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USMLE-Step 1 ERP re 20 ‘Ad. year-old man comes tothe emergency rom wih crushing sustemal ches pain ht radiates to holo arm Ho sas that eso symptoms ‘tated to Reus ago andhave worsened. orl iors Siva fr coronary ate csease in oth hs ater and unc before te age of Soiyears old EXG is consstnt wh an ator wal mjeca‘al farcon. He s shed for amergentcardaceshetoastion at shows cccsion of thelettanteror descending aly He's slblaed fe nepal Faster wrk reveal snieayeevaed ves oftiglyenides and he ald ‘hat ise that he sufers fom fama hypertraycenderia “This sease is de to whic othe flowing? © (A) Avetpoprain A detcency © (8) LDL receptor decency ©(C)_ Lectin cholestrol acytansforasadecioncy (0) Upoproten pase decency ©(E) Veryiow densty ipoprten underprosucton ‘sow / he Extention USMLE-Step 1 ERP re 20 |ASmonthkd chic is rough othe ofce er afoliow up appinment He was clagnasad wth alu ore athe previous vist and has been ‘returning every cher week Weighed He nly was treastiog but wae steno caane neh commer frmula athe ast et Ati vo ‘he paont re cencemed beeavse Ne ds not seem to recogni he face anymore Tay alsa sy hatha dees noo ever math cect hoc elds head up wihout support On examination he fess han the S% for bot length ad waght. Ha hea hag puplay constcton fo Sat betray and op atophy noted on fioscome examination. Hs examinations these wit nol ints Laborstoy studies reveel 3 ‘decrease n gaacoosebrosKase act win He Rakes. ie of te otowng ste most key dagnos's? © (A) Gaucher's dsoase (8) Hers asease ©(C) Krabbe’ asease » (0) Mtactromate oukodstophy ©(E) Taysacs disease ‘sow / he Extention USMLE-Step 1 ERP re 20 |At.manth.ot git ot Asana Jewish decntis brought oe cnc fra wel cid evaluate. On review of developmental milastones her parents ‘note hat she doos not coo, bate, rlaugh but does fecoghze het paenis voices and tins towards them ule speleng Sh can ony ol fam is ‘femecn ahs bate nat tom her sk to font On examinton shee athe 1% for naght and weight She has apstosplencregay and a tery. spot onthe macs, “Thi patent mosey Ns high eves of whi oe foeweny? » (A) Corami tmexoside © (8) Corbrsiée sutote (6) Galacocerbrese » (0) Glucocerebrosige ©(E) Sphingomyein Sow / de Exlenaton

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