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NBME 22 Answers

nbme22/Block 1/Question#11 (reveal difficulty score)
A 6-month-old boy has recently been diagnosed ...
Sphingomyelin degradation πŸ” / πŸ“Ί / 🌳
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 +15 
submitted by βˆ—seagull(1789),
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hisT is a ince poarpcha ot yingeidfnti adn itnraget ciiotastuf oddeirr.s

as6hk.tecl?latMgsvcchbXeFsorbgR+ht/w=2oipgo.kcbi+c=t&t#w/o8/p6m:wnaemhOasqd:r=pmx


 +6 
submitted by βˆ—atstillisafraud(214),
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kaec-mNinnPi seiDsae esnspert iwth mlnaet aitroadenrt, pliid ldnea (mafo slecl) ni oben rarmwo dna rcryeh edr spto on aulm.ac No nshisoyigeaemnpl setslru ni ulibpud of nigoshlnmiype wchih dluisb up ni ahaeogr.csmp

meningitis  "Pick your **Big** **Foamie** **Zeibra** nose with your Sphinger" Choose options with the letter I. SpIngomyelin, Sphingomyelinase, bIgorgans (hepatomegaly etc), zeIbra bodies, Foam cells +2  



 +3 
submitted by βˆ—meningitis(619),
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i"Pck uyor Bgi, eomaFi, rbieaZ enso htiw yuor nhSgipr"e

Csoheo ponoits itwh eht ttelre I.

nimnSpIyloeg, haSgipnneom,liyes sgrbgIona tmahaleype(go ct)e, rbaIze ,bodesi mFao lsecl




 +1 
submitted by βˆ—castlblack(71),
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Huh. hWo lsee otghuht thsi was Ghacure ntilu uoy ahd to pkci ?D

elephantbuddy  I think that the hepatosplenomegaly is definitely a common characteristic but you would see more bone abnormalities in Gaucher. This patient has no bone crises and exhibits developmental delay, HSM, and foam cells which is more characteristic of Niemann-Pick. +2  



 +0 
submitted by βˆ—chaosawaits(63),
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Can noeesmo easelp xenalpi wyh isht is nto ?oerlslrphieyhoteceam tI osal nspstere iwht HSM adn rehet ear snouumer amof clle.s iecSn ythlpeoersearloimehc lsao epnetsrs thwi saatinretcp,i ntc'dluo that xepanli eurencrtr o?mintvgi 102F,9A p49. knaTh oyu rof ruyo pso!esesrn

chaosawaits  I mean Type I familial dyslipidemia (hyperchylomicronemia). Am I correct in assuming that the most differentiating presentation is the developmental delay? +  



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