Pediatrics

Question 1

presentation of systemic onset juvenile idiopathic arthritis

Answer 1

> 2 weeks of fever once a day, 1+ jt arthritis, pink macular rash that worsens during fever

Question 2

tricuspid valve atresia findings

Answer 2

cyanotic infant with L axis deviation. absent R waves in precordial leads, peaked P waves, decreased pulmonary marking on CXR

Question 3

scarlet fever presentation

Answer 3

fever, pharyngitis, sandpaper like rash, circumoral pallor, and strawberry tongue

Question 4

Wiskott Aldrich syndrome

Answer 4

eczema + thrombocytopenia + hypogammglobulinemia

X linked

Question 5

Fanconis anemia

Answer 5

AR, average dx age = 8
progressive pancytopenia + macrocytosis
ass’d w/ cafe au last spots, microcephaly, microphthalmia, short stature, horseshoe kidney, absent thumbs

Question 6

Diamond Blackfan syndrome

Answer 6

macrocytic pure red cell aplasia ass’d with congenital abnormalities (short stature, webbed neck, cleft lip, shield chest, triphalangeal thumbs)

• -low retic (b/c intrinsic defect of RBC progenitors)
• -no PMN nucleus hyperseg
• -elev fetal Hb on electrophoresis
• -Tx = steroids +/- transfusion

Question 7

trachoma

Answer 7

Due to Chlamydia trachomatis ABC

• -follicular conjunctivitis + pannus/neovasc in cornea –> eyelid scarring and inversion of eyelashes –> ulceration –> blindness
• -often presents with concurrent nasopharyngeal infection
• -treat the entire village (azith)

Question 8

management of newborns born to mother with active Hep B

Answer 8

Hep B Ig followed by Hep B vaccine

Question 9

which kids with bronchiolitis get palivizumab?

Answer 9

preterm birth <29 weeks gestation
chronic lung disease of prematurity
hemodynamically significant CHD

Question 10

retropharyngeal abscess

Answer 10

feevr + dysphagia + inability to extend neck + muffled voice + widened prevertebral space on lateral Xray

Question 11

Friedreich ataxia

Answer 11

AR, excess GAA repeats –> abnorm frataxin protein

neuro (ataxia, dysarthria, loss of position sense and reflexes)
+ skeleteal (scoliosis, hammer toes/feet deformities)
+ cardiac (concentric hypertrophic cardiopmyopathy, degeneration of cardiac muscle fibers -> can cause T wave inversion)

MRI - atrophy of (cervical?) SC and minimal cerebellar atrophy

death-heart and resp complications

TX = PT, psych support

Question 12

risk factors for jejunal atresia?

Answer 12

prenatal exposure to cocaine and other vasoconstrictive drugs
meconium ileus that causes volvulus and necrosis of bowel

Question 13

erythema chronic migrans

Answer 13

early, localized Lyme disease rash
tx = oral doxy, amor, or cefuroxime
**amox if <8 yo

Question 14

Guillain barre syndrome management

Answer 14

• *serial measurements of FVC to monitor respiratory function to predict impeding resp failure and intubation need
• *cardiopulm support

Question 15

classic presentation of galactosemia

Answer 15

–galactose 1 P uridyl transferase deficiency –> elevated blood levels of galactose

infant/newborn with failure to thrive, bilateral cataracts, jaundice, hypoglycemia
+/-aminoaciduria, hepatic cirrhosis, mental retardation

*increased risk for E coli sepsis

Question 16

galactokinase deficiency presentation

Answer 16

asym except cataracts

Question 17

complications of SGA infants (6)

Answer 17

1. hypoxia, perinatal asphyxia,
2. meconium aspiration
3. hypothermia
4. hypoglycemia
5. hypocalcemia
6. polycythemia (from increased EPO secretion in response to hypoxia)

Question 18

RDS risk factors

Answer 18

1. premie
2. male sex
3. perinatal asphyxia
4. maternal DM
5. C section w/o labor

Question 19

McCune Albright syndrome

Answer 19

3 P’s:
Precocious puberty
+ Pigmentation (cafe au lait syndrome)
+ Polyostotic fibrous dysplasia (bone defects)

+/- endocrine disorders (hyperthyroidism, prolactin or GH secreting pituitary adenomas, adrenal hypercortisolism)

*sporadic defect

Question 20

bug for PNA in CF patients

Answer 20

childhood: staph aureus
adults (20+): pseudomonas

Always treat for both

Question 21

edema in Turner’s syndrome

Answer 21

due to dysgenesis of lymphatic system –> congenital lymphedema
**nonpitting

Question 22

what fluids should be used for IV fluid resuscitation?

Answer 22

NS (isotonic)

Question 23

what is the most common predisposing factor for orbital cellulitis?
complications?

Answer 23

bacterial sinusitis

complications: blind, abscess, cavernous sinus thrombosis, intracranial infection, death

Question 24

most common CHD in Down syndrome?

Answer 24

complete atrioventricular septal defect

-load S2
-systolic ejection murmur (b/c incr pulm flow from ASD)
+/- holosystolic VSD murmur

Question 25

spondylolisthesis

Answer 25

forward slip of vertebrae (usually L5 over S1) usually in preadolescent kids --back pain + neuro dysfunction + palpable step off

Question 26

von Gierke disease (type 1 glycogen storage disease)

Answer 26

glucose 6 phosphatase deficiency impairs glycogen to glucose conversion --> glycogen accumulates in affected organs - -presents at 3-4 months - -hypoglycemia (+/- seizures) + lactic acidosis + hyperuricemia + hyperlipidemia - -doll like face, thin extremities, short stature, protuberant abdomen (b/c hepatomegaly) - -spleen and heart not involved

Question 27

what to do if adverse run to tDap?

Answer 27

give DT instead | -reaction is to pertussis component

Question 28

blount disease

Answer 28

genu varum/bow legs in >2yo | ortho consult

Question 29

legg calve perthes disease

Answer 29

avascular necrosis of femoral head painless limp in boy <10yo ortho consult

Question 30

slipped capital femoral epiphysis

Answer 30

painful limp in obese boy | ortho consult

Question 31

osgood schlatter disease

Answer 31

overuse injury of quads and patellar tendon --> traction apophysitis of tibial tuberosity --> localized pain and swelling tx = RICE and NSAIDs

Question 32

lichen planus

Answer 32

purple polygonal pruritic papules | tx = PO steroids

Question 33

Henoch Schonlein purpura

Answer 33

anaphylactoid IgA mediated small vessel vasculitis --> palpable purport on butt and legs + renal disease + ileocecal edema --> colicky abdominal pain and risk for intussusception

Question 34

porphyria cutanea tardis

Answer 34

mut uroporphobilinogen decarboxylase --> photosensitivity to UV light + tea colored urine + recurrent attacks of abdominal pain

Question 35

alopecia areata

Answer 35

hair stops growing suddenly --> small patches of complete hair loss with exclamation point stubs

Question 36

cherry red macula w/o HSM

Answer 36

Niemann pick disease | mut spingomyelinase

Question 37

retained primary teeth

Answer 37

job syndrome (hyper IgE)

Question 38

single central incisor

Answer 38

GH deficiency

Question 39

pes cavus

Answer 39

high arched foot ass'd with freidrich ataxia and Charcot Marie tooth disease

Question 40

micropenis

Answer 40

GH deficiency

Question 41

neonatal dx of HIV

Answer 41

PCR b/c mom's Ab can cause false +

Question 42

PPx for cat/dog bite

Answer 42

Augmentin

Question 43

salmon patch (nevus flammeus)

Answer 43

benign splotchy red rash on forehead, eyelids, back of neck of newborn --more prominent with exercise or emotion

Question 44

pustular melanosis

Answer 44

benign little pustules that leave a hyper pigmented spot when ruptured

Question 45

erythema toxicum

Answer 45

benign yellow-white papule with surrounding erythema | --fluid exam shows eos

Question 46

sebaceous nevus

Answer 46

raised yellow orange hairless lesion on scalp | tx = resection before adolescent b/c it can undergo malignant transformation

Question 47

incontinentia pigmenti

Answer 47

XR inflam bullae that evolve into hyper pigmented lesions lethal in males

Question 48

transient tachypnea of the newborn

Answer 48

tachypnea, grunting, +/- cyanosis in c/s baby due to retained fetal lung fluid CXR - palm vascular markings Tx - 100% oxy (improves sats unlike RDS) Resolves within 3 days

Question 49

congenital varicella syndrome

Answer 49

limb hypoplasia + clear vesicles --> cutaneous scars, chorioretinitis, cortical atrophy Tx VZIG if mom has chickenpox 5 days prior - 2 days after delivery; otherwise reassurance

Question 50

caput succedaneum

Answer 50

soft tissue swelling of scalp where baby was delivered | crosses midline

Question 51

cephalohematoma

Answer 51

bleedings between periosteum and skull --> squishy feel to scalp that doesn't cross midline

Question 52

subgaleal hemorrhage

Answer 52

bleeding under gall aponeurosis --> squishy feel to rapidly expanding scalp that crosses midline tx = admit to NICU for possible hemorrhagic shock

Question 53

vit A toxicity

Answer 53

inc CSF pressure (HA, blurry vision) dr skin teratogenic found in animal liver consumption

Question 54

vitamin B7 / biotin deficiency

Answer 54

seborrheic dermatitis enteritis alopecia *found in kids who eat raw eggs or biotinidase deficiency

Question 55

vit C toxicity

Answer 55

uric acid stones

Question 56

vit E deficiency nd toxicity

Answer 56

``` deficiency = hemolytic anemia toxicity = hemorrhagic diathesis ```

Question 57

what important nutrient is goats milk missing? cows?

Answer 57

``` goat = folate cow = iron ```

Question 58

management of aspirin poisoning

Answer 58

sodium bicarb +/- dialysis

Question 59

cyanide poisoning and tx

Answer 59

headache --> confusion, agitation --> seizures and meta alka | tx = nitrites, hydroxocobalamin + thiosulfate

Question 60

phenothiazine and metoclopramide poisoning

Answer 60

EPS (torticollis, opisthotonus, dyspahgia) | tx = diphenhydramine