Step 2 Pediatrics 1 (10-22-15)

ruhland1's version from 2015-10-22 14:05

Section 1

Question Answer
anakinraDMARD IL-1 antag
gold saltfor RA causes GM suppression
hydroxycholorquinedown TLR signaling in dendritic cells, also used in Lyme dz
Glucocorticoid used in AI to reduce immune response
AI Hepatitisanti-centromere
Pemphigus Valgarisanti-desmoglein
Bullous Pemphigusanti-hemidesmosome
Sjorgenanti-ro (anti-ss-A)
SLE specificantismith an anti dsDNA
mixed connective tissue diseaseanti-rnp(ribonucleoprotein)
primary billiary cirrohsisanti-mitochondrial
dermatomyositisanti-jo (anti-SS-B)
polymyositisanti-jo (anti-SS-B)
primary billiary cirrohsisanti gp 210
cutaneous systemic sclerosisanti centromere
drug induced lupusanti histone
C...Chlorpromazine H...Hydralazine I...INH M...Methyldopa P...Penicillamine `s P...Procainamide I...Interferons Q...Quinidinecause drug induced lupus
chlorpromazinecauses drug induced lupus is a antipsychotic
hydralazinecauses drug induced lupus is a smooth muscle relaxant for preeclampsia to dn BP
methyldopacauses drug induced lupus is alpha 2 ag
procainamidecauses drug induced lupus is an antiarrythmic for a-fib
quinidinecauses drug induced lupus class 1 antiarrythmic

Section 2

Question Answer
osteochondritis dissecansknee medial femoral condyle, vascular insult leading to fragmentation and separtion of adjacent bone and cartilage
age slipped captial femoral epiphysis10-16
leg calves perthboys 4-10yo (avasc necrosis of femoral head)
true diverticulumhas 3 layers (mucosa, muscularis and adventitia
failure of closure of vitiline ductmeckels divert, viteline duct cyst(enterocystoma), omaphocele
meckles divert dxnuclear radio tracer for gastric tissue uptake which is in meckels, or use arteriogrpahy to reveal an anomalous superior mesenteric artery branch supplying the diverticulym
transmural inflamm of entire GI tractchrons
chordeeupward penis curvature seen in hypospadias
bowel borbobrygmusintussusception
painless gastrointestinal bleeding age 2think meckels diverticulum
repititive stress of proximal tendons of extensor carpi radialislateral epicondylitis
repitive stress of proximal tendons of flexor carpi ulnarismedial epicondylitis
anterior radial headfalls backward on supinated forearm
sublux radial head txflex elbow to 90 degrees while also supinating forearm

Section 3

Question Answer
recurrent thromboemolic episodeshomocysteinuria (cannot compeletly degrade a methionine metabolite)
histidine decarboxylase defcannot break down ___ into histamine so up Tourettes
Hyperprolinemia(AR) sz, retard, high [ ] of it in plasma
def proline oxidasehyperprolinemia
RTA I(distal)alkaline urine, up Cl- up H+, inability to excrete H+ and regenerate HCO3-
RTA II(proximal) cannot resorb HCO3-, acidic urine, hypokalemia
HCO3 in urinebinds K+ so if up HCO3- excretion * up K+ excretion
RTA I txoral HCO3-
RTA II tx thiazides
RTA II causescarbonic anhydrase inhibitors, hyperparathyroidism
RTA I causesamphotericin, light chains in multiple myeloma
RTA IVhypo renin * hypoaldo + dn Na+ and up K+ so up Cl-, urine acidic
li fraumeniswide variety of cxs at multiple sites and young age of onset
fanconi syndromprox convul tubule unable to resorb many things
fanconi causeswilsons disease, expired tetracycline
fanconi assocrickets, RTA II, hypokalemia
RTA I dxNH4Cl test, severe change in pH confirms

Section 4

Question Answer
cutaneous photosensitivityhartnup finding
defect in transporting neutal AA including tryptophandn serotonin dn melanin dn niaicin in hartnup
def in cystthionine beta-synthasehomocystinuria
homocystinuriaocular lens down and in sublux, skeletal abnormalitiies, thromboembolic episodes
neonate with severe metabolic acidosisgutathione synthtase def (rbc thorn cells)
phenylketonuria vs glutathione synthase defPKU has later lactic acidosis, GSDef has immediate metbaolic acidosis
key hole signdilated posterior ureter seen in posterior urethral valves
doll face + hypertriglyceidemia + hyperuricemia + hypotoniavon gierke
von gierkedef glucose-6-phosphatase
elevated direct bilirubin due to liver damahegalactosemia
wide fix split S2ASD
turnersyndMC = coarc aorta 2nd MC = bicuspid aortic valve
patent ductus arteriosuscogential rubella, widened pulse pressure
holosystolic at left lower sternal bordeeVSD