Step 2 Pediatrics 1 (10-21-15)

ruhland1's version from 2015-10-21 21:29

Section 1

Question Answer
dial testPCL tear, knee joint external rotation
Legg-Calve-Perth(Leg-Calf-Patella-Hip) avascular necrosis of femoral head
Legg-Calve-Perthreduced abduction and int rotation
Leg-Ca-Per vs Osgood vs Kohlerall AVNec LCP=femoral head Os=tibial tuberosity Kohl= Navicular
LeCaPerth tx<8 yo therefore cast/bedrest, >8 year old femoral/pelvic osteotomy
Osteoachondromametaphyseal benign firm mass
Saphenous neuritispain inferior and medial knee
trendelenburg signin DDH dip pelvis in oppositie side when patient stands
DDH dxuse ultrasound, x-rays unreliable
DDH <6 moPavlik Harness
DDH 6-15 mohip spica cast
DDH 15-24 moOpen reduction followed by hip spica cast
DDH 24 mo +closed reduction
DDH complicationsleg calve perths
CMV headvisual change, microceph, chorioretenitis
CMV skinblueberry muffin
CMV headperiventricular calcifications
CMV txgancyclovir/foscarnet

Section 2

Question Answer
MEN IPit, Para, Panc
MEN IIaPheo, thyroid(medullar cx), Para
MEN IIbPheo, thyroid(medullar cx), mucocutaneous neuromas
osteosarcomacodmans triangle
codmans trianglesunburst pattern
hypokalemia and up pHbartterm gitelman, liddle
bartternormal/dn BP
Gittelmannormal/dn BP
Liddlesup BP
Bartter labud Mg
Gitelman labdn Mg
Bartter txinc salt, K+, NSAID
Gitelman txK+,Mg++,Amilioride
Liddle TxAmiloride

Section 3

Question Answer
Hodgkin historeed sternberg
Hodgkin sxlow F, night sweats, dn lb
ALLdown syndrome, 2-5 yo, HS-meg, LAD
ALL stainTdT nuclear positive
AMLrecurrent infection, bleeding, fatigue
AML>20% blasts, Auer Rods
CMLbcr-abl fusion uninhibited growth
CML finding9;22
CML pxexS-meg
2-5yo + bone pain + unilateral testi involcedALL
monoretroauricular lymphadenitis, fever, sore throat
EBV ddx CMVEBV has gray white membrane on tonsils
Heterophile +EBV
CMVheterophile (-)
acute huge cheeks huge testesmumps
Charcot Marie Toothdefective production of periphreal nerves of myelin sheath
wk distal limbs, loss proprio and vibCMTooth

Section 4

Question Answer
wasting hips and shoulderlimb girdle muscular dystrophy
myotonic dystrophyCTG repeat
facial wk, inability to relax hand grip, cataractsmytonic dystrophy
thombocyptopeniadn platelet up Bleeding Time
VWD geneAuto dom
VWD up BTime up PTT
VWD affectsF8 and platelet function
Hemophilia A geneX-linked recessive
Hemophilia Aup PTT
DICdn Pl8 up Btime up PT up PTT
Scurvyno change in clot function but up bleeding gums
Idiopathic thrombocytopenic purpuradn Pl8, up BTime

Section 5

Question Answer
williams syndromelfin facie, bicuspid aortic valve
lithium during pregcauses ebstein
ebsteintricuspid displaced down, RAH, S1+S2+S3+S4
ebstein ausctricuspid regurg murmur AND mid diastolic murmur
causes middiastolic murmur in ebsteinRA->LA flow ASD
perimembranousat junction between membranous and muscular portions
interventricular septumhas membranous and muscular portions
pulmonary atresiapulmonary valve orifice fails to develop obstructing outflow from heart to lungs
early systolic murmur at left sternal edgeStill's Murmur

Section 6

Question Answer
Hartnuptrypto def, niacin deff, photosenitive derm, dementia, diarrhea
whooping coughtx 14 days erythromycin for pt and family
Kid+ liver dz + brain dzwilsons
histidine decarboxylase defup Tourettes synd
scm spasmclavicle frx
homocystinuriaosteoporosis, recurrent thromboemboli, AR, lens dislocation
absent C1 esterase inhibitorHereditrary Angioedema
hereditary angioedemaAD, Urticarial swelling, Nigga Lips, N/V/D,
danazoltx herediatery angioedema
apthous stomatitiscanker sores
Ataxic Telangictasiadn DNA repair, Ataxia b/c dn periphreal nerves, dn UV damage repair so up telangectasia
microtubular dysfunctionCHAIN
zinc def hairloss
zinc def mendelayed maturation
zinc def skindry/scaly
zinc healslow
zinc sensesdn smell/tast