se=sedation), haloperidol, hypnotherapy

UTI 1. frequency based on gender 2. pathogenesis 3. clinical presentation

1. up to 6 months of age >boys, after 6 months of age more frequent in girls 2. ECOLI, klebsiella, pseudomonas, s.saprophyticus, serratia, proteus (high pH urine), enterococcus -->ascend in the urethra 3. neonates/infants present with fever, lethargy, irritability, jaundice children present with noctural enuresis, or diurnal enuresis, low grade fever, dysuria, freq/urge

complications of GERD 1. upper/lower airway 2. gastrointestinal

1. upper and lower airway disease exacerbation bronchopulmonary constriction aspiration chronic laryngitis, hoarseness, wheezing, vocal cord nodules, subglottic stenosis 2. barretts esophagus(stratified squamous --> columnar-->esophageal adenocarcinoma

GERD 1. treatment

1. upright positioning during sleep and after feedings, thicken feeds H2 blockers, PPIs, metoclopromide (MOTILITY AGENT) surgery - nissen fundoplication - wrap the fundus of the stomach around the esophagus leading to decreased transient lower esophageal sphincter relaxation + gastrostomy to assist with the adaptation to the smaller gastric size after fundoplication

hyperphosphatemic hypocalcemia 1. pathogenesis

1. uremia, renal failure, excess phosphate intake--> elevated phosphate/calcium binding and deposition

vesicoureteral reflux 1. pathogenesis 2. dx/tx

1. ureterovesicular junction abnormalities (short submucosal tunnel)--> reflux of urine into the ureters and collecting system--> UTIs (30-50% of UTIs in infants) --> pyelonephritis/RENAL SCARRING**** --> reflux nephropathy (segmental scars/contraction/interstitial nephritits) 2. voiding cystourethrogram (VCUG) tx = low dose abx,

Epispadias + its associated clinical findings

1. urethral meatus located on dorsal surface of the penis 2. bladder extrophy - bladder protrusion from the abdominal wall with exposure of the mucosa

Hypospadias -is it associated w/inc UTI?

1. urethral meatus located on ventral surface of penis 2. no not associated with increased with inc. UTI

Maple syrup urine disease 1. clinical pres 2. dx/tx

1. urine has maple syrup odor, hypoglycemia, severe acidosis, lethargy, hypotonia, coma 2. inc. serum/urine BCAA~~

Pertussis 1. pathogenesis + clinical presentation 2.dx -tx

1. usually UNVACCINATED --> bordetella pertussis exposre --> 10 day incubation--> CATARRHAL STAGE (2 wks ) URI, rhinorrhea, nasal congestion, low grade fever --> PAROXYSMAL STAGE (4 wks) cough + whoop+ post tussive emesis, cyanosis, apndea, choking--> CONVALESCENT PHASE recovery phase and cough becomes less frequent 2. clinical features + CULTURE ON REGAN LOWE OR BORDET GENGOU MEDIUM, LYMPHOCYTOSI, fluroescent antibody tests tx = azithro/erythro, respiratory isolatoin

sacrococcygel teratomas 1. clinical presentation + tx

1. usually in females, arises form the coccyx and forms soft tissue mass, benign and can be excised

congenital torticollis 1. pathogenesis + clinical pres 2. tx

1. uterine constraint or birth trauma --> head tilts toward affected side, asymmetry of the head/ears if intreated **may feel soft tissue mass in SCM = bleeding into muscle 2.stretching exercises, helmet therapy (misshapen head), radiograph to find any cervical spine abnormalities

extra intestinal manifestations 1. ulcerative colitis 2. crohns disease

1. uveitis, arthropathy, pyodermagangrenosum, sclerosing cholangitis 2. FTT, delayed sexual development, oral aphthous ulcers, erythema nodosum, arthritis, renal stones, strictures, fistulas, abscesses

neonatal conjunctivitis 1. pathogenesis + clinical presentation 2. dx/tx

1. vagianl canal transmits neisseria gonorrhoeae, chlamydia trachomatis and HSV, 1% silver nitrate (for gonorrhoea)--> red watery eyes

neisserira gonorrhoeae conjunctivitis (neonatal) 1. pathogenesis + clinical presentation 2. dx/tx

1. vaginal canal --> 2-4 days purulent dishcharge/eyelid welling/corneal ulcer 2. dx: gram negative diplococci tx: IV cefotaxime, topical erythromycin

chlamydia trachomatous conjunctivitis (neonatal) 1. pathogenesis + clinical presentation 2. dx/tx

1. vaginal canal -->4-10days of life -->serous or purulent discharge +/- lid swelling 2. cytoplasmic influsion bodies, positive culture oral erythromycin

treatment of: 1. generalized epilepsy 2. absence epilepsy 3. partial epilepsy 4. medically intractable epilepsy

1. valproate or phenobarbital or phenytoin 2. ethosuximide or valproate 3. carbamazepine or phenytoin 4. surgery to remove epileptic tissue

acute cerebellar ataxia of childhood -MCC ataxia in children 1. pathogenesis + clinical presentation 2. dx / tx

1. varicella, influenza, EBV, mycoplasma--> 2-3 weeks -->AUTOIMMUNE/POST INFECTIOUS--> truncal ataxia, slurred speech/nystagmus, afebrile 2. dx of exclusion, NORMAL HEAD CT SCAN tx = supportive, completely resolves in 2-3 months

hemolytic uremic syndrome 1. pathogenesis 2. lab findings

1. vasculitis characterized by MAHA, thrombocytopenia, acute renal failure + intestinal ulceration and infarction of the bowel, cortical necrosis of the kidney 2. hemoglobinuria, proteinuria, elevated BUN/creatinine

Complications of hyperbilirubinemia: Kernicterus and bilirubin encephalopathy 1. pathogenesis 2. clinical presentation

1. very high concentrations of indirect bilirubin ⇒ pass through BBB ⇒ damage to the basal ganglia, hippocampus, brainstem 2. choreoathetoid cerebral palsy, hearing loss, opisthotonus, seizures, oculomotor paralysis

Genetic evaluation of the fetus 1. Chorionic villus sampling date and tissue collected 2. Amniocentesis sampling date and tissue collected 3. Percutaneous umbilical blood sampling

1. villus tissue from the chorion of the trophoblast at 10-13 weeks 2. amniotic fluid containing sloughed fetal cells 16-18 weeks 3.obtain sample of fetal blood to assess for hematologic abnormalities, genetic disorders, infections, and fetal acidosis

dilated cardiomyopathy 1. causes 2. clinical presentation 3. dx/tx

1. viral (coxsackie B + same as myocarditis), mitochondrial abnormalities, carnitine deficiency, nutritional deficiency in selenium and thiamine, hypocalcemia, anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) ,doxorubicin, alcohol 2. CHF signs and symptoms 3. viral serologies, serum carnitine, ECG (low voltage + ST/T wave changes), evidence of infarction, echocardiogram (shows dilated left ventricle) tx = manage CHF/nutritional deficiencies, repair of ALCAPA, cardiac transplant

acute cerebellar ataxia 1. patho + clinical pres

1. viral illness (cox/vari/echo) -->autoimmune response ---> cerebellar ataxia (nystagmus, vomitting, truncal ataxia)

henoch schonlein purpura - usually younger than 10 years of age 1. pathogenesis 2. labs

1. viral syndrome or URI or GABHS-->IgA mediated vasculitis presents with palpable purpuric rash on the buttocks and lower extremities, edema of hands/feet/scrotum/scalp large joint arthralgias of knee/ankle (early) glomerulonephritis (gross hematuria/nephrotic/chronic renal insufficiency) intussusception, bowel perforation/bleed, colicky GI pain (early) 2. normal platelet count, elevated IgA, normal platelets

rickets 1. pathogenesis 2. risk factors 3. clinical presentation 4. dx + XR

1. vitamin D def--> inadequate mineralization of bones with NORMAL bone matrix 2. breast feeding with no sun, phenytoin, phenobarbital, renal/hepatic failure, fad diets, celiac disease, cystic fibrosis, 3. first 2 years/adolescence, knobby wrists, knees, ribs, bowing of weight bearing bones, short stature, rachitic rosary, craniotabes, frontal bossing, delayed suture closure 4. low phosphorus, low/nrml Ca, inc. Alk phos, inc. PTH, HYPOCALCURIA wrist radiographs show WIDENED, FRAYED, CUPPPED metaphysis, widened space between epiphysis and metaphysis

near drowning 1. mechanism (2)

1. wet drowning-asphyxia from aspirating liquid dry drowning - laryngospasm --> denaturing surfactant, alveolar instability/collapse, pulmonary edema--> decreased compliance, increased resistance, increased pulmnoary artery pressure, decreased gas exchange

Fetal phenytoin syndrome

1. wide anterior fontanelle, thick hair, low nasal bridge with low hairline, hypoplasia of the distal phalanges and nails/ cardaic defects, cleft lip/palate

sexual abuse 1. how do patients who are victims to sexual abuse typically present 2. treatment of suspected sexual abuse

1. with non-specific complaints including abdominal and urogenital, sexual behavior 2. safety of the child, child protective services, pregnancy prevention, empiric antibiotics for STDs

stains for: 1. acid fast baccili 2. fungus 3. stool WBCs 4. herpes family, RSV, adeno, influenza A/B 5.trichamonas vaginaliss 6. treponema pallidum 7. cocci/ m.tuberculosis 8. EBV, CMV, VZV, HIV, toxo, bartonella, myocplasma

1. ziehl- neelsen 2. silver stains 3. wright stain 4. fluorescent antibody 5. direct observation 6. dark field 7. intradermal skin testing 8. antibody testing

physiologic reflux 1. presentation *compare this to GERD in children

1."happy spitters" 60% of all infants have episodes of spitting up/vomitting related to overfeeding THIS IS BENIGN EMESIS, but parents take their kids to the doctor because they are worried

dynamic LVOT obstruction in hypertrophic cardiomyopathy mechanism

1.. anterior mitral leaflet is swept into the subaortic region during systole

neurofibromatosis type 1 1. pathogenesis + clinical presentation

1.AD mutation (defect in neural crest differentiation)--> cafe aulait spots (>6), axillary/inguinal freckleiing, skin neurofibromas, optic glioma, intracranial calcifications, lisch nodules (iris hamartomas), CNS neurofibromas, scoliosis, hypertension

1. labs which should be ordered in suspected sepsis/meningitis 2. lab results that indicate low risk 3. who should be hospitlaized

1.CBC + blood culture, urinalysis + urine culture, CXR 2. WBC <15k bands <1.5k normal urinalysis <10WBCs <5 WBCs normal 3. all infants <28 days of age infants 29-3 months with toxic apppearance, meningitis, pneuonia, pyelonephritis, skin/soft tissue who do not meet low risk criteria

rocky mountain spotted fever 1. pathogenesis + clinical presentation 2. dx/tx

1.SOUTH EASTERN tick bite transmits rickettsia rickettsii gram negative intracellular coccobacillus --> 2. petechial rash BEGINS ON THE FLEXOR SURFACES OF EXTREMITIES AND MOVES CAUDAL AND CENTRIPETAL DIRECTION - includes the palms/soles myalgias, hepatosplenomegaly/jaundice, CNS symptoms like headache, coma, seizure, hypotension 2. dx = thrombocytopenia, hyponatremia, elevated AST/ALT tx = IV doxycycline and supportive care

Angelman syndrome 1. pathogeneiss 2. clinical presentation 3. dx

1.also known as the "happy puppet" syndrome: deletion of a region on the maternally derived chromosome 15. 2. jerky arm movements, small WIDE HEAD, blond hair/blue eyes, prognathia, tongue protrusion ataxia, inappropriate laughter, MR + speech delay 3. FISH probing for both

spinal muscular atrophy 1. pathogenesis + clinical presentation 2. type 1 vs 2 vs 3

1.autosomal recessive SMN1 mutation chromocomse 5 --> anterior horn degeneration by astrocytes/microgia -->hypotonia, weakness, tongue fasiculations, bell shaped chest, frog leg posture, areflexia, NORMAL EXTRAOCULAR MOVEMENTS NORMAL SENSORY EXAM 2. type 1 = infantile(werdnig hoffman) onset, death by 1 year type 2 = 6-12 months onset, adolescent survival type 3 = >3 years onset adult survival

Meconium Aspiration Syndrome (MAS) 1. What is meconium? 2. What is MAS?

1.first stool;is a material in the fetal gut that consists of water,mucopolysaccharides, desquamated skin and gastrointestinal mucosal epithelial cells, vernix, bile salts, and amniotic fluid. 2. acute respiratory disorder caused by the aspiration of meconium into the airways of the fetus or neonate

Tyrosinemia type 1 1. clinical presentatoin 2. dx/tx

1.fumarylacetoacetate hydrolase --> elevated tyrosine --> rotten fish odor, episodic peripheral neuropathy, RENAL TUBULAR FUNCTION AND LIVER, hepatocellular carcinoma/cirrhosis 2. succinylacetone in urine, diet restriction of phenylalanine

RDS 1. pathogenesis 2. in utero assessment of fetal lung maturity 3. epidemiolgoical risk factors 4. clinical risk factors

1.pulm surfactant produced 23-24 weeks, sufficient by 30-32 weeks ~ birth <32 weeks ⇒inc. surface tension due to dec. surfactant⇒ RDS 2. sample amnion ⇒ lecithin to sphingomyelin ratio >2:1, and presence of phsosphatidylglycerol = maturity 3. higher in white male preterm infants 4. low L:S, prematurity**, maternal diabetes**, neonatal hypothermia/asphyxia, male sex, ceserean section w/o labor

Niemann pick disease 1. patho

1.sphingomyelinase deficiency --> progressive neurodegeneration, ataxia, seizures hepatosplenomegaly, cherry red macula ~ death by age 4 (like tay sachs)

HSV conjunctivitis (neonatal) 1. pathogenesis + clinical presentation 2. dx/tx

1.vaginal canal --> 6 days - 2 weeks of life- usually unilateral with serous discharge, 2, dx = multinucleated giant cells on gram stave, positive culture tx = acyclovir, topical triflurothymidine

1. Is gynecomastia common in adolescnet males common? 2. ddx 3. tx

1.yes very common, 60% of adolescent males 2. medications, testicular tumors, thryoid dz, liver dz 3. reassurance - usually resolves in 12-15 months

caloric irrigation (come eval)

10-30ml of ice water into the ear, eyes deviate toward irrigated side, defective response indicates pontine injury

what is the diagnostic test to test for suspected congenital heart disease

100% oxygen challenge test- PaO2 does not rise following administration of 100% oxygen ~~~ then do an echocardiogram

how to calculate maintenance water requirement

100mL/kg/day for the first 10kg 50mL/kg/day for the second 10kg 20ml/kg/day for each kg above the first 20 kg of body weight

Birth length triples by

13 years

Regain birth weight

2 weeks

what fraction of childhood diabetes is type 2 in children

2-3 %

how to calculate maintenance sodium how to calculate maintenance potassium

2-3 mEq/kg/d maintenance sodium 2 mEq/kg/d maintenance

when after birth does Hgb reach its lowest point (nadir)

2-3 months of age, and 1-2 months in preterm infant

what is the risk of congenital heart disease for a fetus who has a sibling with congenital heart disease

2-6% general population = 1%

Expected weight gain: 2 years - adolescence

2.3 kg/year

what is visual acuity at birth

20/200, and improves during the first 3-4 months of life

Expected weight gain: 3 - 6 months

20g/day

cyclic neutropenia

21 day cyclic alterations in neutrophil counts --> episodes of neutropenia-->

what is the gold standard of GERD diagnosis

24 hour reflux monitoring

Expected height increase: 0 - 12 months

25 cm/year

Head size progression from birth to one year

25% adult size--> 75% adult size

Expected weight gain: 1 - 2 years

250 g/month

management of renal failure 1. what maintenance fluid should be given to a patient with acute renal failure 2. other management for acute renal failure

300ml/m2/day+urine/stool replacement ~~ only correcting for insensible loss other = electrolyte intake (low sodium, potassium, phosphorus diet) protein restriction dialysis monitor BUN/creatinine, calcium, ALP blood pressure management EPO for anemia

Expected weight gain: Birth - 3 months

30g/day

When does suture closure normally occur

90% by age 2, complete by age 5

what is the frequency of cleft palate in siblings who have cleft palate vs general population

4% for siblings general population is 1/1000

Double birth weight by

4-6 months

what percentage of children have a single afebrile seizure, what fraction go on to develop epilepsy

4-6 percent <1/3 go on to develop epilepsy epilepsy freq = 0.5-0.8% of the population

ALL 4. clinical presentation 5. lab diagnosis

4. fever, bone/joint pain/refusal to bear weight, pallor, bruising, hepatosplenomegaly, lymphadenopathy +/- epistaxis, anorexia, fatigue, abdominal pain 5. CBC: leukemic blasts in blood, high 1/3 >50k, 1/3 normal, 1/3 <10k **MAY BE NORMAL, thrombocytopenia, anemia Marrow tap --> replacement of marrow by lymphoblasts

frequency of stools at 1 week of life 1 year 4 years ~ adults defecate 3x/day to 3x/week

4x/day, 2x/day, 1x/day

1. what fraction of adolescents are clinically depressed 2. which gender has more depression

5% 2. girls 2x>boys

Treatment for Adrenal crisis

5% dextrose in normal saline to stabilize volume + glucose level, IV steroids to replace lost cortisol

depressed fontanelles, tachycardia, sunken eyes, loss of skin elasticity = ~ what % of dehydration

5-9% dehydrated

Dx criteria for major depression

5/9 almost every day for 2 weeks of: a. Depressed or irritable mood b. Diminished interest or pleasure in activities c. Weight gain or loss d. Insomnia or hypersomnia e. Psychomotor agitation or retardation f. Fatigue or energy loss g. Feelings of worthlessness h. Diminished ability to concentrate i. Recurrent thoughts of death or suicide

compartment syndrome pneumonic

5Ps pallor, pulselessness, paraysis, pain, paresthesisas

ALL 6. tx 7. treatment of relapse

6. induction - corticosteroids, vincristine, L-asparginine, **intrathecal methotrexate consolidation - **intrathecal methotrexate, cranial irradiation (children>5yo) maintenance - daily/periodic chemo up to 3 years and discontinued after 2-3 years of disease free 7. BMT

Expected height increase: 2 years - adolescence

6.25 cm/year

what is normal birth weight

7 pounds 8 ounces, 3400 grams

when should a child get a lumbar puncture for a febrile seizure

<12 months uncertain neurological exam **DONT GIVE LP FOR >18 MONTHS WITH NORMAL NEUROLOGICAL EXAM AND NO SIGNS OF MENINGITISA

what is the content of breast milk

70% whey, 30% casein, lactose lactoferrin, lysosyme, secretory IgA

oliguria

<1ml/kg/hr urine output

sail sign

<2 year old CXR - large thymic shadow overlaps with the heart and looks like a "boat sail"

what temperature should an infant with fever be evalulated for meningitis, pneumonia, sepsis, bone/joint infections

<28 days ANY FEVER >28 days fever >39 (102.2)

what fraction of calories should come from fats

<30%

ALL 8. infectious complications of ALL 9. what does fever >1 week while on antibiotics indicate 10. tumorlysis syndrome

8. neutropenia (<500) + assc sepsis - s.aureus, s.epidermidis, + oppurtunistic: pseudomonas, HSV, PCP, candida, aspergillus 9. fungal infection 10. hyperuricemia/hyperkalemia/hyperphosphatemia-->hypocalecmia~~ from rapid death/lysis of tumor cells

Clinical features of lead intoxication

Acute lead intoxication may lead to: -acute onset of anorexia -ap- athy, lethargy, anemia, irritability, and vomiting. These symptoms may progress to encephalopathy

When does secondary tooth eruption begin?

Begins with the lower central incisor between 6 and 8 years of age There are 32 secondary or permanent teeth.

Edema of the feet with hypoplastic nails

Characteristic of: •turner syndrome •noonan syndrome

Dental Trauma

A permanent tooth that has been traumatically avulsed may be re-implanted if the avulsed tooth is placed into the socket rapidly. a. Extra-oral time is the most important factor affecting the prognosis for successfully re-implanting a tooth. b. Prognosis is highest if the avulsed tooth is stored in liquids, especially milk. c. A dry-stored tooth has a poor prognosis for re-implantation, even after only 30 minutes. Management includes gentle rinsing of an avulsed tooth with saline, placement into the socket, and referral to a dentist.

poisoning---- 1. what are the causes of anion gap metabolic acidosis 2. what are the radioopaque substances that can be seen on abdominal Xray

AMUDPILES (AG>16) 1. alcohol, methanol, uremia, DKA, paraldehyde, iron/isoniazid, lactic acidosis, ethylene glycol, salicylates 2. CHIPE - chloral hydrate, calcium, heavy metals, iodine/iron, phenotyiazines, enteric coated tablets

Which congenital heart diseases cause increased pulmonary flow

ASD/VSD/PDA transposition of the great arteries truncus arteriosus, TAPVC

most common cause of subarachnoid hemorrhage in children

AVM~~~ associated with childhood seizures

Clinical features of lead intoxication

Acute lead intoxication may lead to acute onset of •anorexia, apathy, lethargy, anemia •irritability, and vomiting. **These symptoms may progress to encephalopathy Chronic lead intoxication is most commonly asymptomatic; however, even patients with very low levels of lead may suffer neurologic sequelae, including: •developmental delay •learning problems •mental retardation.

Common Behavioral Concerns

A. Colic B. Enuresis C. Encopresis D. Sleep Problems E. Eating Problems F. School Phobia G. Temper Tantrums H. Breath-holding spells I. Sibling Rivalry J. Toilet Training K. Discipline Issues

Evaluation of the Newborn

A. General Appearance B. Skin Examination C. Craniofacial Examination D. Neck and Clavicle Examination E. Chest Examination F. Respiratory Examination G. Cardiac Examination H. Abdominal Examination I. Genitalia Examination J. Extremity Examination K. Spine Examination

Disorders of Development

A. Motor deficits -Cerebral palsy -Extrapyramidal cerebral palsy B. Cognitive deficits -Mental retardation -Learning disabilities C. Pervasive developmental disorder (PDD) -Autism -Asperger syndrome

Pectus carinatum and pectus excavatum

A. Pectus excavatum (depressed sternum) B. Pectus carinatum (prominent and bulging sternum) *Generally benign

Abnormalities of Maturity

A. Preterm Delivery B. Post-term Delivery

Growth Abnormalities

A. Small-for-gestational-age (SGA) infants and intrauterine growth retardation (IUGR) B. Large-for-gestational-age (LGA) infants

1. what is the primary survey* when a child comes to the emergency room 2. what else is done in the emergency room when a child first arrives

ABCDE • Airway, • Breathing and ventilation (on 100% O2), • Circulation (control of hemorrhage) • Disability (GCS), • Exposure/environmental (undressed for examine then warmed to prevent hypothermia) 2. ECG monitor (dysarrythmias = cardiac injury), pulseless activity (tamponade, pneumothorax, hypovolemia urinary catheter/NG tube Radiographs: CXR, cervical spine, pelvis, CT,

treatment of a patient who has nearly drowned

ABCs cervical spine immobilization, remove wet clothing intubation/mechanical ventilation with positive end expiratory pressures for respiratory failure rewarming of body core (warm saline lavage, gastric lavage,

what test for adrenal insufficinecy

ACTH stimulation test~~ if there is blunted response to ACTH there is primary adrenal indsufficeincy

diagnosis of neural tube defects + spina bifida + any spinal defect

ALPHA FETO PROTEIN******************* MEASURED AT 16-18 WEEKS + fetal ultrasound

Macroglossia

May suggest: •Beckwith-Wiedemann syndrome •Hypothyroidism •Mucopolysaccharidosis

When should children be screened for lead?

All children 9 months - 6 years of age: •living in old housing who are siblings, visitors, or playmates of children with lead intoxication •living near lead smelters or lead- processing plants •whose parents or family members have a lead-related occupation or hobby. Children of any age living in older housing where renovation is occurring. All children of any age living in areas in which the percentage of 1- to 2-year-olds with elevated lead levels exceeds 12%.

Neonatal Metabolic (State) Screening

All states screen for: •congenital hypothyroidism •phenylketonuria (PKU) Majority of states also screen for: •galactosemia •sickle cell anemia and other hemoglobinopathies Some states screen for: •additional metabolic disorders (e.g., congenital adrenal hyperplasia)

Congenital microcephaly

Associated with abnormal induction and migration of brain tissue. Causes: •Early prenatal infection (e.g., HIV, TORCH) •Maternal exposure to drugs and toxins (e.g., fetal alcohol syndrome) •Chromosomal abnormality (e.g., trisomy 13, 18, or 21) •Familial microcephaly (AD or AR inheritance) •Maternal phenylketonuria **TORCH = toxoplasmosis, other (syphilis), rubella, cytomegalovirus, herpes simplex virus.

Absence/hypoplasia of radius

Associated with: •TAR syndrome (thrombocytopenia absent radii) •Fanconi anemia •Holt- Oram syndrome.

Contraindications to rotavirus vaccine

Anaphylaxis to vaccine ingredients History of intussusception History of uncorrected congenital malformation of the gastrointestinal tract (e.g. Meckel's diverticulum) Severe combined immunodeficiency disease

What can cause a hypertrophied clitoris?

Androgen excess associated with virilizing adrenal hyperplasia

cartilage hair hypoplasia syndrome

Auto recessive - short stature, immunodeficiency, fine hair, neutropenia

what kind of neoplasm common in HIV positive patients

B cell lymphoma caused by EBV

1. treatment of CML adult CML - chronic phase - contorlled by chemo/BMT-->blast crisis acute leukemia -->unresponsive to therpay juvenille CML often fatal, relapse in 50% of cases

BMT - for both adult/juvenille

abnoraml gag/corneal reflexes

BRAINSTEM INJURY

does breastfed or formula fed infant defecate more

BREASTFED UP UNTIL 4 MONTHS THEN IT IS THE SAME

what can a mother do to decrease food allergies/atopic dermatitis

BREASTFEEDING

Head shape in craniosynostosis

Based on which suture closes prematurely. a) dolichocephaly or scaphocephaly b) brachycephaly c) trigonocephaly Premature closure of multiple sutures is rare but is associated with severe neurologic compromise.

What is the range for initial tooth eruption?

Between 3 and 16 months, with an average of 6 months. *Tooth eruption may be delayed or early

hereditary angioedema patho + clinical presentatino

C1 esterase inhibitor deficiency --> angioedema of the hands and feet + GI symptoms + laryngeal edema/obstruction

erbs palsy

C5 C6 nerve root injury --> flaccid arm/asymmetric moro reflex *arm internally rotated, elbow extended, forearm pronated, wrist/fingers in flexion

klumpkes palsy

C7 C8 - claw hand from unopposed finger flexion, decreased ability to extend the elbow +/- horners syndrome*******

why is cyanosis a bad marker of poor oxygenation if Hbg < 5g

CANNOT SEE THE CYANOSIS

doxorubicin toxicity

CARDIOTOXICITY, alopecia, N/V, stomatitis, tissue necrosis

lab diagnosis/radiological diagnosis of crohns/ulcerative colitis

CBC shows leukocytosis/anemia, ESR is elevated, albumin/serum transaminases assess nutritional status and liver disease, serum antibody tests (ANCA, or anti-saccharomyces cerevisiae) UGI study, colonoscopy with biopsys

hypertension lab evaluation

CBC, electrolytes, BUN, creatinine, urinalysis, renin, chest radiograph

clotting abnormality lab evaluation

CBC, platelet count, blood smear, PTT, PT, platelet function assay

lab studies to order for diarrhea

CBC, serum electrolytes, stool guaiac, WBCs, ova/parasite, ELISA - for rota, giardia, c.diff

symptoms of large PDA

CHF due to increased pulmonary blood flow

what virus must a transfusion be screened for before giving to an HIV patient

CMV

congenital CMV vs toxoplasmosis intracranial calcifications

CMV = periventricular toxo = coritcal

what viral infections seen in HIV positive patients

CMV retininitis/esophagitis/colitis HSV, VZV

1. what is the cause of heterophile negative infectious mononucleosis 2. how to diagnose EBV in children <4 years of age

CMV, toxo, HIV, also monospot test is less sensitive in chidlren less than 4 years of age 2. EBV antibody titers anti- VCA, EA, EBNA EBNA = late antibody IgM anti VCA = early antibody

HITB vaccine 1. components 2. timing

CONJUGATE - H-influenza polysaccharide linked to protein antigens including diptheria/tetanus toxoid • Recommended either at 2, 4, and 6 months with a booster at 12-15 months or at 2, 4, and 12 months, de- pending on the type of vaccine conjugate.

what are the markers of systemic inflammation/infection

CRP (Rises fast) and ESR (rises slow)

Acquired microcephaly

Caused by a cerebral insult in the late third trimester, perinatal period, or first year of life. • Affected children are born with a normal head circumference that does not grow after the cerebral insult. Causes: • Late third trimester or perinatal infections • Meningitis or meningoencephalitis during first year of life • Hypoxic or ischemic cerebral insult • Metabolic derangements (e.g., hypothyroidism, inborn errors of metabolism)

Poland syndrome

Chest asymmetry, as a result of absence of the formation of ribs or agenesis of the pectoralis muscle

Temper tantrums - expression of anger beyond childs control 1. age of onset 2. cause 3. tx

Common between 1-3 years 2. frustration/fatigue more likely with coexisting poor fine motor/expressive language delays 3. ignore demands, wait until age 3 when emotion can be verbalized, holding by parent to help calm

Genital ulcers

Commonly caused by: •herpes simplex virus types 1 and 2 •Treponema pallidum (syphilis)-chancre •Haemophilus ducreyi (chancroid)

Mechanical intestinal obstruction

Congenital • Intestinal atresia or stenosis • Meconium ileus • Meconium plug • Malrotation (with or without volvulus) • Duplication • Incarcerated hernia • Annular pancreas • Preduodenal portal vein • Intestinal cysts • Anorectal malformations Acquired • Intussusception • Adhesions • Postnecrotizing enterocolitis stenosis • Lactobezoar

prevention of malaria

DEET repllants, bed nets, protective clothing chemoprophylaxis with chloroquine/mefloquine/doxycycline/atovaquone/proguanil hydrochloride

treatment of ingestion of acid or alkali

DONT NEUTRALIZE (this will generate an exothermic reaction NO IPECAC, GASTIC LAVAGE, OR ACTIVATED CHARCOAL endoscopy, do not treat for household bleach ingestion

Normal sexual differentiation

During the first 7 weeks of gestation, the gonadal tissue remains undifferentiated. Male sexual differentiation is an active process, whereas female sexual differentiation develops when genetic and hormonal influences are absent.

Dysthymic disorder clinical features

Dysthymia is a more chronic mood disturbance that lasts at least 1 year. Symptoms are milder than depression, include 2/5 of: a. Poor appetite or overeating b. Insomnia or hypersomnia c. Diminished energy d. Difficulty concentrating e. Feelings of hopelessness

autoimmune neutropenia

EBV/drugs/SLE/juvenille rheumatoid arthritis --> anti-neutrophil antibodies --> neutropenia

salter harris fracture classification I II III IV V

I= fracture in the physis II = fracture in the physis extends to metaphysis III= fracture in the physis extends to the epiphysiss IV = fracture in the physisi through both metaphysis/epiphysis V = crush of the physis

What are the causes of peripheral precocious puberty?

INDEPENDENT OF THE HPGA •exposure to exogenous sex steroids •gonadal tumors (leydig & B-HCG tumors in boys, virilizing ovarian tumors eg. granulosa cell) in girls •Testotoxicosis in boys •adrenal tumors •non-classic CAH •McCune albright

what is phototherapy used for

INDIRECT HYPERBILIRUBINEMIA ONLY, NOT DIRECT

stills murmur: age, location, characteristic

INNOCENT MURMUR AGE 2-7, systolic vibratory/buzzing - found at the mid left sternal border, loudest when supine/exercise grade 1-3

pulmonic systolic murmur: age, location, characteristic

INNOCENT MURMUR any age systolic blowing high pitched, found at upper left sternal border loudest when supine/exercise grade 1-2

kid falls and hits his head with a pencil in his mouth, 24 hours later stroke like symptoms

INTERNAL CAROTID ARTERY DISSECTION

Fetal cocaine

IUGR, microcephaly, GU abnormalities

Visual Impairment

Incidence is 1:1600 children. Classification of blindness uses measures of corrected acuity in the better eye as determined by Snellen chart testing and by determination of visual fields.

Pneumonia 1. etiology 0-3 months 2. 3 months to 5 years 3. >6 years

Infection and inflammation of lung parenchyma 1. TORCH, GBS, gram negative rods, listeria, RSV chlamydia, ureaplasma, mycoplasma, PCP 2. adeno, influenza a/b, parainfluenza, RSV, s.pneumo, s.aureus, HIB 3. adeno, influenza a/b, parainfluenza, s.pneumo, mycoplasma, chlamydia pneumoniae

What labs should be ordered for suspected IEM

Initial evaluation includes: •metabolic acidosis •elevated serum ammonia Further evaluation depends on whether metabolic acidosis or hyperammonemia are present.

Nonphysiologic jaundice 1. indirect vs direct hyperbilirubinemia 2. which type is pathologic in neonates

Jaundice that is secondary to a pathophysiologic cause 1. indirect <15% conjugated, direct >15% conjugated 2. direct hyperbilirubinemia

spondylolithesis

L5-S1 vertebral body slips anteriorly and can impinge on nerve roots, "PALPABLE STEP OFF" in the lumbosacral area

what is the auscultation finding of pulmonary hypertension (often from congenital heart disease)

LOUD P2 *****

MMR vaccine 1. diseases of MMR 2. timing

Live attenuated vaccine •measles - pneumonia/koplic spots + encephalitis •mumps - parotitis, meningoencephalitis, orchitis •rubella - causes a mild viral syndrome in children but may cause severe birth defects in infants born to mothers infected during pregnancy •MMR is recommended at 12-15 months with a booster at either 4-6 years or 11-12 years of age

Trisomy 18/13 triple screen

Low values of •AFP •Unconjugated estriol •β-HCG

burn surface area (BSA)

Lund-browder classifcation arms = 9% legs = 18% anterior trunk = 18% posterior trunk = 18% head and neck = 9% Or can estimate burn area with palm size ~ one childs palm = 1% of body area

back strain

MCC back pain, muscular soreness from overuse or bad body mechanics

what toxicity is associated with methotrexate

MUCOSITIS (unique), bone marrow suppression, skine erythema, heaptic dysfunction

Congenital hypoplastic anemia (Diamond-Blackfan syndrome) 1. pathogenesis + clinical pres 2. dx 3. tx

Macrocytic anemia Low reticulocyte count Congenital anomalies (webbed neck, cleft lip, shielded chest, triphalangeal thumbs) 1. AR/AD inheritance--> pure red cell aplasia--> rapid onset anemia in first year of life, craniofacial, renal, cardiac abnormalities, triphalangeal thubs 2. decreased Hgb, dec retics, increased HbF, decreased RBC precursors in the marrow 3. RBC transfusion, corticosteroids, BMT

What is calculation of male/female mid-parental height (MPH)

Male = fathers height + (mothers height + 5inches)/2 Female = (fathers height -5) + mothers height /2 ~ when children have completed their growth they are within 2-4 inches of this calculation

which tumor of childhood mets to bones most often

NEUROBLASTOMA****

do WPW, kawasaki disease, and cardiac dyarrythmias need prophylaxis for infectious endocarditis

NO ~<6months status post heart sturgery, all congneital heart defects except secundum ASD

medullary thyroid carcinoma calcium and phosphours

NORMAL NORMAL (despite elevated calcitonin)

transient neonatal hypoglycemia 1. causes

NOT ENOUGH SUBSTRATE: prematurity, perinatal asphyxia, fetal distress, SGA, LGA (ie. all of these conditions have low glycogen stores) INAPPROPRIATE HYPERINSULIN: maternal diabetes

Isolated short stature

NOT FAILURE TO THRIVE ***** height is the most abnormal growth parameter In FTT, weight is the most abnormal growth parameter

is PVR reversable if VSD is closed + what happens if PVR exceeds SVR

NO~~ if PVR>SVR there is shunt reversal and this is called EISENMENGER SYNDROME

treatment of juvenille rheumatoid arthritis

NSAIDs glucocorticoids, MTX, sulfasalazine, hydroxychloroquine surgery for recalcitrant joints psychosocial support

Most common congenital anomaly of the CNS

Neural tube defects

Night terrors

Night terrors occur 90-120 minutes after sleep onset during non-REM stage 4 sleep. •common at 3-5 years of age but have a pattern distinct from ordinary nightmares. (1) History. Parents describe a child who suddenly arouses screaming and thrashing with signs of autonomic arousal such as tachypnea, tachycardia, and diaphoresis. The child does not respond to visual or verbal cues, and parents report the child stares "glassy- eyed" without seeing. The child does not remember the incident the next day. (2) Management. Reassuring parents and telling them that the episodes usually terminate spontaneously and will resolve over time is helpful.

Polio disease

Poliovirus is an enterovirus (picornavirus) • destroys gray matter of the spinal cord in the anterior horn leading to lower motor neuron weakness and transient/perminent paresis and meningoencephalitis

Enuresis epidemiology

Nocturnal enuresis is more common in boys. Incidence is based on age. Bed-wetting occurs at least monthly in: (1) 30% of 4 year olds (2) 15-20% of 5 year olds (3) 10% of 6 year olds (4) 3% of 12 year olds

When do primitive reflexes normally disappear, and what is the effect of CNS injury on the primitive reflexes

Normally disappear by 3-6 mo Infants with central nervous system (CNS) injuries show stronger and more-sustained primitive reflexes

how to treat chlamydia conjunctivitis in an infant

ORAL SYSTEMIC erythromycin due to the risk of chlamydia pneumonia

Neonatal and natal teeth

Occasionally infants are: 1) born with teeth (Natal teeth) or 2) have teeth that erupt within the first month of life (Neonatal teeth) • Etiology is often unknown but may be caused by exposure to environmental toxins or may be familial. • Teeth most commonly present early are mandibular central incisors. • More than 90% are primary teeth that erupt early and < 10% are supernumerary teeth (teeth in excess of usual number) No intervention is needed unless the teeth are HYPERMOBILE, cause breast feeding difficulty or trauma to the infant's lip or tongue

Spasmodic croup

Occurs year round in preschool age children Likely secondary to a hypersensitivity reaction acute onset of stridor croup at night, resolves without treatment

Noonans syndrome 1. pathogenesis 2. clinical presentation

Often described as the male version of Turner syndrome, but females may also be affected. 1. chromosome 12 deletion 2. short stature, shield chest,short webbed neck, low hairline, hypertelorism, epicanthal skin folds, downslanting palpebral fissures, and low-set ears. Cardiac defects include right-sided hear lesions, most commonly PULMONARY VALVE STENOSIS (In contrast, patients with Turner syndrome have left-sided heart lesions.)

Physical effects of marijuana use and long term consequences

Physical effects: •tachycardia, mydriasis, sleepiness, conjunctival injection, dry mouth, auditory/visual hallucinations Long term consequences: •asthma, impaired memory/learning, diminished interpersonal interactions, depression

Testicular neoplasms 1. clinical presntation + cell type 2. dx 3. Management

Originate from germ cells within the testicle and are one of the most common malignant solid tumors in males 15-35 years of age. 1.present as firm, irregular painless nodules in the testicle 2. doppler ultrasound, serum tumor markers (AFP, B-HCG), find mets 3. surgery, radiation, and chemotherapy

Eating problems

Overfeeding during infancy is common: Parents may equate food with love and enjoy seeing their infant grow and thrive. a. Appetite normally decreases after 1 year of age. b. Control is the major issue in toddler feeding problems. Autonomy is more important than hunger to the child at this stage. c. Management includes avoiding power struggles, offering food without comment, and counseling parents to avoid bribes, pressuring, or forcing the child to eat.

TTP clinical presentation

PENTAD- thrombocytopenia, MAHA, neurologic disturbance, fever, renal dysfunction

charcot marie tooth clinical presentation

PMP 22 mutation --> peripheral demyelination--> atrophy of the peroneal and intrinsic foot/hand/proximal leg muscles

1. head righting 2. appears/dissapearsg

POSTURAL REACTION 1. keep head vertical despite body being tilted 2. 4-6 months/persists

Persistent pulmonary hypertension of the newborn 1. what age group infants does this effect 2. pathogenesis 3. clinical presentation

PPHN is any condition, other than congenital heart disease, associated with low blood flow to the lungs after birth 1. near-term, full-term, or post term 2. perinatal asphyxia/MAS ⇒ inc. pulmonary vascular resistance (due to dec. O2) ⇒ R-L shunt through foramen ovale/PDA 3. cyanosis, respiratory failure, large dec. PaO2 in response to minimal inspired oxygen changes or stimulation, pre/post ductal PaO2 are notably different

interventricular hemorrhage pathogenesis + clinical presentation

PREMATURITY/LOW BIRTH WEIGHT --> bulging fontanelles, pallor, cyanosis, focal neurologic signs, hypotension

what group of infants have a high incidence of PDA

PRETERM

Dolichocephaly/scaphocephaly

Premature closture of the sagittal suture, results in elongated skull * most common form of craniosynostosis

Brachycephaly

Premature closure of the coronal suture leading to shortened skull • more common in boys • more commonly associated with neurologic complications such as optic nerve atrophy.

Trigonocephaly

Premature closure of the metopic suture leads to triangular-shaped head

When are primary teeth established?

Primary teeth (20 teeth in total) are generally established by 2 years of age

Transient erythroblastopenia of childhood 1. pathogenesis + clinical pres 2. dx 3. tx

Pure red cell aplasia without macrocytosis. 1. *post viral autoimmune --> slow onset symptomatic anemia 2. low Hgb, low retics, dec. RBC precursors in the marrow 3. no treatment, spontaneous recovery Most cases are diagnosed after 1 year of age and there is no associated congenital anomalies

thalassemia vs IDA RDW

RDW increased in IDA (inc. variablility of red blood cell width) RDW normal in thalassemia trait (TIBC and ferritin normal)

Newborn Respiratory Examination

Respiratory distress is diagnosed if: •tachypnea (respiratory rate > 60 breaths/min) •deep respirations •cyanosis •expiratory grunting, or •intercostal or sternal retractions are present.

Apnea of Prematurity

Respiratory pause without airflow lasting > 15-20 seconds, or a respiratory pause of any duration if accompanied by bradycardia and cyanosis or oxygen desaturation, as evidenced by pulse oximetry monitoring.

how does S2, mitral filling rumble change in a VSD in which the PVR is beginning to increase

S2 gets louder due to inc. pulmonary pressures mitral filling rumble decreases because of diminished pulmonary blood flow

what can cause a false positives/negatives urine dipstick for protein

SG >1.025, ph>7, penicillin, aspirin, oral hypoglycemia false negative - dilute urine

what mother condition leads to a neonate with third degree heart block

SLE

Periodic breathing

Seen in PRETERM infants •breathe irregularly with short, apneic bursts that last less than 5-10 seconds and have no clinical significance

Learning disabilities

Significant discrepancy between a child's academic achievement and the level expected on the basis of age and intelligence. Causes include: •CNS insults (e.g., prematurity, closed head injury, lead poisoning, fetal alcohol syndrome) •genetic disorders (e.g., fragile X syndrome) •metabolic disorders (e.g., galac- tosemia) The most common cause is idiopathic.

Differential diagnosis for colic

Sources of pain and discomfort that may be confused with colic include: •intestinal gas •milk protein intolerance •food allergy •corneal abrasion •otitis media •testicular torsion •inguinal hernia •digital ligature (i.e., hair wrapped around finger or toe).

Pneumococcal disease

Streptococcus pneumoniae is the most common cause of acute otitis media and invasive bacterial infections in children younger than 3 years of age There are two types of vaccines: • Pneumovax • Prevnar

complications of short bowel syndrome

TPN cholestasis, intestinal bacterial overgrowth, nutritional deficiencies, poor bone mineralization, renal stones, secretory diarrhea

Sibling rivalry

The arrival of a newborn is especially stressful for children younger than 3 years of age. Jealousy is frequently demonstrated up to 5 years of age. Management: a. Before arrival of newborn. Methods for prevention include talking about the arrival of the new baby and praising the child for mature behavior. b. In older children. Children should be encouraged to settle their own arguments without hitting, name calling, or property damage.

Nightmares

They occur during rapid eye movement (REM) sleep. •common after 3 years of age, although they may occur as early as 6 months of age. •These frightening dreams tend to have themes of threats to security, separation, self-esteem, or survival. (1) History. The child is able to give a detailed recall of ex- tended and frightening dreams. The child rapidly becomes alert and oriented after awakening. (2) Management. Reassurance by the parents and comforting measures are helpful. Any inciting causes (e.g., frightening movies) should be identified and eliminated.

Autism

This condition is the prototypical PDD. (1) Incidence is subject to debate. (2) Onset is prior to age 3 years. (3) Autism is more common in boys

Pervasive developmental disorder (PDD)

This spectrum of develop- mental disabilities affects multiple developmental areas, especially behavior and learning, with a wide range of severity.

Management of polycythemia

Treatment includes partial exchange transfusion •blood is removed and replaced by the same volume of plasma substitute (normal saline) in a stepwise manner.

IUGR 1. type 1 2. type 2 3. type 3

Type I:Early interference with fetal growth from conception to 24 weeks gestation Type II:Intrauterine malnutrition from 24 to 32 weeks gestation Type III:Late intrauterine malnutrition after 32 weeks gestation

arthritits of inflammatory bowel disease

UC/crohns HLAB27 positive, have axial skeleton arthritis indistinguishable from ankylosing spondylitis

most common acquired hear diesease in children in the UNITED STATES VS WORLD

United states = kawasaki world = rheumatic fever

Hearing impairment prognostic factors

Variables influencing impact of hearing loss on function and development include: a. Degree of loss b. Etiology (inherit fare better, acquired have higher risk for other neurologic impairment) c. Family atmosphere (e.g. willingness and ability to use sign language) d. Age at onset (<2 are at disadvantage) e. Timing of amplification and educational interventions (the earlier the better) f. Cochlear implants

viral meningitis: CSF: WBC, protein, glucose, gram stain

WBC 10-1000 monos + lymphs, HSV shows RBCs protein normal, glucose normal, enterovirus/HSV identified by PCR

parameningeal focus (brain abscess) CSF findings

WBC 10-200, PMNs/monos protein high glucose normal culture negative

tuberculosis meningitis: CSF: WBC, protein, glucose, gram stain, CT FINDINGS

WBC 10-500 lymphos protein very high, glucose low PCR may be positive, smear usually negatie CT FINDINGS = ENHANCEMENT OF THE BASILAR CISTERNS

bacterial meningitis CSF: WBC, protein, glucose, gram stain

WBC 100-50k, high protein, low glucose positive gram stain

fungal meningitis:CSF: WBC, protein, glucose, gram stain

WBC 25-500 lymphos protine - normal to high glucose low culture positive, india ink for cryptococcus

lumbar puncture results for bacterial meningitis

WBC>5k with predominance of neutrophils hypoglycorrhachia CSF:serum <0.40 increased protein positive gram stain and culture

what tumors present as an abdominal mass

WILMS TUMOR NEUROBLASTOMA

what are the MCC of SVT (pulse >250)

WPW CHD sympathomimmetic drugs

rett syndrome pathogenesis + clinical presentation

X chromosome MeCP2 mutation-->rapid decline at 6-18 months of age --> dec. ability to communicate/socialize

congenital aqueductal stenosis 1. pathogensis

X linked trait, associated with thumb abnormalities, and other CNS anomalies like spina bifida

auspitz sign

a drop of blood is formed when a scale from a psoriasis patch is removed

Micrognathia

a small chin

Contraindications to immunization

a. Anaphylaxis to a vaccine or its constituents b. Encephalopathy within 7 days after DTaP vaccine c. Patients with progressive neurologic disorders, including uncontrolled epilepsy, should not receive the DTaP vaccine until neurologic status is stabilized. d. Immunodeficient patients should not receive OPV, MMR, and varicella vaccines. e. Household contacts of immunodeficient patients should not receive OPV vaccine as it is shed in the stool. f. Pregnant patients should not receive live vaccines.

Social skills milestones

a. Attachment b. A sense of self and independence c. Social play

Two types of newborn hearing screen tests *The most effective screening is thought to be use of both of these tests in combination.

a. Brainstem auditory evoked response (BAER) **most accurate test *requires costly equipment and trained operators -measures EEG waves in response to clicks via electrodes pasted to the infant's scalp b. Evoked otoacoustic emission (EOE) **accuracy may be affected by debris or fluid within the external or middle ear - measures sounds generated by normal cochlear hair cells that are detected by a microphone placed into the external auditory canal

Etiology of hypergonadotropic hypognadism

a. Chromosomal disorders •Klinefelter syndrome •Turner syndrome b. Autoimmune disorders •Hypogonadism in autoimmune oophoritis

Newborn Mouth examination

a. Clefts of the lip and of the soft and hard palates b. Micrognathia c. Macroglossia d. Neonatal teeth e. Epstein pearls

Medical evaluation for hearing loss

a. Complete history (including history of perinatal infections and antibiotic exposure) and physical examination, focused on a thorough ear examination b. Genetics evaluation and chromosome studies if there are suspicious clinical findings or family history c. Creatinine level, because of the association between kidney disease and ear abnormalities (Alport syndrome) d. Viral serologies if clinically indicated, looking for toxoplasmosis, rubella, cytomegalovirus, herpes, and other viruses that can cause deafness (i.e., TORCH infections) e. Consider a computed tomography scan of the inner ear if etiology is undetermined.

Etiology of hypogonadotropic hypogonadism

a. Constitutional delay of puberty b. Chronic diseases (e.g. IBD, anorexia nervosa, renal failure, and heart failure) c. Hypopituitarism of any cause (e.g. brain tumors) d. Primary hypothyroidism e. Prolactinoma f. Genetic syndromes •Kallman syndrome •Prader-Willi syndrome •Lawrence-Moon-Biedl syndrome

Normal sleep patterns

a. Day-night reversals •are common in the first weeks of life. •The normal pattern is random sleep for 4 weeks, after which clustering of sleep time occurs. b. Sleeping through the night •is defined as sleeping more than 5 hours after midnight for a 4-week period. •Fifty percent of infants sleep through the night at 3 months of age.

Adaptive skills in blindness

a. Developing auditory perception skills b. Using haptic perception, (e.g., feeling someone's face to form a mental image of them by combining kinesthetic spatial feedback and input from tactile sensation)

Management of uncomplicated nocturnal enuresis

a. Education-demystification. uncover misconceptions, remove blame from the child, and explain anatomy and physiology. b. Conditioning alarms. requires patient motivation and parental support. This therapy must be used for a mini- mum of 3-5 months. c. Pharmacotherapy. Medications, if used alone, result in frequent relapses. (1) Desmopressin acetate (DDAVP)--relapse after medication is common (2) Tricyclic antidepressants: Imipramine is the most widely used agent. Patients often relapse after medication is discontinued, and there is a danger of fatal cardiac dysrhythmias with an overdose. d. Other management options. Behavioral modification (e.g., star charts, praise for dry nights, limited fluids before bed, voiding before bed),

Risk factors for suicide or depression

a. Family or peer conflicts b. Substance abuse c. Significant loss, including death of a loved one d. Divorce or separation of parents e. Poor school performance or learning disability f. Physical or sexual abuse g. Family history of depression or suicide h. Previous suicide attempt i. Physical illness

Phases of menstural cycle

a. Follicular (proliferative) phase •begins with menstrual flow and ends with ovulation (lasts 7-22 days) •pulsatile release of GnRH⇒ release of FSH and LH⇒ estradiol⇒ endometrial proliferation b. Ovulation phase •after LH surge⇒ruptured follicle develops to functioning corpus luteum c. Luteal (secretory) phase •begins with ovulation and ends with menstrual flow (lasts 12-16 days) •Progesterone ⇒ secretory endometrium ⇒ unfertilized corpus luteum involutes⇒ low progesterone and estradiol⇒ endometrial sloughing and GnRH release⇒ start cycle again

Enuresis etiology

a. Genetics. Strong familial tendency for nocturnal primary enuresis is supported by a gene identified on chromosome 13. b. Psychosocial. Secondary enuresis is often associated with stressful situations, such as the birth of a sibling, death of a family member, or the separation of parents. c. Chaotic social situation at home may contribute to poor voiding habits and daytime enuresis. d. Sleep-arousal mechanisms play an elusive role. Although not proven, parents frequently report children with nocturnal enuresis have deeper sleep and are more difficult to arouse than dry siblings. e. Urine volume. Some patients may produce large volumes of dilute urine, which may be caused by lack of normal diurnal variation in vasopressin release. f. Bladder capacity. Some children may have a small bladder capacity. g. Organic causes of secondary enuresis. Etiologic factors include urinary tract infections, child abuse, and diabetes mellitus. h. Constipation is a comorbid or etiologic factor in some patients. Hard stool can impinge on the bladder. Symptoms of encopresis may be present.

Why do 1/2 of sexually active adolescents not use contraception?

a. Ignorance of the contraceptive methods available b. Denial of the risk of pregnancy c. Barriers toward obtaining contraception, including issues of confidentiality and cost d. Refusal by partner to use contraception e. Religious beliefs f. Ambivalence to, or desire for, pregnancy

Risk factors for lead intoxication

a. Ingestion of lead-containing paint or putty from homes built before 1978 b. Drinking water from lead pipes or pipes with lead-containing solder c. Exposure to lead smelters or lead-painted commercial structures during demolition d. Use of lead-glazed pottery in food preparation e. Use of lead-containing folk remedies

When is jaundice suspicious for underlying pathology?

a. Jaundice appears at <24 hours of age. b. Bilirubin rises > 5-8 mg/dL in a 24-hour period. c. The rate of rise of bilirubin exceeds 0.5 mg/dL per hour (suggestive of hemolysis).

Normal menstrual cycle

a. Length of menstrual cycle: 21-35 days b. Duration of menstrual flow: 2-8 days c. Blood loss during menstruation: 30-80 mL

Family history suspicious for IEM includes:

a. Neonatal deaths in siblings or affected males on the maternal side b. Parental consanguinity c. Mental retardation or neurologic disability d. Unusual dietary preferences in relatives

Characteristic features of colic

a. Occurs in healthy, well-fed infants b. Begins at 2-4 weeks of age and resolves by 3-4 months of age c. Involves periods of irritability, which typically begin in late afternoon or early evening

Risk factors for iron-deficiency anemia

a. Prematurity b. Low birth weight c. Early introduction of cow's milk (before 9 months of age) d. Insufficient dietary intake of iron e. Low socioeconomic status

Classification of Adrenal insufficiency

a. Primary adrenal insufficiency b. Secondary adrenal insufficiency c. Congenital adrenal insufficiency includes CAH d. Acquired adrenal insufficiency includes Addison's disease and patients taking chronic steroids resulting in adrenal suppression.

Skeletal dysplasias: a. Rhizomelia b. Mesomelia c. Acromelia d. Spondylodysplasias

a. Rhizomelia: proximal long bone abnormalities (e.g., short humerus and femur). b. Mesomelia: medial long bone abnormalities (e.g., short ulna and tibia). c. Acromelia: distal abnormalities (e.g., small hands and feet). d. Spondylodysplasias involve abnormalities of the spine, with or without limb abnormalities.

Laryngotracheobronchitis (croup) management

a. Supportive care involves using cool mist and fluids/ cool night air b. Children with stridor at rest benefit from systemic corticosteroids e.g. IM dexamethasone, neb budesonide, or oral steroids which reduce airway edema c. Children with respiratory distress benefit from racemic epinephrine aerosols, vasoconstrict subglottic tissues d. B2-Agonists (e.g., albuterol)- wheezing e. Hospitalization is indicated for children in respiratory distress.

Leading causes of blindness in children

a. Trachoma infection is common in developing nations and is the primary cause of blindness worldwide. b. Retinopathy of prematurity c. Congenital cataracts

Abnormal sleep patterns

a. Trained night waking •occurs between 4 and 8 months of age when the infant does not resettle without parental intervention (e.g.,feedings, rocking, attention) during normal night stirrings and awakenings. •Management includes establishing routines and placing the infant in bed while drowsy but awake. b. Trained night feeding •occurs when the infant continues to wake to eat because the parents keep responding with a feeding. •Management includes lengthening intervals of daytime feeding and teaching parents not to respond with a feeding every time the infant stirs.

Management of colic

a. Treat any identified conditions. b. Reassure parents that their infant is healthy and that the colic is not the parents' fault. c. Recommend comfort measures, which may include decreased sensory stimulation (e.g., placing in front of a blank wall), increased sensory stimulation by movement or vibration (e.g., automobile rides, rocking, stroller rides), or positioning (e.g., swaddling or placing infant on side or stomach while awake).

Bronchiolitis management

a. Treatment is primarily supportive with nasal bulb suctioning, hydration, and oxygen as needed b. Careful handwashing c. Nebulized bronchodilators are controversial and may only be effective in up to 50%of patients d. Steroids are controversial (may be effective in prior h/o wheezing) e. Nebulized racemic epinephrine may be effective in reducing airway constriction f. Aerosolized ribavirin (in vitro activity against RSV, may be considered for very ill infants) g. Hospitalization is indicated for respiratory distress, hypoxemia, apnea, dehydration, or underlying cardiopulmonary disease. h. RSV monoclonal antibody (palivizumab) may be given prophylactically by monthly IM injection during RSV season

trichinella spiralis clinical pres

abdominal pain, N/V --> muscle invasion, edema of the eyelids, myalgia, weakness, fever, eosinophilia

renal dysplasia + clinical finding in utero

abnormal renal development due to in utero obstruction or developmental impairments -->defective concentration, RTA, renal insufficiency in utero see oligohydrominos--> pulmonary hypoplasia

prune belly syndrome

absense of rectus abdominus, bladder outlet obstruction, cryptochordism, rednal dysplasia/dysfunction---> OLIGOHYDRAMINOS--> lung hypoplasia, hydronephrosis,

thyroid dysgenesis

absent thyroid gland, some have ectopic thyroid that can be found between tongue and mid chest

how should vaccines be administered to a preterm infant

according to chronological age (after birth) not gestational age----- there is no significant reduction of immune response in preterm infants so vaccines are OK...... BUT NO HEPATITIS B until infant is <2kg***

Hydrocele + what condition are they associated with

accumulation of fluid through the tunica vaginalis into the testis , ~ ASSC with INDIRECT INGUINAL HERNIA

treatment of aspirin poisoning

acetazolamide (CA inhibitor) and sodium bicarb

1-5% sulfur used to treat

acne

acutane

acne treatment, highly teratogenic, women must be using birth control while on this drug

local side effects of steroids

acne, hirsutism, folliculitis, striae, hyper/hypopigmentation, atrophy, eccymosis/telangiectasias, tachyphylaxis

kawasaki disease 1. treatment acute/subacute/convalescent phase -consider steroid use in pts. refractory to IVIG 2. general JRA laboratory findings

acute = high dose IVIG (2g/kg), high dose aspirin subacute = low dose aspirin convalescent = low dose aspirin if aneurysms remain

waterhouse friedrichsen syndrome

acute adrenal hemorrhage, shock, DIC and MENINGOCOCAL SEPSIS***

telogen effluvium

acute stressful even --> forces hair to change from anaogen to telogen--> generalized hair loss (>100 hairs/day)

diseases that cause primary adrenal insufficiency

addisons CAH adrenoleukodystrophy (X linked)

acquired adrenal insufficiency pathogenssis

addisons chronic corticosteroid use

MCC viral myocarditis

adenovirus and coxsackie B virus

how to assess testicles ability to produce testosterone

administer B-HCG to stmulate the testicles to produce testosterone ~ this is a method to assess testicular function

what are the side effects of systemic steroids

adrenal suppression, depressed growth, cataracts, glaucoma, cushings syndrome

When does left or right hand dominance normally occur?

after 18 months of age

When does vision screening for ophthalmologic disorders begin?

after birth

juvenille rheumatoid arthritis diagnositic criteria

age of onset <16 arthritis >1 joint- swelling or effusion or limited motion, tenderness, increased warmth, >6 weeks duration exclusion of other causes

how to decrease the edema in a patient with nephrotic disease

albumin and a diuretic combination

venous hum: age, location, characteristic

any age but usually school age continuous murmor heard in the neck and below the clavicles, heard only when sitting or standing, disappears when supine, changes with compression of the jugular vein or with movment of the neck

what are the possibilities for the causes of a cold thyroid nodule

any cancer (anaplastic, folliculary, medullary) BUT A DYSGENIC THRYOID WILL NOT APPEAR COLD

what congenital heart diesease cause obstruction of flow

aortic/pulmonic/mitral stenosis, coarctation of the aorta, interrupted aortic arch, hypoplastic left heart syndrome

pseudostrabismus

apparant strabismus caused by prominent epicanthal folds

what is the most common clinical criteria in rhuematic fever

arthralgias

Metachromatic leukodystrophy

arylsulfatase A def --> accumulation of sulfatides --> ataxia, seizures, progressive mental retardation~ death by 10-20

demographics of IgA nephropathy

asia, australia, native americans

dermatomyositis complications

aspiration pneumonia (dec. gag reflex), intestinal perforation (GI vascultis), osteopenia(secondary to intestinal perforation)

which drugs inhibit platelet function

aspirin, valproic acid

traumatic hair loss

associated with anxiety

Ectodermal dysplasia

associated with conical-shaped teeth, dysmorphic facial features, decreased numbers of sweat glands, and alopecia

A infant has RSV, what condition is this patient now at a higher risk for

asthma

what are the causes of blood eosinophilia

asthma recrrent urticaria infantile eczema serum sickness helminth infections collagen vascular disease

what is the clinical associations with allergic rhinitis

asthma, chronic sinusitis, otitis media w/ effusion, nasal polyps, atopic dermatitis

non-cardiac causes of chest pain

asthma, esophagitis, costochondritis, anxiety, pneumothroax, pneumonia, sickle cell chest syndrome peptic ulcer

clinical presentation of a neonatal clavicular fracture + tx

asymmetric mororeflex, or pseudoparalysis, palpable crepitus over the fracture tx = sling 4-6 weeks for children, infants no treatment

clinical features of hepatitis** (infectious, autoimmune, drug induced)

asymptomatic jaundice, hepatosplenomegaly, ascites, increased abdominal vascular markings, caput medusae, spider hemangiomas, clubbing

clinical presentation of congenital hypothyroidism

asymptomatic at birth * T3 essential for brain development ~ prolonged jaundice/poor feeding, large tongue/posterior fontanelles, protruding tongue, umbilical hernia, myxedema, mottled skin, hypothermia, PERMANENT mental retardation *CRETINISM

acquired causes of hydrocephalous (list)

bacterial meningitis, brain tumors, intraventricular hemorrhage (preterm)

what is the major complication of laryngotracheobronchitis + clinical pres + tx

bacterial tracheitis presentation = several days of viral URI-->acute fever + respiratory distress tx = airway management

causes of acute hemorrhaggic cystitis

bacterial, viral (adenovirus) , chemotherapeutic (cyclophosphamide)

treatment for aortic stenosis

balloon valuloplasty palliative valvuloplasty, aortic valve replacement(using pts own pulmonary valve = Ross procedure) or using prosthetic valve

TB meningitis brain imaging results

basilar enhancement

what genetic condition is associated hepatoblastoma

beckwith wiedemann

what is the treatment for scoliosis before and after puberty growth spurt

before puberty growht spurt can treat with bracing or surgery after puberty growth spurt, bracing is not an option, however the scoliosis should not progress at this point

how best to establish rapport with an adolescen

begin history with non-threatening hobbies, interest or activities

diabetes insipidus effect on Na and K leveles

both go up due to hemoconcentration

how does T3/T4 circulate in the blood

bound to thyroid binding globulin, and thyroid binding pre-albumin

treatment for spondylolysis and spondylolithesis

bracing and surgery if bracing does not improve the condition

what is the initial sign of cerebral herniation

bradycardia***

how to evaluate the red reflex in an infant

bruckner test - direct ophthalmoscope from 2 feet away into the patients eyes

why is a corn rich diet more likely to lead to pellegra

corn is poor in tryptophan which leads to low niacin --> PELLEGRA

labs which should be ordered to evaluate hypocalcemia

ca, phos, mg, EKG (look for long QT), PTH level, vitamin D level (if BOTH Ca/phos are low), radiograph of wrists and knees

crest syndrome 1

calcinosis, raynauds, esophageal, sclerosis of the skin, telangiectasias less systemic involvement compared to scleroderma

which electrolyte is severely deficient in breast milk and poses risk for electrolyte imbalance in the neonate

calcium

MCC adult diarrhea in the united states

campylobacter

Anal patency

can be confirmed with careful introduction of either a soft rubber catheter or a rectal thermometer into the anus.

what fungal infections seen in HIV positive patients

candidal thrush/esophagitis cryptococcus pneumonia/meningitis histo, cocci, aspergillus

kernig sign, bruduzinski's sign

cannot extend the knee with the hip flexed flexing the neck causes hip flexion

Testotoxicosis

cause of PPP ~ testes enlarge bilaterally independent of HPGA

central vs peripheral hypotonia effect on DTRs

central increased DTRs, peripheral decreased`

diaphyseal fracture

central shaft fracture

what is the risk of correcting hypernatremia too quickly

cerebral edema, central pontine myelinolysis

Apnea of prematurity

cessation of breathing > 20seconds in premature infant as a result of immature respiratory center control

Spastic quadriplegia

characterized by motor involvement of the head, neck, and all four limbs.

Spastic hemiplegia

characterized by unilateral spastic motor weakness.

neonate with erbs or klumpkes palsy has tachypnia and respiratory distress- test + diagnosis

chest fluroscopy and ultrasound shows paralyzed diaphragm due to damage to damaged C3/C4/C5 phrenic nerve roots

When does Iron-Deficiency anemia most commonly occur?

children < 6 years of age, peaking between 9 and 15 months of age.

What age group is tuberculosis not contagious?

children <12 years old because their cough is minimal and their pulmonary lesions are usually small.

alpha 1 antitrypsin deficiency clinical presentation

cholestasis, bleeding into CNS/GI tract, or umbilical stump, elevated transaminatses, portal hypertension,

renal osteodystrophy

chronic renal failure --> secondary hyperparathyroidism + rickets + osteitis fibrosa cystica, 1,25(OH)D3 deficiency

hand/foot syndrome + dactylitis******

classic presentation of sickle cell disease, symmetric swelling of the hands and feet

poisonings that can cause fever

cocaine, TCA, phencyclidine, salicylates, thyroixine, anticholinergics, amphetamines, theophylline

Chlamydia trachomatis* pneumonia 1. clinical presenation 2. dx +CXR 3. tx

common cause of afebrile pneumonia at 1-3 months of age. (1) Symptoms include a staccato-type cough, dyspnea, and absence of fever. A history of conjunctivitis after birth may be identified in 50% of patients. Physical examination may demonstrate tachypnea and wheezing 2. EOSINOPHILIA**** CXR = interstitial infiltrates 3. erythromcyin or azithromycin

Extrapyramidal cerebral palsy

commonly referred to as athetoid cerebral palsy). These patients have problems modulating the control of the face, trunk, and extremities, often writhing Significant oral motor involvement often occurs.

patellofemoral stress syndrome

commonly seen in runners, pain when descending stairs

GM1 gangliosidoses type 1 patho + clinical presentation

complete absence of acid - beta galactosidase *******--> infantile onset hypotonia (compare to tay sachs) --> HSM, CNS degeneration, ant. breaks in cervical vertebrae, enlargement of the sella, thickening of calvarium (dystosis multiplex)

kostmann syndrome

congenital agranulocytosisis, frequent life threatening pyogenic infections, ANC <300

Laryngomalacia 1) pathogenesis 2) clinical presentation

congenital flaccid larynx appears as epiglottis rolling on laryngoscopy---> presents as inspiratory respiratory sounds that are improved when the child is prone ~~ approves with time as the child ages tx = feed the child while upright and keep the child upright for 30mins after feeds

most common metabolic disorder most common cause of acquired hypothyroisism most common cause of hyperthyroidism in childhood

congenital hypothyroidism hashimotos graves

longitudinal striations on the metaphysis TORCH infection

congenital rubella

osteochondriitis/periostitis TORCH infection

congenital syphillus

causes of red teary eyes in a newborn

conjunctivitis, glaucoma, dacryocystitis, enophthalmitis

what conditions cause short stature with delayed bone age

constitutional growth delay, hypothyroidism, hypercortisolism **NOT in genetic short stature, IUGR, turners, skeletal dysplasias, growth retardation ~~~ all of these the bone age is the same as the chronological age

how is low grade vesicoureteral reflux managed

daily antibiotics and urinalysis/culture every 3-4 months

bacterial meningitis complications

death 5-50% (gram neg>s.pneumo>HIB>neisseria) HEARING LOSS GLOBAL BRAIN INJURY SIADH, seizures, hydrocephalus, cranial nerve palsy, learning disability

propranolol effect on fetus

dec. heart rate, dec. ability to compensate during asphyxia, hypoglycemia/apnea

what is the clinical presentation of a tension pneuothorax + tx

dec. lung sounds + displaced heart sounds tx = immediate needle aspiration and chest tube placement

decerebrate vs decorticate postureing

decerebrate = extended arms and legs extremities- subcortical damage decorticate = flexion of arms and extension of legs indicates bilateral cortical injury

1. hypovolemic shock 2. what % of volume loss leads to inability of endogenous catecholamines to maintain blood pressure

decreased circulating volume ex. hemorrhage/dehydration 2. 25% ~~ now called decompensated shock

what antenatal and neonatal findings indicate hypotonia

decreased fetal movements weak breech presentation seizures in the neonatal period weak cry, decreaesd spontaneous movement, frog leg posturing, muscle contractures

how does maternal phenobarbital use effect neonatal jaundice

decreases jaundice because phenobarbital induced glucuronyl transferase

bartter syndrome

defect in Na/K/Cl cotransporter in thick ascending limb --> hypokalemia, alkalosis (inc. distal delivery of sodium), hyperaldosteronism, normotension, hyperreninemia

90% of viral meningitis

echovirus (enterovirus) and coxsackie

grade 1-5 vesicoureteral reflux

distal ureter extension to calyces extension to calyces with dilation clubbed calyces/greater dilation dilation of entire system,severe clubbin and tortuosity of the ureters

What is the side effect of metranidazole?

disulfiram like reaction if alcohol is consumed

how to know if deferoxamine is the right treatment for iron poisoning if serum iron is not known

do a test dose, if the urine turns a pink color, then the test is positive and deferoxamine should be continued

why do antipsychotics cause amenorrhea

dopamine antagonsits results in inc. prolactin which inhibits the HPG axis causing amenorrhea

causes and clinical presentation of airway compromize by a vascular ring

double aortic arches,right sided aorta, pulmonary sling etc -- symptoms worse when supine, relieved by neck extension, no response to bronchodilators/corticosteroids

chiari type 2 + other associated conditions

downward displacement of the cerebellum/medulla through the foramen magnum blocking CSF flow (-->hydrocephalous) + lumbosacral myelomeningocoele*** also assc- cervical hydrosyringomyelia, gyral anomalies, agenesis of the corpus callosum, orthoedic problems, genitourinary sx

what are the symptoms of polycythemia in an infant + MCC

due to hyperviscosity of blood: respiratory distress, seizures, cyanosis, jitteriness, irritability, poor feeding MCC = placental blood transfer due to delayed clamping of the cord

guillain barre dx/tx

dx - lumbar puncture shows albuminocytologic dissociation*** (inc. CSF protein*, but normal cell count) EMG - dec. conduction velocity spinal MRI to rule out compression Tx = IVIG, plasmaphoresis, steroids

dx/tx of SMA

dx = muscle biopsy no cure gastrotomy feeding physical therapy/surveillance for infections

tar used to treat

eczema, psoriasis

tick paralysis- pathogenesis + clinical presentation

eastern dog tick/rocky mountain wood tick--> toxin injection via saliva--> ataxia/areflexia--> prgressive ascending paralysis

what is the first step when an infant has fever >102.2, signs of septic shock, and a ***bulging fontanelle

empiric antibiotics first then CT scan (NOT LP FIRST) to see if there is a mass that will lead to herniation. If there is not then can proceed with the LP

pseudohypoparathyroidism (Albrights hereditary osteodystrophy)

end organ (bone/kidney) unresponsiveness --> short stature, short metacarpals, elevated PTH, delayed bone age, increased bone density, 4-5 digit brachydactly, obseity, subcapcuslar cataracts, periventricular/perivasclar/basal ganglia calcifications

Complications of Marfans

endocarditis, retinal detachment, sudden cardiac death aortic root dilation (1)HTN and chest trauma increase dissection risk (2)Endocarditis prophylaxis and regular ophthalmologic examinations are warranted

rachitic rosary

enlargment costochondral junctions seen in vitamin D deficient rickets

hirschberg test

evaluate symmetry of light off the patients corneas

cushing 'disease'

excess *********ACTH production by pituitary tumor

what causes brachial plexus injury during birth

excess traction on neonates head/neck/arm which stretches the brachial plexus

truncus arteriosus pathophysiology

excessive blood flow to the lungs leads to CHF

schwachman diamond syndrome

exocrine pancreatic insufficeincy (chronic diarrhea), short stature, (metaphyseal chondroplasia) neutropenia

clinical presentation of hyperthyroidism

exophthalmos, smooth* goiter, tachycardia/palpatations, flushing of skin, nervousness, fine tremors, fatigue, delayed menarche, gynecomastia, delayed puberty

anterior shoulder dislocation

ext. rotation, abduction and extension of the shoulder~~~ occurs in gymnastics/wrestling recurrance is 90%

phenothiazine toxicity symptoms + tx

extrapyramidal symptoms, oculogyric crisis, tremors, torticollis, opisthotonus, dysphagia tx = DIPHENHYDRAMINE

bacteremia in an infant clinical presentation

fever, left shift + leukocytosis, headache, malaise, poor feeding

transient tic disorder

eye blinking, facial movements, throat clearing, resolves in about a year

renal agenesis

failure of metanephritc blastema or mesonephric duct to develop~ can be uni/bilateral

what is the major cause of clavicular fractures in childhood and neonates

falling ont the shoulder, and birth injury in neonates

incontinentia pigmenti

familial skin blistering and seizures

hepatitis E infection

fecal oral , common in developing countries (50% of all cases), ***** 20% MORTALITY IN PREGNANT WOMEN**** NO CHRONIC DISEASE IgM/IgG anti HEV diagnostic

fetomaternal transfusion

fetal blood loss to mother during birth, occurs during normal/abnormal pregnancies

clinical presentation of bacterial meningities in an older child

fever and signs of meningeal irritation altered LOC, nuchal rigidity, kernigs (painful flexion of the leg with the hip at 90'/brudzinskis sign (pressure on the cheek causes flexion of arms and hips) seizures, photophobia, emesis, headache

1. Clinical presentation of pericarditis 2. physical exam findings 3. diagnosis 4. ECG findings 5. CXR findings 6. tx

fever, dyspnea, CHEST PAIN MOST INTENSE WHILE SUPINE,RELIEVED WHILE UPRIGHT 2. pericardial friction rub, distant heart sounds, PULSUS PARADOXUS, hepatomegaly 3. any child with fever/dyspnea + recent history of heart surgery, dx with PERICARDIOCENTESIS, ESR, echocardiogram 4. diffuse ST elevation and low voltage QRS complexes 5. large heart shadow 6. antibiotics, anti inflammatory ages such as aspirin or steroids, drainage

clinical features of infectious endocarditis

fever, non-specicifc new murmur hematuria (embolism or glomerulonephritis) splinter hemorrhages retinal hemorrhages osler nodes (swollen tender lesions on palms/soles) janeway lesions - small hemorrhageic lesions on the palms/soles roths spots - round or oval white spots on the retina

lead poisoning diagnosis in a child

first fingerstick blood lead level >10ug/dL confirm with serum lead level >10ug/dL

congenital rubella syndrome (CRS)

first trimester rubella infection --> 30-50% CRS blueberry muffin rash (purpura) congenital cataracts PDA, microcephaly sensorinerual hearing loss meningoencephalitis thrombocytopenia, hepatosplenomegaly, jaundice, purpura Late = mental retardation, type 1 diabetes, hypertension, autoimmune thyroid disease

how to remove a foreign body from a childs vagina

first try warm water irrigation to flush it out if this doesnt work remove it under general anesthesia

what vitamin is critical for pregnant mothers to take to prevent spina bifida/neural tube defects

folate

treatment for tricuspid atresia procedure

fontan procedure- flow from the inferior vena cava is directed into the pulmonary arteries by means of extracardiac conduit Glenn shunt - SVC anastomosed is placed **goal is systemic venous return to the pulmonary artery

Hips examination

for developmental dysplasia of the hips

galeazzi fracture

fracture of the radius with distal radioulnar joint dislocation

hematemisis vs hematochezia vs. melena

fresh red blood from the mouth (upper GI bleeding) vs the rectum (lower GI bleeding) melena = dark tarry stools upper GI bleed proximal to ligament of treitz

lap belt injury

from MVA chance fracture (flexion disruption of lumbar spine) liver/spleen laceration, kidney damage bowel perforation

when do sphenoid and frontal sinuses become large enough to harbor infections

frontal = 6th-10th year of life sphenoid = 5th year of life

two galactose related metabolic disorders

galactose 1 - phosphate uridyl transferase deficiency (severe hepatomegaly, mental retardation, vomitting, diarrhea) galactokinase - mild (just cataracts)

breastfeeding contraindications

galactosemia/PKU/urea cycle defects maternal use of antimetabolits, chemotherapy, radioisotopes illicit drug use HSV (w/ breast lesions), active TB**, HIV

treatment of iron poisoning

gastric lavage ACTIVATED CHARCOAL DOESNT HELP treat hypovolemia, blood loss, shock intravenous deferoxamine if iron >500ug/dL or >300ug/dl + acidosis, hyperglycemia, or leukocytosis

salicylate poisoning treatment

gastric lavage, activated charcoal serumsalicylate 6 hours after injection and plot on a done nomograph, alkalinization of the urine with sodium bicarbonate, dialysis

treatment of acetaminophen poisoning

gastric lavage, activated charcoal, serum acetaminophen 2-4 hours after ingestion plot on matthew rumack monogram to determine hepatitis potential *N-ACETYLCYSTINE 140mg/kg loading dose then 70mg/kg every 4 hours for 17 doses

what drugs cause fanconis syndrome

gentamicin, OUTDATED tetracycine, cephalothin (1st gen cephalo), cidofovir (anti-CMV), valproic acid, streptozocin, 6-MP, azathioprine (6-MP prodrug), cisplatin, ifosfamide, heavy metals, paraquat, maleic acid, glue sniffing (toluene)

what diseases have UDP glucuronyl transferase deficiency

gilberts syndrome, crigler najjar type 1 (AR) - 90% of enzyme funciton deficient crigler najjar type 2 (AD) ~ 100% of enzyme function

riboflavin deficiency clinical presentation

glossitis, seborrheic dermtitits (of groin), chelosis, pharyngitis, edema/erythema of the mouth

what is the major risk in 46XY turner females

gonadoblastoma--- must do bilateral gonadectomy

VACTERL (VATER) association clinical features

group of malformations that occur sporadically (1) V—vertebral defects (2) A—anal atresia (3) C—cardiac anomalies,predominantly ventricular septal defects (4) TE-tracheoesophageal fistula (5) R—renal and genital defects (6) L—limb defects, including radial hypoplasia, syndactyly and polydactyly

stool guiac test + what causes false positive vs false negative

guiac is a colorless dye that changes color from peroxidase activity of hemoglobin in the presence of hydrogen peroxide false positives= ingested iron, red meats, beets, cantaloupe, broccoli, cauliflower false negative = large doses of vitamin C

traction alopecia

hair loss assoicated with hair bands, find patchy areas of alopecia

type 1 polyglandular syndomre

hashimotos + type 1 diabetes + addisons

clinical presentation of hydrocephalous in infnats

head circumference >97% for age, large anterior/posterior fontanelles with split sutures sunset sign - downward deviation of both eyes (from pressure of third ventricle on superior colliculus)

clinical presentation of hyper tension in children

headache, dizziness, visual disturbance, irritability, nocturnal wakening

what people are more likely to have latex allergy

healthcare workers, and patients with myelomeningocoele (due to multiple catheterizatoins)

transient tachypnea of the newborn

healthy infant, normal birth, --> respiratory distress and inc. pulmonary vascular markings with fluid fissures and hyperexpansion tx= oxygen to maintain oxygen saturation

what patients with VSD should get surgical closure

heart failure refractory to medical treatment large VSD with pulmonary hypertension small to moderate VSDs (at age 2-6)

laboratory studies for upper GI bleeding

hemoglobin, platelet counts, coagulation studies, serum transalminases, BUN (ELEVATED WITH UPPER GI BLEED),

what conditions have a high reticulocyte count

hemolytic anemia, sickle cell anemia (increased marrow production)

membranous nephropathy - progresses to renal insuficiency 1. associaation

hepatatits B

causes of direct hyperbilirubinemia in an infant

hepatitis, biliary atresia, choledochal cyst (abdominal ultrasound, radionucleotide imaging, liver biopsy)

clinical presentation of systemic venous congestion (right heart failure)

hepatomegaly, peripheral edema

what neonatal history accompanies tricuspid regurgitation

history of asphyixia or other heart defects

treatment for urolithiasis

hydration, abx for struvite UTI related stone

congenital toxocplasmosis clinical presentation

hydrocephalus, intracranial calcifications, chorioretinitis

What is the most common abdominal mass in both male and female newborns

hydronephrosis **** others include ovarian cysts, wilms tumor, multicystic kidneys,

1. treatment for carbon monoxide poisoning 2. when should patient with carbon monoxide poisoning be hospitalized

hyperbaric oxygen 2. CO -Hb >25%, neurologic symptoms, metabolic acidosis or ECG changes

what electrolyte imbalance is seen in an immobilized fracture of a weight bearing limb (aka. IMMOBILIZATION HYPERCALCEMIA) + tx

hypercalcemia --> high BP, altered mental statuis, encephalopathy, convulsions 2. diet restriction of dairy, diuretics, calcicitonin

what electrolyte imbalance associated with RTA in the blood / urine

hyperchloremic metabolic acidosis with a normal serum anion gap ******** positive urine anion gap in the urine (Na + K - Cl)

what metabolic diseases cause neutropenia

hyperglycine*mia, methylmalonic acidemia, gauchers disease

essential hypretension secondary hypretension

hypertension with no known etiology (rare) hypertension with a etiology (most common)

1. 11 B hydroxylase deficiency clinical presentation 2. lab diagnosis

hypertensive and hyperkalemic (because 11-deoxycorticosterone has some mineralocorticoid activity) + ambig genetalia in females 2. elevated 11-deoxycortisol

what are the causes of spherocytes

hyperthermia hereditary spherocytosis G6PD deficiency ABO incompatibility (NOT RH INCOMPATIBILITY)

what are the manifestations of polycythemia in a SGA infant + tx

hyperviscosity syndrome - seizure, tremulousness, jitteriness--> seizure activity due to slow blood in the brain + renal vein thrombosis, NEC, tachypnea tx = partial exchange transfusion

list of seizure causes

hypocalcemia, hypoglycemia, hypomagnesemia, hyper/hyponatremia, pryixoxine deficeincy, contusion, subdural hematoma astrocytoma, meningioma amphetamines, cocaine meningitis, encephalitis, brain abscess, neruocysticercosis cerebral infarction, intracerebral hemorrhage

child just had a seizure and mom says she dilutes the fomrula

hyponatremia from too much water

type 2 polyglandular syndrome (schmidt syndrome)

hypoparathyroidism, chronic mucocutaneous candidiasis, + addisons

dandy-walker malformation

hypoplastic cerebellar vermis (truncal ataxia), 4th ventrile enlargement blocks CSF flow (-->hydrocephalous)

conditions that can cause hair loss

hypothyroidism, diabetes, hypopituitarism, hypervitaminosis A, zinc deficiency, marasmus, warfarin, heparin, chemotherpay,

hypotonia vs weakness

hypotonia = dec. RESISTANCE during passive stretching weakness = decreased force on active contraction

Most common cause of hypercortisolism

iatrogenic ~ ie. giving steroids to patients

1. clinical presentation of brain tumors

inc intracranial pressure --> worse when supine during sleep/awakening: headache, vomiting, drowsiness, irritability, abnormal behavior, ataxia (cerebellar), seizures (supratentorial), head tilt enlarged/bulging fontanelles, nystagmus, papilledema, 6th nerve palsy, lethargy/irritability

pathogenesis + clinical presentation of severe pulmonary stenosis

inc. PVR ==> shunting of blood through the patent forament ovale leading to right to left shunt and cyanosis

1,25 vitamin D effect

inc. reabsorbtion of phos/Ca2+ at kidney inc. release of Ca/phos from bones ***inc. reabsorbtion of Ca through the intestines

what is the hematologic finding in SGA infants

inc. risk of hypoxia/asphyxia/MAS--> polycythemia ~ also see hypothermia, hypoglycemia, hypocalcemia

why does pulmonary hypertension occur in VSD

incrased blood flow from left to right

treatment of breastfeeding jaundice

increase feeding frequency and duration to increase breast milk production which will reduce dehydration and reduce transit time and dec. enterohepatic circulation

how should maintenance fluid be adjusted for a patient with fever

increase maintenance fluids by 12% for every degree above 38'c

how does cutaneous absorbtion of substances differ in a preterm infant

increased absorbtion through the skin

clinical presentation of hydrocephalous in older children

increased intracranial pressure 1headache, nausea, unilateral 6th nerve palsy, papilledema,brisk DTRs + downward plantar response

pseudotumor cerebri

increased intracranial pressure with normal with normal brain anatomy (mimics a tumor) caused by imparied reabsorbtion of CSF---- symptom = headache, complication is optic atrophy/blindness tx = serial spinal taps, acetazolomide, steroids

what is the risk of cryptorchid testis

increased risk of testicular cancer

how does a neonate adapt to a cold environment, what are the consequences if the neonates is not brought to a warmer ambient temperature

increasing metabolic rate, increasing heat production from brown fat if they are not brought to warmer temperature, lactate will accumultae and resultating metabolic acidosis and compensatory hyperventilation

treatment of PDA

indomethacin

how much protein is required in the diet of an infant vs adult

infant 2.2g/kg 0.8 g/kg

neonatal abstinence syndrome

infant born to heroin/methadone addicted mother sneezing, tachypnea, irritability, poor feeding/sleeping, vomitting, diarrhea,

pyelonephritis clinical presentation

infants - nonspecific fever, lethargy irritability children - back/flank pain, high fever, vomiting, dehydration

what infants are at high risk for renal vein thrombosis

infants of diabetic mothers

causes of red eye in older infants/children

infectious, allergic, contact lens, corneal abrasion, bacterial corneal ulcer, HSV keratitis,

lichen planus

intensely pruritic raised papular rash is found on flexoral surfaces of the wrists, forearms, inner thighs,

Why give a misbehaving child time out, how long

interrupt misbehavior to give child time to think about behavior 1 minute/year of age to a maximum of 5 minutes

what interventional strategies for heart failure in children/infants

interventional catheterization (baloon valvuloplasty) surgical repair

Persistent neonatal hypoglycemia 1. causes

islet cell hyperplasia (nesidioblastosis) beckwith widemann (visceromegaly, hemihypertrophy, macroglossia, umbilical hernias, distinctive ear creases) von gierke, galactosemia, maple syrup urine disease, tyrosinemia, growth hormone deficiency congenital hypopituitarism

why is the bladder succeptible to rupture in children who have blunt trauma

it is an abdominal organ in a child

how does IVIG work to treat ITP

it saturates the RES binding sites for platelet bound self immunoglobulin

iron poisoning parthenogenesis + tx

kid eats ferrous sulfate tablets, or prenatal vitamins--> GI tract damage -->hemorrhage, hepatic injury/necrosis, pooling of blood and fluids in GI tract (+hematemesis)/pleural/peritoneal cavity (Third spacing), interference with ox phos causes lactic acidosis + tx = ipecac and deferoxamine

1. what is most common developmental delay domain 2. what is the best indicator for intellectual potential

language language

language vs speech

language = abiltiy to communicate with symbols speech = vocal expression of language

What risk factor increases clavicle fractures in neonates?

large birth weight

kasabach-merritt syndrome

large hemangiomas --> MAHA, thrombocytopenia, DIC

takayasu arteritis

large vessel vascultis with aneurysmal dilation of the aorta, carotid, and subclavian artiers~~ seen in asian adolescents/young adults

congenital syphilis clinical presentation

lesions on palms/soles, jaundice, anemia, RHINORRHEA, periostitis, metaphyseal dystrophy, Hutchinson teeth, saddle nose,

clinical presentation of renal failure

lethargy, N/V, respiratory distress, hypretension, seizures, OLIGURIC

clinical presentation of hydrocephalous in a neonate

lethargy, prominent scalp viens, DOWNWARD GAZE, tense fontaneles, poor suckling, hypotonia

causes of supraclavicular lymphoadenopathy

leukemia, lymphoma, metastatic disease

what drugs are contraindicated during breast feeding

lithium, cyclosporine, anti-neoplastics, ergotamines, bromocriptine, illicit drugs

side effects of anabolic steroids

liver damage, dec. testicular size, oligosperrmia, aggression, mood swings, dec HDL, premature closure of the epiphysis

treatment for biliary atresia

liver transplant

Volvulus

loops of intestine twist if attached to a narrow band of mesentery ⇒ restricted circulation to the rotated (or obstructed) segment⇒ intestinal gangrene

vitiligo + tx

loss of skin pigmentation tx = psoralen + UV light

hypomagnesemia related hypocalcemia

low magnesium interferes with PTH release

what risk factors increase the probability that a person will abuse a child

low self esteem, social isolation, depression, history of substance abuse history of child abuse history of mental illness history of violent temperament single parenthood, unemployment, poverty, marital conflict

pseudohypocalcemia

low serum albumin in nephrotic syndrome looks like low total calcium levels

The first tooth is generally a:

lower central incisor

labs + imaging which must be tested for a suspected case of bacterial menintgitis

lumbar pucture with culture blood culture CT with contrast (to find brain abscess)

wolman syndrome ~patho + clinical pres

lysosomal acid lipase deficiency --> calcification of the adrenal gland (KUB) HSM, diarrhea, vomitting, FTT,

Clinical presentation of mucopolysaccharidoses

lysosomal storage disorders --> organomegaly, short stature, MR, DYSOSTOSIS MULTIPLEX - thickened cranium, J shaped sella, ovoid or beak like vertebrae, short/thickened clavicles, dolichocephaly

tinea corporis,/pedis/cruris 1. pathogenesis + clinical pres 2. dx/tx

m.canis, t.tonsurans, other trichophyton corporis = oval/circular scaly erythematous patches with partial clearing pedis = scaling/erythema of the toes/plantar foot cruris = scales/erythema in the groin/inguin 2. KOH skin scrapings, topical antifungals (clotrimazole, terbinafine, ketoconazole

what is a patients total body fluid requirement

maintenence (sensible losses + insensible losses) + ongoing losses

treament of beta thalassemia major/minor

major - splenectomy, lifelong transfusions, desferoxamine (tx. for hemochromatosis) minor - NO IRON SUPPLEMENTATION (often misdiagnosed as IDA)

What self exam instructions should be given to adolescent male/female?

male = testicular exam female = breast exam

How does puberty begin in males vs females

males begins with testicular enlargment (next is pubic hair, facial and axilary hair is 18-24 months after pubic hair) females begins with breast enalrgerment (next is pubic hair, menarche is 2-3 years after thelarche)

Bladder extrophy pathogenesis

malformation: failure of infraumbilical mesenchyme to migrate and form the lower abdominal wall

anemia of chronic disease

malig/infection/kidney disease/lead poisoing-->microcytic hypochromic anemia hypochormic microcytic anemia, low iron, low TIBC

homocystinuria clinical presentation + tx

marphans like appearance, downward subluxation of the lens, and tx = B6

inspissated bile syndrome

massive hemolysis ex. large hematoma or ABO incompatability -->

when should VZIG be given

maternal onset of varicella within 5 days prior to delivery, or if onset is <2 days after delivery ~ outside of this time frame, infants have no higher risk of complicatiosn than older children

maternal autoimmune thyroid disease effect on the fetus

matrnal anti-thyroid IgG cross placenta and destroy fetal thyroid with anti- TPO, anti-thyroglobulin, anti-TSH-R

what does bone pain indicate in a child with cancer

metastatic disease, primary tumor of bone/connective tissue, or leukemic infiltration

GERD clinical presentation in older children

mid epigastric pain that is relieved with food or antiacids, exacerbated by fatty foods, caffeine, and supine position

describe the arthritis of rheumatic fever

migratory polyarthritis effecting mostly the LARGE JOINTS, pain and swelling of one joint subcides as it begins in another joint *Fever/carditis may or may not be present at this time SPECC - subcutaneous nodules, polyarthreitis, erythema nodosum, chorea, carditis

what are three levels of dehydration

mild (3-5%), moderate (7-10%), severe (>12%)

MELAS 1. patho + clinical pres

mitochondrial disorder ~ Mitochondrial encephalopathy, lactic acidosis, stroke like episodes

Kearns sayre syndrome 1. patho + clinical pres

mitochondrial disorder ~ ophthalmoplegia, pigmentary degeneration of retina, hearing loss, heart block, neuro degeneration

what vaginal disease in girls predisposes to type 1 diabetes

monilial vulvovaginitis

medulloblastoma spinal tap CSF findings

monocytic** pleocytosis

morbilliform rash vs scarlatiniform rash

morbilliform = measles like scarlatiniform = papular, vesicular, petechial

what is the management of MOST kidney injuries with the exception of traumatic vascular/ traumatic hydrocoeles

most = observation vascular/hydrocoeles =

multicystic dysplastic kidney

most common abdominal mass in newborns associated with atretic ureter

cubitus varus

movent of limb to midline after healing of a supracondylar fracture due to poor positioning of the distal fragment

treatment of impetigo

mupirocin--- b/c it covers gram positives as well as MRSA

what is an "innocent murmur"

murmors from turbulent flow and NOT structural heart disease~~ seen in 50% of children

which spinal defect requires immediate surgical repair

myelomeningocoele

list some mitochondrial disorders

myoclonic epilepsy and ragged red fibers MELAS lebers hereditary optic neuropathy

wegeners granulomatosis

necrotizing granulomas of the upper/lower respiratory tract and kidneys---- sinusitis, hemoptysis, glomerulonephritis

what age in childhood has the highest risk for diabetes inspidus

neonatal period

clinical presentation of sever aortic stenosis in neonate and older children

neonate: normal appearing neonate at birth becomes cyanotic at 12-24 hours and develops CHF as the ductus arteriosus closes older children: exercise intolerance, chest pain, syncope, sudden death

NSAIDS effect on the kidney

nephotic syndrome and interstitial nephritis

leukocyte adhesion deficiency clinical presentation

neutrophilia, delayed seperation of the umbilical cord, recurrent bacterial infections, gingivitis/peridontitis--> LOSS OF TEETH

should ipecac be given for hydrocarbon ingestions

no becuase of the risk of aspiration and subsequent necrotizing pneumonia ~~ so - no ipecac for corrosives and hydrocarbons (ex. mineral oil)

what are the causes of central cyanosis (non cardiac and cardiac)

non cardiac: pulmonary, sepsis, hypoglycemia, polycythemia, neuromuscular disease, impaired chest wall cardiac: the 5Ts

acid base disturbance in diarrhea

non-anion gap hyperchloremic metabolic acidosis ~~ due to loss of bicarbonate

Lab findings of fatty acid oxidation defects

non-ketotic hypoglycemia, hyperammonemia, myopathy, cardiomyopathy

clinical features of infection in young infants

non-specific = fever, dec. appetitie, irritability, cough, rhinorrhea, vomitting, diarrhea

what is the clinical presentation of a patient with a severe coarctation

normal appearing infant that develops cyanosis and CHF symptoms as the PDA closes -->weak pulses in all extremities, and loss of the murmur

what type of anemia in renal failure

normochromic normocytic anemia due to dec. EPO

anemia of prematurity

normochromic normocytic anemia, due to dec. production, shorter half life of RBCs

slipped capital femoral epiphysis 1. pathogenesis/clinical pres 2. dx/tx

obese* adolescent adult boy/hypothyroidism* --> slipping of femoral head off femoral neck--> painful limp, decreased internal rotation/abduction of the hip-->avascular necrosis of femoral head/chondryolysis/limb length discrepancy/osteoarthritis 2. radiograph shows klein line (line drawn from lateral surface of femoral neck doesnt cross epiphysis) Tx = pinning the epiphysis to prevent slippage

Omphalocele 1. Definition 2. associated congenital anomalies (compare with gastroschisis)

occurs in approximately 1:6,000-8,000 live births. 1. true hernia through the umbilical ring, covered with peritoneal sac 2. ToF, ASD, Beckwith-Wiedemann syndrome, chromosomal disorders e.g. trisomy 13

MAC clinical presetnation

often seen in HIV positive patients with CD4<50 fever, weight loss, night sweats, abdominal pain, bone marrow suppression, inc. AST/ALT

incomplete fracture (aka greenstick) + treatement

one side of the cortex fractured with the other side intact tx = fracture other side of cortex and reduce

what fraction of hepatitis A infections in children have syymptoms

only 30%

Hearing impairment or a communication disorder

only language skills are delayed

Visual or fine motor problems that interfere with manipulative tasks

only problem-solving skills are delayed

is premature adrenarche, thelarche, or pubarche concerning

only pubarche is concerning because this is associated with CNS disorder

open vs closed fracture

open = break in the skin closed = skin intact

treatment of acyanotic congenital heart disease

open heart surgery

treatment for iron deficiency in an infant between the ages of 9 and 15 months

oral ferrous sulfate ~~ expect to see reticulocytosis and rise in hemoglobin/hematocrit

orbital vs periorbital (pre-septal) cellultitis

orbital cellulitis- shows proptosis, pain, ophthalmoplegia, and dec. visual acuity periorbital cellulitis- has full range of motion and no effect on vision

what does pulse oximetry measure

oxygen saturation SaO2

what blood gas values does oxygenation vs ventilation refer to***

oxygenation = SaO2 ventilation = PCO2

treatment for sickle cell bone crisis (vasoocclusive crisis)

pain control + high volume 1.5-2x maintenance IV fluids

Henoch Schonlein purpura treatment

pain control, hydration, steroids

what physical exam finding should be taught to parents whose children have sickle cell disease

palpable spleen, to detect ACUTE SPLENIC SEQUESTRATION which can lead to hypovolemia/circulatory collapse

What do discrepancies between verbal and non-verbal skills on standardized intelligence tests in school age children suggest?

possible learning disability

todds paralysis

post focal or jacksonian seizure, lasts only 24-48 hours

complications of strep impetigo

post strep glomerulonephritis staph scalded skin syndrome bacteremia NOT RHEUMATIC FEVER****

which post strep infection complications are not prevented by antibiotics

post-strep glomerulonephritis post strep arthritis

what condiditons can patients under age of 18 consult for without a parent

pregnancy related care, dx/tx of STDs, reproductive health care, counseling and treament of drug/alcochol problems, mental health treatment

digestion

process of breaking down foods using mechanical breakdown, digestive enzymes and bile acids

Why does prolactinoma cause amenorrhea?

prolactin inhibits HPG axis and dec GnRH

clinical features of WPW

prolonged PR SVT delta wave

hirschprung disease clinical presentation + diagnosis

prolonged constipation from birth, FAILURE TO PASS MECONIUM and signs of enterocolitis (late) barium enema and manometry

treatment for coarctation of the aorta

prostaglandin E keeps the PDA open, inotropic medications keep cardiac output sufficeint to perfuse the kidneys, surgery, balloon angioplasty for recurrent COARCTATION IS COMMON************ IN THESE PTS)

guilllan barre spinal tap findings

protein HIGH (2x) glucose normal cell counts normal

signs of rapid onset of precocious puberty and androgen excess

pseudo puberty - independent of of the HPG axis ~~~ ex. late onset CAH, exogenous androgens, or androgen secreting tumor

what is the early signs of myasthenia gravis + what are the EMG signs

ptosis, weakness of extraocular muscles, dysphagia and facial muscle weakness, rapid muscle fatiguing EMG = decremental response during rapid muscle stimulation

how to evalulate for rickets

radiographs of wrists/knees

how to diagnose meckles diverticulum

radionucleotide scan

scabies pathogenesis + clinical presentation + tx

sarcoptes scabiei var hominins pruritic threadlike burrows in interdigital areas, groin, elbows, ankles, palms/soles, NO FACE/HEAD infants present with bullae/pustules tx = 5% permethrin cream

lab studies for poisoning

screening- serum glucse, serum/urine toxicology screen, electrolytes/anion gap

Trisomy 18

second most common trisomy syndrome and is three times more common in females. 1. mental retardation and hypertonia (with scissoring), small facial features, clenched hands with overlapping digits, rocker bottom feet ~ death by first year

orthostatic proteinuria + dx

seen in athletic individuals have increased protein when upright, but not supine dx = normal TP/CR in the morning, but elevated in the afternoon

sprengels deformitiy

seen in klippel feil syndrome scapula is rotated laterally leading to shoulder asymmetry and dec. shoulder motion

what are the general non-specific signs/symptoms of poisoning *calculate potential poison dose as WORST CASE****

seizures, vomiting/diarrhea, dysrhythmias, altered mental status, abnormal behaviors, shock, trauma, metabolic acidosis

what history suggests FFR (inorganic constipation)

sentinal event after which bowel change occured - change in psychosocial environement, inappropriate toilet trianing, abuse

what are the most common complications of parenteral feeding

sepsis MCC thrombosis of central lines pneumothorax brachial plexus injury REVERSIBLE mucosal atrophy

1. hemorrhagic disease of the newborn 2. dx/tx

serious bleeding from vitamin K deficiency - presents only with cutaneous bleeding and bleeding from circumcision site or umbilical cord 2. dx = prlonged PT and PTT tx = intramuscular vitamin K at birth***, FFP, platelets

lab evaluation of acute renal failure

serum electrolytes, BUN, creatinine, urinalysis, urinary protein, renal/pelvic ultrasound, nuclear renal scan

parvo b19 infection of pregnant mother effect on the fetus

severe anemia, hydrops fetalis, and death of the fetus

scorpion sting clinical rpesentation

shock, salivation, convulsions tx - phenobarbital (not morphine), and antivenin

sjogrens syndrome

sicca sydnrome (dry eyes and mouth), high titers of autoantibodies, connective tissue disease

pityriasis alba

similar to atopic dermatitis, hypopigmented dry scaly patches on the cheeks

extracranial headache causes

sinusitis, perioral abscess, toothache, chronic otitis, refractive errors

What skills are delayed in mental retardation?

skills are delayed significantly in both language and problem- solving domains

Cognitive development involves

skills in thinking, memory, learning, and problem solving.

somniloquy

sleeptalking, can occur in any phase of sleep

how should needle be advanced in spinal tap of an infant + what is the risk of using no styloid

small advances followed by removing the styloid no styloid can introduce a core of epidermis into the subarachnoid space which can then form a epidermoid tumor

polyarteritis nodosa

small and medium size vessel aneurysms, associate with hepatitis B

sebaceous nevi

small sharply edged slightly raised yellow-orange hairless lesions that occur on the head and neck

1. what is the relationship between the size of the VSD and the intensity of the murmur 2. what kind of murmur from a moderate size VSD 3.what kind of murmur + symptoms from a large VSD

smaller size of VSD causes a more intense murmur 2. holosystolic, but because 2x the ammount of blood goes to lungs --> mitral valve there is also a diastolic murmur of mitral turbulence at the apex 3. shorter/lower pitch systolic murmur, with a mitral filling rumble at the apex ~~ present with signs of CHF

clinical features of allergic rhinitis

sneezing, nasal congestion, rhinorrhea, pale nasal mucosa allergic shiners- dark circles under eyes dennies lines - creases under eyes from chronic edema allergic salute - use palm of hand to elevate tip of nose to help itching

tricyclic antidepressant overdose treatment

sodium bicarbonate, helps to normalize the wide QRS seen overdose

early morning headache and vomiting in a child

space occupying tumor in the CNS

what make spinal cord injury difficult to diagnose

spinal cord injury without radiographic abnormality

treatment of forearm fractures

splint for 4-7 days until swelling resolves, then cast for 6-8 weeks

ann arbor staging system of lymphomas

stage 1 = single lymph node or extra lymphatic site stage 2 = 2 or more node regions, same side of diaphragm, extension to extralymphatic site or extra nodal site + one lymph node same side of diaphragm stage 3 = nodes both side of diaphragm (spleen counts) stage 4 = diffuse disseminated of one or more extralymphatic organs/tissues A = asymptomatic, B is symptomatic*****

what are the causes of post-infectious glomerulonephritis

strep A, HIV, hepatitis B

spondylolysis + clinical presentation

stress fracture of pars interarticularis from repetitive hyper-extension of the spine most commonly in L5 ~~ pain with hyperextension

what kind of fractures lead to compartment syndrome

supracondylar fractures of the radius and humerus

1. neuroblastoma treatment 2. what are poor prognostic features of neuroblastoma

surgery alone for stage I/II chemotherapy for metastatic disease (IV/IVs) radiation for advanced diseaes ****IVs may spontaneously regress 2. N-myc III/IV, amplification of N-myc (chromo 2) diploidy, high levels of ferritin, lactic dehydrogenase, neuron specific enolase

what are the causes of upper GI bleeding in a child

swallowing maternal blood during delivery, or epistaxis, gastric ulcers (stress, burns, H.pylori), mechanical injyr (mallory weiss tears, foreign body/caustic ingestion, varicies (rare)

what conditions cause chorea

syndhams chorea encephalitis kernicterus SLE huntingtons disease wilsons disease

denys drash

syndrome associated with wilms tumor, mesangial sclerosis/renal failure, pseudohermaphrodism

what are the two broad causes of hypotonia

systemic pathologies - sepsis/meningitis, electrolytes, hepatorenal abnormalities etc neurological pathologies - damage to the neurons (central or peripheral)

clincal presentation of CHF

tachypnea, cough, wheezing, rales CXR = pulmonary edema tachycardia, sweating, pale or ashen skin, decreased urine output, enlarged cardiac silouette FTT, poor feeding (infants), exercise intolerance

what is the potentially life threatening consequence of lobar pneumonia (staph) + treatment

tension pneumothorax + treatment = chest tube or needle aspiration at the 2nd or 3rd intercostal space

guthrie test

test that detects metabolic products in the urine ex. PKU

What is the secondary survey in pediatric emergency

• full history and physical • head to toe examinations

What is the most common cause of airway obstruction + how to fix this in a patient during CPR

the tongue--> fix using the head tilt method, or the jaw thrust method if suspect cervical spine injury

Periodic breathing

three or more respiratory pauses at least 3 seconds each with less than 20 seconds in between

complicatiosn of polycythemia

thrombosis (vasoocclusive, stroke, MI), bleeding

pendred syndrome

thyroid organification defect + sensorineural hearing loss ~~ most common cause of thyroid dyshormonogenesis

for a neonate with jaundance, lethargy, distended abdomen, dec. ins/outs what should be initial treatment

thyroixine - 10-15mg/kg

chronic non-specific diarrhea

toddlers diarrhea-- diarrhea with normal growth and physical examination-- no treatment neccessary

somogyi phenomenon

too high evening insulin dose --> hypoglycemia in the morning--> epi/glucagon-->ketones + hyperglycemia ~~ TX BY LOWERING THE BEDTIME INSULIN

what is the major risk associated with cryptochordism in a child vs adult

torsion of the testicle because testicle is not fixed to the scrotal sac. in adulthood major risk is testicular cancer, this IS NOT DECREASED BY ORCHIOPEXY it just makes the testicles easier to examine~ thus surgical intervention does not decrease the risk of testuclar torsion

PTU use during pregnancy effect on teh fetus

transient hypothyroidism

neonatal myasthenia

transient myasthenia from transplacental transfer of maternal antibodies to the fetus

honeymoon period after starting treatment for diabetes

transient recovery of residual islet function decreases insulin need ~ islets temporarily respond to carbohydrate load

what does positive fluorescein staining indicate in the eye

trauma, bacterial corneal ulcer, HSV keratitis

child with nephrotic syndrome has chest pain, fever what should be done

treat for pneumococcal pneumonia empircilly and obtain blood cultures, urine cultures, chest radiograph

drugs that cause nephrotic syndrome

trimethadione, penicillamine**, catopril, probenicid, ethosuximide, methimazole, lithium, procainamide, chlorpropamide, phenytoin, paramethadione, tolbutamide, NSAIDs, gold, mercury

Rocker bottom feet

trisomy 18 (Edward's)

how to treat a patient with croup whose O2 sat is falling

try inhaled epinephrine , if this does not work then intubate

what factors decrease risk of vertical transmission of HIV

undetectable viral load cesarean section post exposure prophylaxis of the infant + mother being adherent to treatment

1. cluster headaches 2. tx

unilateral frontal or facial pain + conjunctival erythema, lacrimation, nasal congestion last less then 30 minutes but recur several times a day then go away for several months tx = sumatriptan, prophylactic CCB/valproate

multicystic renal dysplasia 1. clinical presentaiton - most common renal mass in a newborn

unilateral renal mass

peritonsillar abscess clinical presentation

unilateral tonsillar swelling and uvular deviation

neotnatal tetanus pathogenesis and clinical presentation

unimmunized mother/unclean obstetric techniques outside hospital leads to umbilical stump infection with tetanus --> erythematous umbilical stump, spasms, opisthotonus, poor feeding

causes of acquired torticollis + first diagnostic test

upper respiratory infections, cervical lymph adenitis, peritonsillar/retropharyngeal abscess, cervical diskitis, osteomyelitis, neoplasms, strabismus, refractive errors, CERVICAL VERTEBRAE FRACTURE**** first test = xray of the neck

what metabolic conditions effect platelet funciton

uremia, and liver disease

where can obstructions distal to the collecting duct occur

ureteropelvic junction ureterovesicular junction posteriour urethral valves prune belly syndrome (bladder outlet)

UTI diagnosis UTI treatment

urinalysis shows >5-10 WBC/high powered field, positive nitirite/leukocyte esterase, urine culture shows >10000 colonies by urethral cath urine shows >50000 colonies by clean catch tx = TMPSMX, cephalexin toxic appearing- IV antibiotics neonates - ampicillin/gentamycin

laboratory evaluation for suspected glomerulonephritis

urinalysis, urinary TP/CR, blood chemistries (electrolytes/BUN/creatinine/serum albumin/liverenzymes/cholesterol), serum complement, antibody testing (ANA, ASO, anti-DNase B), IgA level ~ can also consider hep C/B titers, HIV testing

serum sickness

urticara + systemic signs (fever/adenopathy) after medications

Developmental quotients

used to determine whether a child's development is delayed and to measure the extent of delay. a. DQ > 85: normal b. DQ < 70: abnormal c. DQ 70-85: close follow-up is warranted

KOH

used to identify fungal hyphae

woods light

used to identify pigmentary changes + some dermatophytes

absorbtion

uses intestinal mucosal surface and villous brush border for transport of macronutirent monomers and micronutrients

What fine motor skills are learned in the second year of life?

using tools

non-pharmacological treamtnet for epilepsy

vagal nerve stimulator, ketogenic diet (suppresses seizure activity)

miller fisher syndrome

varient of guillian barre -- ophthalmoplegia, ataxia, areflexia

what is angioedema

vascular reaction of the dermis with localized edema from dilted capillaries characterized by giant wheals

sulfonamides effect on the kidney

vasculitis and nephritis

behcet syndrome

vasculitis of small/medium sized arteries non-destructive arthritis of multiple joints, fever, erythema nodosum, aphthous stomatitis, uveiits, CNS (incl pseudotumor cerebri)

treatment of hydrocephalous + complications

ventriculoperitoneal shunt diverts flunt of CSF --- complication = shunt infection/obstruction

causes of benign transient proteinuria

vigorous exercise, fever, dehydration, CHF

potential environmental triggers for diabetes

viral infections (coxsackie and rubella), cows milk (maybe)

tx. for viral and TB meningitis

viral is self limiited TB- 4 drug TB treatment + corticosteroids

CSF shows lymphocytic pleocytosis, elevated protein, lowered glucose

viral meningitis,

what is a common cause of acquired CHF in older children/adolescents

viral myocarditis (coxsackie B)

which vitamin should be given to children with measels

vitamin A, decreaes measels releated mortality

scurvy

vitamin C deficiency --> weakness of collagen especially in blood vessels, poor wound healing, diffuse tenderness in the legs, petechiae/ecchymosis (fragility of BV)

what vitamins are inadequate in breast milk

vitamin D

which hormones release calcium from the bones

vitamin D/PTH

what skin manifestation indicates polyglandular autoimmune disease

vitiligo or alopecia

Hypercortisolism short stature

• most common cause is iatrogenic, due to prolonged use of steroids •leads to poor growth and increasing weight gain, purpuric stretch marks and a dorsal neck fat pad on examination, and delayed bone age

When do the male and female gonads become differentiated?

week 9

when does the lower esophageal spincter normally relax

when food is being pushed by peristalitic contraction down the esophagus

clinical findings of PDA

wide pulse pressure (accentuated peripheral pulses), and continuous murmor

aniridia in an infant is associated with what syndomre

wilms tumor, aniridia, genitourinary nomalies, retardation , HEMIHYPERTROPHY WAGR complex

hypertension in a child with cancer

wilms tumor, pheochromocytoma, neuroblastoma

prophylactic treatment for infants born to HIV positive mothers

zidovudine for 6 weeks post exposure TMPSMX for PCP NO BREASTFEEDING urine CMV culture

acute infantile hemiplegia

~ DOES NOT NECESSARILY OCCUR ONLY IN INFANTS thrombotic occlusion of the MCA or branches --> classic hemi-syndrome with eyes looking away from the paralyzed side

treatment for hypocalcemia

~ treat when Ca2+ <8 mg/dl oral (mild) intravenous calcium gluconate (symptomatic) 1,25 vitamin D

when is dialysis started for ARF how long can a kidney graft last after transplant

~ when GFR is 5-10% of normal usually peritoneal dialysis ~ 5 years

Cause and effect

• 9-15 months • understanding which actions cause certain results (e.g., learning that dropping toys over the high chair tray makes them fall to the floor).

Acute Wheezing diagnosis

• Asthma • Hypersensitivity reaction, including anaphylaxis • Bronchiolitis • Pneumonia • Foreign body aspiration • Acute aspiration of stomach contents • Environmental irritants

Plagiocephaly 1. definition 2. clinical features

• Asymmetry of the infant head shape usually not associated with premature suture closure Clinical features: •Most common type of plagiocephaly is POSITIONAL plagiocephaly, associated with flattening of the occiput and prominence of the ipsilateral frontal area •May be associated with congenital muscular torticollis •Incidence has increased as a result of infant sleeping on their back (trying to avoid SIDS)

Sensorimotor period

• Birth to 2 years • learning to manipulate --> manipulate to learn~ time of physical manipulation/inspection of objects e.g. putting object in mouth for oral exploration

Complications from circumcision

• Bleeding • Infection • Poor cosmesis • Phimosis (secondary to insufficient foreskin removal) • urinary retention • injury to the glans or urethra **Repeat circumcision is needed in 10%.

Cerebral palsy

• Cerebral palsy is a group of static (i.e., nonprogressive) encephalopathies caused by injury to the developing brain in which motor function is primarily affected. • Intelligence may be normal, but injuries to the brain that cause cerebral palsy often lead to other neurologic effects, including seizures, cognitive deficits, mental retardation, learning disabilities, sensory loss, and visual and auditory deficits. The basis of diagnosis is repeated neurodevelopmental examinations

Craniosynotosis 3. diagnosis 4. management

• Diagnosis is made by physical examination of the head. • Craniosynostosis is usually noted by 6 months of age. • The diagnosis is confirmed by skull radiographs and head CT scan. Management is surgical repair, most often indicated when cosmetic concerns are significant

Failure to thrive

• Growth rate of less than expected for a child (most commonly weight) cross >2 major percentile bars = MAJOR CONCERN 2. HEAD CIRCUMFERENCE

Hep A vaccine 1. timing

• Hepatitis A is the most common viral cause of hepatitis worldwide •It is asymptomatic in up to 70% of infected children younger than 6 years of age. • Inactivated vaccine recommended at 2 years or older with booster 6 months later for susceptible groups (high hep A communities and travelers to endemic areas)

Functional intestinal obstruction

• Hirschsprung's disease • Hypoplastic left colon • Neuronal dysplasias or muscular diseases

Etiologies of FTT

• Inorganic or psychosocial (most common) • Organic Evaluation of FTT requires a careful history and physical examination, a complete dietary history, and observation of the parent-child interaction.

Immunization precautions: 1. all vaccines 2. DTaP 3. MMR and varicella (live)

• Moderate to severe illness (with or without fever). **Note that mild illnesses, including febrile illnesses, are not contraindications to immunization. • DTaP vaccine (1) Temperature of 40.5°C within 48 hours after prior vaccination (2) Collapse or shocklike state within 48 hours after prior vaccination (3) Seizures within 3 days after prior vaccination (4) Persistent, inconsolable crying lasting ≥ 3 hours occurring within 48 hours after prior vaccination • IVIG administration in the preceeding 3-11 months

Why is universal newborn hearing screening carried out?

• Moderate-to-profound hearing loss in early infancy is associated with impaired language development. • Early detection and intervention for hearing loss may improve speech and language acquisition.

Developmental domains

• Motor development • Language skills • Cognitive development • Social skills Infant development progresses from cephalic to caudal; from proximal to distal; and from stimulus-based reflexes to specific, goal-oriented reactions that become increasingly precise with age

Contraindications to circumcision

• Penile abnormalities (ex. hypospadias) • Prematurity • Bleeding disorders

Early dental eruption

• Primary eruption before 3 months of age Causes include: • familial • hyperthyroidism • precocious puberty • growth hormone excess

Delayed dental eruption

• Primary eruption occurring after 16 months of age Causes include: • familial • hypothyroidism • hypopituitarism • genetic syndromes such as Down syndrome • ectodermal dysplasia

Plagiocephaly management

• Range of motion exercises for associated torticollis • Repositioning the head during sleep • Helmet therapy • Increased time in the prone position when awake ("tummy time").

Cholesterol and Lipid Screening

• Routine screening not recommended • Screening is recommended for children >age 2 with a family hx of hypercholesterolemia, hyperlipidemia, or early MI •Children with elevated cholesterol levels (75th-90th percentile) should have a fasting lipid panel that includes total cholesterol, triglycerides, high-density lipoprotein (HDL), and low-density lipoprotein (LDL).

Respiratory distress syndrome

• Termed hyaline membrane disease/ surfactant deficiency syndrome in preterm infants •Termed meconium aspiration syndrome (MAS) and persistent pulmonary hypertension of the newborn (PPHN)in full-term infants

Inorganic failure to thrive

• a disturbed parent-child bond that results in inadequate caloric intake or retention

Abstract thinking

• adolescent years • manipulating concepts and contingencies

Stage of functional play

• begins at about 1 year • child recognizes objects and associates them with their function ~ playing with toy telephone

Stage of imaginative play

• begins when the child is able to use symbols (24-30 months) • child uses blocks to build forts, or sticks as eating utensils or guns

McCune-Albright clinical presentation

• bony changes (polyostotic fibrous dysplasia) •skin findings ("coast of Maine" cafe-au-lait spots) •endocrinopathies (PPP/hyperthyroidism/hyperadrenalism) •enlarged gonads

Most common cause of pneumonia in older children/adolescents

• chlamydia pneumoniae • mycoplasma pneumoniae

Prevnar 1. prevnar components 2. disadvantage 3. timing

• conjugated polysaccharide composed of seven pneumococcal serotypes • does not confer as broad coverage against pneumococcal strains as Pneumovax. • 2,4,6 months with booster at 12-15 months children • children > 2yo with high risk for pneumococcal disease

Object permanence

• develops at about 9 months • concept that people and objects continue to exist even when an infant cannot see them •As a result of this ability to maintain an image of a person, separation anxiety (common at 6-18 months) develops when a loved one leaves the room.

Well Child Screening

• focus is to identify undetected problems and to identify the risks of such problems Screening assessments include: • complete history and physical examination • growth measurements • blood pressure measurements • strabismus and vision screening • hearing screening • tuberculosis screening • laboratory screening

Lead intoxication (plumbism)

• public health risk among children < age 5 • Children < age 6 are most susceptible to the effects of lead • Up to 4% of all children in the United States have evidence of increased lead absorption, including up to 20% of inner city children.

Mental retardation

• significantly subaverage general intellectual functioning associated with deficits in adaptive behavior, such as self-care, social skills, work, and leisure. • It is manifested before 18 years of age Classification and diagnosis. Using the Wechsler Intelligence Scale for Children or another appropriate psychometric measure of the intelligence quotient (IQ)

Treatment of shock

• supplemental oxygen • early endotracheal intubation • 20ml/kg fluid resuscitation with normal saline or ringers lactate • inotropes (dobutamine) after intravascular volume is restored • broad spectrum abx (septic shock) • broad pectrum ABX, blood products

Organic failure to thrive

• underlying organ system pathology, infection, chromosomal disorders, or systemic illness Evaluation of potential organic etiologies will be directed by the timing or onset of FTT, i.e., prenatal onset of inadequate weight gain (as in intrauterine growth retardation) should be distinguished from postnatal onset of inadequate weight gain.

Milia

• very small cysts formed around the pilosebaceous follicles, which appear as tiny, whitish papules that are seen over the nose, cheeks, forehead, and chin. •usually disappear within a few weeks and do not require treatment

Down syndrome triple screen

• ↓ AFP • ↑ β-hCG • ↓ unconjugated estriol

Mixed gonadal dysgenesis

•45XO/46XY •most present with ambiguous genitalia and a testis and vas deferens on one side and a "streak gonad" on the contralateral side •Fallopian tubes may also be present bilaterally despite the presence of a testis

True hermaphrodites

•46XX or 46XY •ambiguous genitalia with both ovarian and testicular gonadal tissue

Partial androgen insensitivity

•46XY •Have partial or incomplete peripheral androgen resistance resulting in defective androgen binding in genital tissue

Testicular femininzation syndrome

•46XY with no response to testosterone **EXTREME ANDROGEN INSENSITIVITY ~~ these individuals have phenotypic female external genitalia, with internal testes and no mullerian structures, no wolffian structures

Age of onset male puberty +first sign

•9-14 years of age •testicular enlargement (>4 mL)

Causes of obesity

•95% - interaction among genetics + inc. caloric intake, dec. energy expenditure, poor eating behaviors •5% - underlying endocrinologic or genetic causes: cushings, hypogonadism, hypothyroidism, prader willi

What are the three markers in triple screen?

•AFP •Unconjugated estriol •β-HCG

Cystinuria

•AR disorder caused by a defect in renal reabsorption of cystine, lysine, arginine, and ornithine (COLA) that leads to renal stones. •Clinical features may include UTIs, dysuria, abdominal or back pain, urgency, and urinary frequency.

Hartnup disease

•AR disorder caused by a defect in the transport of neutral amino acids. •Most patients are asymptomatic but some may present with intermittent ataxia, photosensitive rash, mental retardation, and emotional lability.

Holt-Oram syndrome

•Also called heart-hand syndrome •Autosomal dominant •Absent radius bone in the arm/abnormally developed bones in upper limbs •ASD/VSD, and first degree heart block

Necrotizing enterocolitis 1. what type of infants most often get this 2. clinical presentation + x ray 3. treatment

•An incidence as high as 8-10% in infants < 30 weeks gestation 1. preterm neonates 2. abdominal distension /tenderness, residual gastric contents, bilious aspirate, bloody stools, abdominal erythema, metabolic acidosis, oliguria •Plain film abdominal xray = PNEUMATOSIS INTESTINALIS (air in bowel wall), dilated bowel loops, venous portal gas 3. bowel rest, gastric decompression, no oral feeding, parenteral fluids/nutrition, resection via surgery

Angelman syndrome vs Prader-Willi syndrome

•Angelman - mother passes on abnormal chromosome 15 - •Prader-Willi - passes on an abnormal 11q region on chromosome 15

Management of diurnal enuresis

•Bladder stretching exercises, if small bladder capacity is suggested •schedule timed voiding every 90-120 minutes •treatment of any coexisting constipation may be effective.

Definition of delayed puberty in boys/girls

•Boys = no testicular enlargement by 14 years of age •Girls = no breast tissue by 13 years of age, or no menarche by 14 years of age

Evaluation of delayed puberty

•CBC •ESR •T4 •Testosterone or estradiol •FSH and LH •Prolactin level •Bone age

Cause of abnormal red reflex

•Cataracts •Glaucoma •Retinoblastoma •Severe chorioretinitis

Dysfunctions of Attention (Attention Deficit/Hyperactivity Disorder, ADHD)

•Characteristics of ADHD include poor selective attention, difficulty focusing, or distractibility. •Hyperactivity may or may not be present •ADHD is more common in boys. •Children may show impulsivity, distractibility, disinhibition, and general behavioral immaturity. •Genetic factors play a large role •Abnormalities in neurotransmitter function, especially dopamine and norepinephrine, also lead to symptoms.

Type I IUGR

•Chromosomal anomalies (trisomies) •Fetal infections (TORCH) •Maternal drugs (chronic alcoholism, heroin) •Maternal chronic illness (HTN, severe DM)

Colic

•Colic is crying that lasts > 3 hours per day and occurs > 3 days per week. (Normal crying usually lasts up to 2 hours/day at 2 weeks of age and increases to 3 hours/day at 3 months.) •Colic is significant because it has the potential to disrupt attachment between infant and parents, and it can be a source of family stress. •Colic occurs in approximately 10%of newborns.

Differential Diagnosis of ADHD

•Hearing or vision deficits •Sleep disorders, including obstructive sleep apnea •Food reactions •Thyroid disease •Anemia •Toxins or heavy metal exposure (e.g., lead poisoning) •Anxiety •Depression •Bipolar disorder •Mental retardation •Specific learning disabilities •Medication side effects (e.g., albuterol, methylphenidate, anticonvulsants, decongestants, antihistamines) •Family dysfunction •Normal child, but parents have unreasonable expectations for age or developmental stage •Caffeinated beverages

Beckwith-Wiedemann syndrome

•Hemi-hypertrophy •Visceromegaly •Macroglossia

How to treat IEM associated hyperammonemia and acidosis?

•Hyperammonemia -sodium benzoate -sodium phenylacetate -neosporin and lactulose prevent bacterial ammonemia in the colon •Acidosis - sodium bicarbonate -dialysis

What conditions cause diminished glucose production or substrate supply?

•IUGR and preterm infants with limited hepatic glycogen stores and poorly developed gluconeogenesis •stressed infants who have been asphyxiated or with sepsis •infants with inborn errors of metabolism such as galactosemia, hereditary fructose intolerance, and aminoacidopathies • infants with endocrinopathies such as growth hormone deficiency and panhypopituitarism

Type II IUGR

•Inadequate intrauterine space (multiple pregnancies, uterine tumors, uterine anomalies) •Placental insufficiency from maternal vascular disease (renal failure, chronic essential hypertension, collagen vascular diseases, pregnancy-induced hypertension) •Small placenta with abnormal cellularity

Hirschsprung disease. or congenital aganglionic bowel disease 1. pathogenesis 2. dx 3. management

•Incidence is approximately 1:5,000 live births. 1. lack of caudal migration of the ganglion cells from neural crest⇒ contraction of distal segment of colon, causing obstruction with proximal dilation 2. rectal biopsy showing lack of ganglion cells 3. resection of affected segment or colostomy

Kallman syndrome

•Isolated gonadotropin deficiency associated with anosmia

Sleep Problems

•It is estimated that more than half of infants experience sleep problems as perceived by parents.

Treatment of M.tuberculosis

•Latent tuberculosis - isoniazid for 9 months + B6 to prevent neurologic complications of INH •TB disease - 2 months of INH, rifampin, pyrazinamide then 4 months of INH and rifampin

Causes for increased weight and LGA

•Maternal diabetes •Beckwith-Wiedemann syndrome •Prader-Willi syndrome •Nesidioblastosis (diffuse proliferation of pancreatic islet cells).

Pierre Robin syndrome

•Micrognathia •Cleft palate •Glossoptosis (downward displacement or retraction of the tongue) •Obstruction of the upper airway

Premature adrenarche

•More common in girls • occurs after 5 years of age •Onset of pubic, axillary hair, and apocrine odor without development of breast tissue or clitoromegaly

Smokeless tobacco use

•More than 3 million teens chew smokeless tobacco. •Smokeless tobacco is associated with oral cancers, gingival recession, and low birth weight and premature delivery in mothers who use smokeless tobacco during pregnancy.

Intestinal atresia

•Most common cause of intestinal obstruction in the neonatal period •can occur in the small or large bowel.

Categorization of short stature

•Normal variant short stature -Familial short stature -Constitutional short stature •Pathologic short stature -Proportionate short stature -Disproportionate short stature

Lawrence-Moon-Biedl syndrome

•Obesity •Retinitis pigmentosa •Hypogonadism •Polysyndactyly

Differential Diagnosis of Direct Hyperbilirubinemia

•Obstructive jaundice secondary to choledochal cyst •Obstructive jaundice secondary to biliary atresia •Sepsis •Neonatal hepatitis •Metabolic causes (galactosemia, hereditary fructose intolerance, tyrosinemia, α1−antitrypsin deficiency) •Cholestasis associated with parenteral nutrition •Cystic fibrosis

Choanal atresia 1) tx 2) dx

•Occluded nasal passages, this is a problem in neonates because of obligate nose breathing clinical presentation = child that becomes cyanotic during feeding that improves with crying •Caused by failure of posterior nasal passage to canalize completely, leaving either a bony (90%) or membranous (10%) obstruction 1) tx= PASS NG TUBE, definitive tx is repairing with surgery or endoscopy 2) failure to pass a catheter through the nares into the oropharynx, confirmed by CT scan

Nursing or bottle caries

•Occurs in 3-6% of children and is most often seen in the child at 24-30 months of age Most frequently associated with: • a history of falling asleep with a nipple (breast or bottle) in the mouth •children who breastfeed excessively or who carry around a bottle as a habit. •Streptococcus mutans is the most common bacterial agent Caries involve the maxillary incisors, canines, and primary first molars. The lower teeth are spared initially because they are covered by the tongue. •Management may include placement of dental crowns or extraction.

Clinical problems of small-for-gestational-age infants

•Perinatal asphyxia •Hypothermia •Hypoglycemia •Polycythemia •Thrombocytopenia •Hypocalcemia •Meconium aspiration syndrome •Intrauterine fetal death •Hypermagnesemia (if mother is treated with magnesium for hypertension or preterm labor)

Hearing Impairment

•Permanent hearing loss occurs in at least 1:600 newborns •Half these infants are normal newborns who have no obvious evidence of suspected hearing impairment. •Early identification of the hearing-impaired child is vital because out-come is demonstrably better if intervention occurs before 6 months of age. •Sequelae of late identification include delayed speech and language skills and academic and behavior problems. •Genetic factors account for at least 80%of childhood hearing impairment; •80% of genetic transmission occurs by autosomal recessive inheritance.

Post-term delivery

•birth >42 weeks gestation •complications include increased death from placental insufficiency including: intrauterine asphyxia, meconium aspiration syndrome, polycythemia

Differential Diagnosis of Indirect Hyperbilirubinemia

•Physiologic jaundice •Increased RBC load from bruising •Increased bilirubin from hemolysis •Upper GI obstruction (inc. enterohepatic recirculation) •Breastfeeding jaundice •Breast milk jaundice •Sepsis •Inborn errors of metabolism (e.g., hypothyroidism) •Inherited disorders of bilirubin uptake and conjugation (gilbert's and crigler-najjar)

Type III IUGR

•Placental infarct or fibrosis •Maternal malnutrition •Pregnancy-induced hypertension •Maternal hypoxemia (lung disease, smoking)

Complications of LGA infants

•Polycythemia •Hypoglycemia •Congenital malformations

Inorganic causes of FTT

•Poor formula preparation •Poor feeding techniques •Child abuse and neglect •Parental immaturity •Maternal depression •Alcohol or drug use •Marital discord •Mental illness •Family violence •Poverty •Isolation from support systems

Craniosynotosis 1. definition 2. etiology

•Premature closure of one or more of the cranial sutures •Etiology is often unknown. 80 to 90% of cases are sporadic and 10 to 20% are familial or a part of a genetic syndrome (e.g., Crouzon and Apert syndromes). • Known etiologies or risk factors include intrauterine constraint or crowding and metabolic abnormalities, including hyperthyroidism and hypercalcemia.

Development of the male internal/external genetalia

•SRY gene causes differentiation into testes which produce AMH which causes involution of the müllerian structures and leydig cells produce testosterone which develops the wolffian system •external genitalia are formed by the conversion of testosterone to dihydrotestosterone by 5 alpha reductase ~ finished by 12 weeks

Clinical features of microcephaly

•Small brain •Developmental delay and intellectual impairment •Cerebral palsy or seizures

Clinical features of respiratory distress syndrome

•Tachypnea •Decreased air entry or gas exchange •Retractions (intercostal, subcostal, or suprasternal) •Grunting •Stridor •Flaring of the alae nasi •Cyanosis.

what vaccines for the adolescent

•Td booster@ 11 or 12 years of age, and every 10 years after this •Varicella vaccine if haven't had chicken pox •If not given earlier: give MMR and hep B series

Newborn Skin Examination

•Texture differs with gestational age; •skin is softer and thinner in premature infants.

Lanugo

•Thin hair that covers the skin of PRETERM infants •It is minimally present in term infants.

Epidemiology of anorexia/bulimia

•Typically occur in females 13-18 years of age. •0.5-1% of adolescents have anorexia •1-5% have bulimia

Diseases for which passive immunity can be administered

•Varicella zoster immune globulin (VZIG) for immunocompromised patients who have been exposed to varicella and are at high risk for severe varicella infection. •HBIG - Newborns born to hepatitis B-positive mothers receive hepatitis B immune globulin at birth. •HAIG- Visitors to high-risk areas may receive hepatitis A immune globulin before travel.

Premature thelarche

•Very common and benign condition •First 2 years of life •Transient activation of the HPGA axis --> palpable breast tissue, no other secondary sex characteristics

Measures of growth used for a child

•Weight, height, head circumference (until 2 years of age) • Sexual maturity Standardized growth curves represent normal values for age for 95% of children and are used to plot weight, height, body mass index, and head circumference.

Turner syndrome

•XO paternal non-dysjunction •2. short, webed neck, shield chest, swelling of dorsum of hands and feet, ovarian dysgenesis/delayed puberty, COARCTATION OF THE AORTA.

Undervirilized male

•a male pseudohermaphrodite, who is usually a genetic 46,XY with ambiguous genitalia and one or both testes palpable;

Management of cyanosis

•administration of oxygen •rapid correction of abnormalities of temperature, hematocrit, and glucose and calcium levels. In severely cyanotic infants: •intubation and mechanical ventilation may be necessary until a final diagnosis is made and definitive treatment is initiated

Jaundice

•always abnormal if detected within the first 24 hours of birth •frequently seen during the first few days after birth but usually is not associated with serious disease

Why does B-HCG tumors cause PPP?

•are unique to boys; found in the chest, pineal gland, gonad, or liver (hepatoblastoma) •B-HCG has some LH like activity which causes the testes to enlarge and Leydig cells to produce androgesn

Neonatal torticollis

•asymmetric shortening of the sternocleidomas- toid muscle •may result from being in a fixed position in utero or from a postnatal hematoma resulting from birth injury

Strawberry hemangioma

•benign proliferative vascular tumors occurring in approximately 10% of infants •Often first noticed a few days after birth, they increase in size after birth and usually resolve within 18-24 months •Hemangiomas that compromise the airway or vision require intervention.

Erythema toxicum neonatorum

•benign rash seen most frequently in the first 72 hours after birth •characterized by erythematous macules, papules, and pustules (resembling "flea bites") on the trunk and extremities but not on the palms and soles •occurs in about 50% of full-term infants and is found much less frequently in preterm infants •Lesions are filled with eosinophils. •No treatment is required.

Pustular melanosis

•benign transient rash characterized by small, dry superficial vesicles over a dark macular base •more frequently seen in African American infants •must be differentiated from viral infections, such as herpes simplex, and from bacterial infections, such as impetigo

When is Universal screening of hemoglobin levels recommended?

•between 9 and 15 months of age and between 4 and 6 years of age.

Persistent urachus 1)pathogenesis 2)clinical presentation

•complete failure of the urachal duct to close •results in a fistula between the bladder and the umbilicus •may present with urine draining from the umbilicus, especially when pressure is applied over the bladder

Pneumovax 1. pneumovax components 2. disadvantage 3. indications in children

•composed of polysaccharide capsule antigens from 23 serotypes covering almost ALL bacteremia/meningitis causing strains • little immunogenicity in children younger than 2 years old • primarily for older children and adults at high risk for pneumococcal disease (e.g., patients with sickle cell anemia who are functionally asplenic, immunodeficiency, chronic liver disease and nephrotic syndrome, and patients with anatomic asplenia).

Nevus flammeus or port wine stain

•congenital vascular malformation composed of dilated capillary-like vessels (a form of capillary hemangioma) •may be located over the face or trunk and may become darker with increasing postnatal age •Those located in the area of the ophthalmic branch of the trigeminal nerve (cranial nerve V-1) may be associated with intracranial or spinal vascular malformations, seizures, and intracranial calcifications (Sturge-Weber syndrome).

Intrauterine devices (IUD) 1. mechanism 2. advantages 3. disadvantages

•copper - impedes sperm transport and motility •progesterone - induces endometrial atrophy 3.convenience/ privacy 4. NO STD PROTECTION, uterine bleeding and cramping, higher initial cost, inc. RISK OF PID

Acrocyanosis

•cyanosis of the hands and feet frequent in first 48-72 hours •in some infants it can last throughout the first month of life,particularly when the infant is cold

Mongolian spots

•dark blue hyperpigmented macules over the lumbosacral area and buttocks of no pathologic significance •most frequently seen in Hispanic, Asian, and African American infants

Effects of blindness on development

•delayed locomotion •decreased fine motor skills •difficulties with attachment

Caput succedaneum

•diffuse edema or swelling of the soft tissue of the scalp •crosses the cranial sutures and usually the midline.

Clefts of the lip and of the soft and hard palate

•easily noted by inspection •submucous clefts in the soft portion of the palate should be ruled out by digital palpation •These clefts may be isolated or associated with other dysmorphic features.

Hypergonadotrophic hypogonadism 1. labs

•end-organ dysfunction (ie. gonadal failure)--> rising FSH/LH, low testosterone/estradiol •There is no abnormality in the hypothalamus or pituitary gland.

Two causes of normal variant short stature

•familial short stature •constitutional growth delay -- delayed puberty

Binge drinking

•five or more consecutive drinks at one sitting •As many as 50% of all college students report binge drinking •binge drinkers are more likely to fight, drive drunk, and have un- planned sexual intercourse.

what special things should be looked for in the adolescent physical exam

•height/weight •BP, vision, and hearing •acne/fungal infections of the skin •oral hygene •enlargement/nodules of the thyroid •scoliosis/kyophosis •tanner rating •male genetalia for hernias/scrotal masses •female complete pelvic exam should be performed annually if patient is sexually active, or if hx of pelvic pain, discharge of abnormal bleeding or if 18 or older

What labs for adolescent screening (non sex related)

•hemoglobin/hematocrit to screen for anemia •urinalysis - for proteinuria/hematuria •cholesterol/fasting lipid panel •HIV if indicated •PPD - at least once in adolescence

What are the abdominal masses in the neonate?

•hydronephrosis (most common) •multicystic kidneys •ovarian cysts

Constitutional delay of puberty

•immature hypothalamus or "late bloomers" •more common in boys •often there is a family history in one parent e.g. growth spurt late in college or high school •Frequently associated with constitutional delay of growth

Phimosis

•inability to retract the foreskin •normal up to age 6 but is always abnormal if ballooning of the foreskin occurs during urination.

Hypogonadotropic hypogonadism 1. labs 2. GnRH test

•inactivity of the hypothalamus and pituitary gland •Labs = low FSH, low LH, low testosterone, low estradiol •flat GNRH test

Congenital diaphragmatic hernia 1. pathogenesis 2. clinical presentation + cxr 3. tx

•incidence is 1:4,000-5,000 live births. 1. failure of the diaphragm to form between the fifth and 8th weeks of gestation ⇒ herniation + impaired lung development 2. scaphoid abdomen (abdominal cont. in thorax), respiratory insufficiency, bowel sounds in chest, mediastinal shift on X ray, bowel loops in thorax 3. *** first place NG tube then take XRAY⇒ NO BAG AND MASK VENT (will compress lung), treat acidosis, hypoxemia, surgical reduction of the hernia + closure of defect

Balanitis

•inflammation of the glans of the penis •may be associated with Candida spp. or Gram-negative infections in infants and with sexually transmitted infections in adults.

What defines a drinking problem

•intoxicated >6 times a year or having drinking associated problems with school/ friends/ family or driving intoxicated

What kinds of condoms protect from HIV? What are the disadvantages to condoms?

•latex ONLY •interference with spontaneity of intercourse and rare allergic reactions

What are the complications of adolescent (high risk) pregnancy for mother/infant?

•low birth weight (infant) higher infant mortality (infant) •preterm labor/ anemia/hypertension (mother) •dropping out of school •unemployment and need for public assistance

Pallor in neonate

•may be a sign of neonatal asphyxia, shock, sepsis, or anemia.

Accessory nipples

•may be present along the anterior axillary or midclavicular lines •may later grow because of the presence of glandular tissue in these areas.

Vaginal diaphragm 1. mechanism 2. advantages 3. disadvadvantages

•mechanical barrier placed against cervix, used with spermicide 2. can be put in 6 hours before sex (doesn't impede spontaneity) 3. INDIVIDUAL FITTING health care professional is neccessary, awkward to place, inc risk of UTI

Head growth abnormalities

•microcephaly •craniosynostosis •deformational plagiocephaly •macrocephaly

what are the changes that indicate substance abuse in an adolescent

•mood/sleep disturbance •truancy •decline in school performance •changes in family/friends relationships •diminished appetite or weight loss •depression •diminished participation in school or household responsibilities.

Hypothyroidism (pathologic short stature)

•most common cause of hypothyroidism is Hashimoto's thyroiditis •Patients will present with increased TSH, low T4, and positive antithyroid peroxidase antibodies

Achondroplasia

•most common skeletal dysplasia; characterized by rhizomelia. •AD caused by a mutation in the fibroblast growth factor receptor 3 gene. •Incidence increases with advancing paternal age. Skeletal findings (a) Lumbar kyphosis in infancy → lumbar lordosis in later childhood (b) Rhizomelia (c) Trident-shaped hands

Nevus simplex or Salmon patch or telangiectatic nevus

•most common vascular lesion of infancy •occurs in 30-40% of newborns •appears as a pink macular lesion on the nape of the neck ("stork bite"), upper eyelids, glabella, or nasolabial region. •It is often transient.

Cutis marmorata

•mottling of the skin with venous prominence

What are the causes of apnea in preterm infants?

•neonatal infections •lung disease •hypothermia •hyperthermia •hypoglycemia •seizures •maternal drugs •drug withdrawal •anemia •gastroesophageal reflux in addition to idiopathic apnea of prematurity.

2. Treatment for anorexia/bulimia nervosa

•normal nutrition •hospitalization- for severe weight loss, dehydration, electrolyte abnormalities, dehydration, abnormal vitals, seizures, arrhythmias, pancreatitis or food refusal •Pharmacotherapy Anorexia-SSRI or antipsychotics (olanzapine) Bulimia-SSRI

Breast milk jaundice

•occurs after the first week of life and is likely related to breast milk's high levels of beta-glucuronidase and high lipase content. •Elevated bilirubin is highest in the second and third weeks of life, and lower levels of bilirubin may persist until 10 weeks of life.

Breastfeeding jaundice

•occurs during first week, due to suboptimal milk intake. •Poor intake⇒weight loss, dehydration, and decreased passage of stool⇒ decreased excretion of bilirubin in the stool. *EXACERBATION OF PHYSIOLOGIC JAUNDICE

Esophageal Atresia with Tracheoesophageal Fistula 1. clinical presentation 2. associated conditions 3. dx/tx

•occurs in 1:2,000-3,000 infants and is often associated with polyhydramnios 1. Copious oropharyngeal secretions, inc. risk of choking/ aspiration pneumonia if feeding is attempted 2. congenital heart disease, anorectal/skeletal/renal malformations, VACTERL association 3. oral gastric tube + radiograph~~ surgical repair (closure of the fistula)

Neonatal acne

•occurs in ~ 20% of newborns •It appears after 1-2 weeks of life and is virtually never present at birth •Typically, the lesions are comedones, but inflammatory pustules and papules may be present •No treatment is necessary.

Paraphimosis

•occurs when the retracted foreskin cannot be re-turned to its normal position and acts as a tourniquet •results in obstruction to lymphatic flow and edema •Surgery is required emergently should venous return also become obstructed.

What complications is omphalocoele vs gastroschisis associated with?

•omphalocele = congenital heart defects/trisomies 13/18 •gastroschisis = bowel obstruction (not associated w/ congenital malformations)

Immunizations

•one of the most important components of well child care •are the cornerstone of pediatric preventive care. Types: • Active immunization: vaccines • Passive immunization: eg. immune globulin

What are the causes of primary pulmonary hypertension?

•perinatal asphyxia •meconium aspiration ~ more common in term infants

Clinical features of polycythemia

•poor perfusion •cyanosis •poor feeding •respiratory distress •lethargy •jitteriness •seizures •renal vein thrombosis •metabolic acidosis There is an increased risk of NEC.

Craniosynostosis

•premature fusion of the cranial sutures, which may result in abnormal shape and size of the skull.

Transient hyperammonemia of the newborn

•present in premature infants within the initial 24-48 hours of life Symptoms include: •respiratory distress •alkalosis •vomiting •lethargy rapidly progressing to coma. Aggressive treatment of hyperammonemia is required to prevent neurologic sequelae

Polyurethane female condom •Advantage •Disadvantages

•protection against STDs •vaginal irritation, allergy or awkwardness of placement

What is cervical ectopy and what STD does this inc. the risk for?

•seen in adolescent females: presence of cervical columnar epithelium outside the external os to which C.trachomatis and N.gonorrhoeae attach

Diastasis recti

•separation of the left and right side of the rectus abdominis at the midline of the abdomen •common condition in newborns, especially in premature and African American infants. •No treatment is necessary; gradually disappears as the infant develops and as the rectus abdominis muscles grow.

What prevents puberty in childhood? What changes at the onset of puberty?

•sex steroids (testosterone and estradiol) are depressed by negative feedback at hypothalamus •at puberty, there is a reduction in this hypothalamic inhibition, resulting in activation of the hypothalamic-pituitary-gonadal axis (HPGA). •HPGA releases gonadotropin-releasing hormone (GnRH) from the hypothalamus, which binds to receptors in the pituitary gland and causes the release of FSH and LH.


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