Usmle Ck - Pediatrics

Front 1

lab findings in Reye's syndrome? dz with similar presentation?

Back 1

- increased LFT, NH4, PT, hypoglycemia
- biopsy: microvascular steatosis
- tx: supportive
- similar = systemic carnitine deficiency => increased acyl-carnitine

Front 2

systemic carnitine deficiency?

Back 2

- same picture as Reye's but increased acy carnitine

Front 3

atlantoaxial instability?

Back 3

malformatin in 10-15% down syndrome => lax posterior tranverse ligament => increased C1, C2 mobility => spinal cord compression => behavioral changes => urinary incontinence, vertigo, increased Upper motor
signs
- xray, surgical tx

Front 4

spinal cord infarction

Back 4

- adults
- acute severe pain, weakness, parasthesias

Front 5

6 y.o. boy with pain in his legs at night, few hours? benign exam?

Back 5

- growing pans, observe; sx: poorly localized
- no xray; don't xray if + pain but neg PEX findings

Front 6

ADHD

Back 6

- sx 6 months
- at least since age 7
- sx at school and home
- attention, impulsive, hyperactive

Front 7

autism

Back 7

- aka pervasive developmental disorder
- age 3 y.o., decreased interaction, communication, language, sterotyped behavior
- 1st sx: lack of social smile and separation anxiety

Front 8

HSP

Back 8

- vasculitic condition, pt is s/p URI
- palpable purpura on butt, scrotal swelling, peripheral edema, renal = hematuria, proteinuria
- tx: steroids, monitor renal fx
- NOTE: Increased risk for intussusception => look for abd pain

Front 9

** child with hx of steatorrhea and neonatal jaundice?

Back 9

- r/o CF! with chloride sweat test x 2 followed by genetic testing
- tx: high calories, pancreatic enzymes, fat soluble vitamins
- complications: gallstones, cirrhosis, portal HTN, pancreatic fibrosis
- MC lung infx 2/2 s. aureus, pseudo, h flu

Front 10

dx of pyloric stenosis?

Back 10

- u/s

Front 11

kartagener's syndrome

Back 11

- triad: situs invertus, recurrent sinusitis, bronchiectasis
- AR, dysmotile cilia 2/2 dynein arms

Front 12

bronchiectasis

Back 12

- bronchial obstruction -> secretion rettention => bronchiectasis
- can be congenital with abnormal development of bronchial system => cysts, cul de sacs

Front 13

vesicoureteral reflux

Back 13

- short submucosal portion of ureter btw mucosa and detrusor muscle is abnormal => reflux
- MCC acute UTI in kids
- dx: voiding cystourethrogram

Front 14

vit A def

Back 14

- night blindness, photophobia, dry skin, cloudy cornea, bitot spots, hyperkeratosis

Front 15

infantile thiamine deficiency?

Back 15

fulminant cardiac syndrome: cardiomegaly, tachy, cyanotic, dyspnea, vomiting

Front 16

hyper vit A?

Back 16

anorexia, pruritis, tender bones, chelitis, increased ICP, hepatomegaly

Front 17

coarctation of aorta?

Back 17

- RIB NOTCHING 2/2 dilation of collateral chest wall vessels
- see in turners
- increased arm BP, decreased leg BP => fatigue while walking up stairs=

Front 18

CAH syndromes?

Back 18

- increased andogen, low MC, GC = 21 hydroxylase def => increased 17
- high androgen, high MC => 11 alpha hydroxylase def
- high DHEAS, low T, low MC => 3 beta hydroysteroid dehydrogenase

Front 19

physiologic juandice vs others

Back 19

- occurs within 24 hrs = erythroblastosis fetalisis => increased direct or indirect => rapid rise in bili, anemia, hepatosplenomegaly, reticulocytes
- within 2nd or 3rd day lasts weeks, increased INDIRECT: physiologic jaundice 2/2 increased bilirubin, decreased bili clearance (low UGT), increased enterohepatic circulation
- jaundice at 5 days: neonatla sepsis
- @ 1 week: breast milk jaundice => bottle feed x 2 days
- increased direct bili = bad

Front 20

kawasaki dz?

Back 20

- kids with fever for 5d, painless red eyes, truncal blanching rash, injected pjarynx, unilateral cervical lymphadenopathy
- ASA + IV immunoglobuline

Front 21

rash + unilateral cervical LAD and fever?

Back 21

- kawasaki

Front 22

stranger anxiety vs separation anxiety?

Back 22

- stranger anxiety: begins @ 6-8 mo, peaks at 1`2-15
- separation: older, go to extreme means to avoid seapration from parent

Front 23

REtt's d/o

Back 23

- @ 5 months, start deteriorating previously acquired skills

Front 24

kid s/p sore throat now with pericarditis, subQ nodules?

Back 24

- rheumatic fever, Jones criteria
- major: polyarthritis, carditis, chorea, subQ nodules, erythema marginata
- minor: fever, arthralgias, previous rheumatic fever
- 2/2 group A strep
- tx: PCN G

Front 25

mumps

Back 25

- paramyoxovirus => parotitis/mumps, pancreatitis, orchitits

Front 26

MCC pericarditits?

Back 26

- coxsackie! but ddx rheumatic w/ presence of subQ nodules, chorea

Front 27

child's parent has hx of high cholesterol, or CAD RF?

Back 27

- do screening cholesterol on child
- if high (>200) then do fasting lipid

Front 28

infant with poor growth, low bicarb?

Back 28

- i.e. pt is acidotic = RTA
- type 1: bad H secretion= acidotic, hypoK+, INCREASED URINE PH, genetic, parents with hx nephrolithiasis
- type 2: decreased bicarb resorption in PT = fanconi
- type 4: defect Na/K+ exchange in distal tubule => hyperK+, hyper Cl, acidosis, hx of obstrctive uropathy, renal dz, multicystic dysplastic kidney

Front 29

boy with gynecomastia?

Back 29

- seen in 1/2 boys at around 14 y.o.
- 2/2 increased androgens during adrenal adrenarche => increased estrogen convergion and also increased LH in pubery => increased leydig cell production of E
- transient just watch

Front 30

normal and abnormal testicular size?

Back 30

- prepubertal: 2cm, 3mL volume
- puberty: 25 ml volume if <10ml then consider klinefelter

Front 31

anemia

Back 31

- if suspect, give Fe => if no response => Hg electrophoeresis, colonoscopy, serum Cr for ? renal dz

Front 32

cyclical vomiting

Back 32

- unknown cause, increased hx of parent with migraines
- no tx

Front 33

ITP

Back 33

- often age 2-6
- s/p viral infx => Ab attacks platelet => destroyed in spleen -> sx of petechiase, purpura, hematuria,
- observe, steroids in <30,000 plts, if v severe = splenectomy

Front 34

newborn with noisy breathing?

Back 34

- laryngomalacia = MCC chronic respiratory noise
- direct laryngoscopy => epiglottis rolling in from side to side => improves by age 2
- tell mom to hold baby up x 30 min s/p feeding and never feed lying down

Front 35

immune deficiencies

Back 35

- genetic B cell deficiency: @ 6 mo when decreased mom Ab, increased sinopulm infx from encapsulated bugs = H flu, s pneumo, decreased IgA = increased giardia and GI infx
- chronic granulomatous dz 2/2 decreased NADPH ox metabolism of phag cells = > decreased intracellular killing => abscesses secondary to catalase producing organism e.g. aspergillus, staph
- complement def: gonococcal and meningococcal => S. pneumo, H. flu, no giardia
- thymic hypoplasia => low T cell => increased viral and fungal
-severe combined immuno def = Adenosine deaminase def => low B and T = viral, fungal, bacterial infx

Front 36

phenylketonuria

Back 36

- musty urine smell
- no phenylalaine hydroxylase for phenylalanine to tyrosine => MR, seizures
- dx by blood phenyl or guthrie test
- avoid protein

Front 37

dx porphyria?

Back 37

- aminolevuline acid, porphobillinogen

Front 38

AVM malfomation

Back 38

- MCC subarachnoid hemorrhage in kids => nuchal rigidity
- look for hx of seizures, migraines to confirm diagnosis

Front 39

HIV testing in neonates?

Back 39

- serological testing not useful b/c of mom's Ab in fetal circulation
- test by viral cx and PCR and p24, positive if 2 of 3 are positive
- baby is asympt at birth but at 1 mo: oral thrush, LAD, diarrhea, 3 yr survival

Front 40

Non Hodgkin lymphoma sx?

Back 40

- abd sx, abd distension, ascites, urinary tract obstruction, intestinal bstruction

Front 41

17 y.o. with hirsutism?

Back 41

- increased 17-hydroxy = CAH; pt wil have normal periods
- increased DHEA = cushings
- increased LH:FSH = CAH vs PCOS
- idiopathic = increased T to dyhydrotestosterone in hair follicle = +fhx, medit descent

Front 42

dx classical PKU?

Back 42

- need to have 1. plasma phenlalanine >20mg/dL, 2. normal to low plasma tyrsodine; 3. increased urinary level phenyalainine met products (phenylpyruvic acid, o-hydryoxyphenlacetic acid); 4. normal tetrahydrobiopterin level
- benign = <20 mg/dL phenyalanine
- transient = no urinary phenylpyruvic acid

Front 43

pt smells like boiled cabbage

Back 43

- tyrosinemia 2/2 fumarylacetoacetate hydrlase deficiency

Front 44

alkaptonuria?

Back 44

- homogentisic acid oxidase deficiency => increased homogentisic acid
- URINE TURNS BLACK on standing
- dx: homogentisic acid in urine

Front 45

s/p uri or skin infx now with fever and painful knee, neg xray?

Back 45

- septic arthritis
- do arthrocentesis, IV naficillin or cefazolin 2/2 staph aureus

Front 46

teratogens:
valproic acid
phenytoin
cocaine

Back 46

- valproic acid: NTD, cardiac, dysmorphic facies: broad and flat nasal bridge, cleft lip
- phenytoin: facial hyantoin syndrome = nail and digit hypoplasia, MR
- cocaine: jittery, increased sucking, moro reflex, ADHD, low intelligence

Front 47

turner syndrome

Back 47

- webbed neck, lymphedema 2/2 lymphatic network dysplasia (nonpitting edema)
- other sx: high palate, short 4th metacarpal, mail dysplasia
- DO ULTRASOUND TO SCREEN FOR HORSESHOE KIDNEY

Front 48

clavicular fx 2/2 big baby delivery?

Back 48

- no tx, will heal on own

Front 49

seizures

Back 49

- absence seizure: daydreaming episodes, no loss of postural control, brief lapses of consciousness, decline school performance
- generalized tonic clonic: loss of posturla control, biting tongue, post ictal confusion, bowel and bladder loss
- atonic seizures: loss of postural tone 1-2 sec
- simple partial: no loss of consciousness
- complex-partial: aura, behavioral arrest, automatism, confusion

Front 50

fanconi's anemia

Back 50

pancytopenia, hyperpigmentation on trunks, intertriginous area,s cafe-au lait spots
- shot, hypogonad, skeletal abnormalities

Front 51

diamond blackefen anemia

Back 51

- congenital pure red cell aplasia in 3 months, low retic

Front 52

transient erythroblastopenia of childhood

Back 52

- red cell aplasia in healhty kid
- low retic