NBME 24 Organic acid metabolism disorder (NBME Answers)

Retired NBME 24 Answers

nbme24/Block 4/Question#8 (reveal difficulty score)
A 1-week-old newborn is brought to the ...
Organic acid metabolism disorder 🔍 / 📺 / 🌳 / 📖
tags: biochem artist90

+47 upvote downvote
submitted by ∗lsmarshall(465)

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raUe cyeCl rDosiedsr > Iaosedlt eervse ynheemmpaoarim ;g&t( ;1000 .ie., on oetrh reseev ieltmacob narbscsditeu

tOiirnneh tmlrrnbcsesaayaa yecfinidec > smo(t omoncm ruae elycc s.d)i ciorot eiduaidiri/acaamc, aimephrmnemyoa

gcaOinr saiemidAc &t;g raepnmi,Hmyeamo ani-pgoan saoci,sid sestoki f(omr amcyphelg)yio

nacud-imeihM oCc-lAya gdyoenahdrese dfeycniiec ;t&g moemyain,maepHr tekphociyot eyhlioaypmgc s(nee in -xaiotiβond rsro,ideds XTEEPC kulp)eeooyhsdradtryon

Livre ucdostnifyn g&t; yimaproHneem,ma sTFL meessd ,pu edorl p.t

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lsmarshall ySarmum of atoibcmel usises gnieltra to ermnmmyaieopha +9

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notadoctor Not sreu ywh tshi n'its esdcdioern a diatlorcmhoin irrdsedo nesci teh siuse is hnntOeiir sraeractysanmbla iccfeenyid ni teh iacohmrtnoid? +2

charcot_bouchard fi it wsa rhdlaiioctnom erddrsoi no one dulwo acspee +3

wowo feirgu ni TCO decincy,efi ehty imhgt veah to eypiitxlcl tinenom hte icrtoo riucdiaa ADN ypcylaitl nesptrse ia,eelrr odruan hrs-2484 fo lfei eftar vte'yhe fde t(a etasl rpe +BB) salo rep B,B copiornpi acdaiemi dna MM idcameai eavh na eonst fo kswee ot nomtsh adn deal to dbiul pu fo roagcni sadci &;tg-- iciadaem ni idotidna to oieprmanhmmeay (not eusr yhw, btu aesvlre aa nrete hte TCA lcecy via inlpoopyr oAC gt-&-; tomeanllylyhm oCA g-&-t; ilnucycs ACo, tbu nwo isth si untfecd td/ znyeem ncee.efd.ii?.)is.c wyho,nA inorpocip daiameci sbdceedir no F201A9 ,85p but etdsno' sitl merpyhaineaomm +2

artist90 i nhtik it nnaotc be iniOhetnr arntmbesaaacrsyl ycdeniifce cb it si RX saedsi.e tsih pt ahs a yhhatle 2ry dol robhret ihchw lsrue otu nleXdki- scseiveer daieses orertcc em if i m rognw +7

artist90 it is %100 oniprPioc ediamcai dlorUw i:dQ- 1.430 it is an atcex pcyo qseuinto fo duorwl. i ogt ti rgwno cb i otfrog tshee rea gniacor sa.cdi utB i am siltl scdnfeou no 2 ghhni-1o wst osde osiasidc suace aomcyyipHgle nda s.yoKesthw-2i si mAaimno leedevta ni teseh tsp bc eaur cclye lwli be i?fen +2

yb_26 )1 eiaomypemrahnm si seen in lal reau ccely soirsredd teepxc aegrnias )icen ie2ydfc nraiocg csadi dietlcry tnhibii euar cyelc =g;t& ehpmrnmaayimeo (omrf dl)oWrU +2

yex gconcdirA to ,UW eetrh is toahrne tieuosnq # 3.114 This eno efrers ot temcyonihlmla iiecamda CA(OIRNG EIAD.)AMIC GPEYYLIMCHOA rselsut rmfo llovaer ceiesandr alibmeotc aert eadinlg ot eeidsrnca eguoscl ittunizaoil nda riectd toxic )(- fo inglsognoueesec yb irnoagc s.adic Teh esneepcr fo cylygmahoiep dsael to eaecdirns eefr atyft acdi mmeslboati htat soperduc KNSEETO, tnsgirelu ni a uhfretr anino apg met caiicso.d yFalinl, gacirno asdci osal tlriydec (-) eht aeru cyc,le ilgeand ot OPMHEYE.AMRINMA +19

ih8payingfordis I nktih trehe has eneb mose siocuonfns eseabcu FA deso a proo bjo inegrgtatin tseeh tpcecnso goreteht ubt rhee rae soem ersnfee:ecr AF 9012 gp 5:8 oicopniPr daceiimA th(si si eon pmaxlee fo norigca idac eolmibtasm oerr)ddsi nI teh ryasmmu rgeiuf on gP ,47 loko at the pornite tesoabmlim ald(ctoe mbtoot tihg)r. icsaylBal, you nac veah odrdirsse weerh uoy eahv pnoPAyil-ooCr uulpidb enaol OR ynP-ooirClAo adn oo-lmClanhMleAyyt du.builp Ad n etesh gnircao adiasimec aer aoomlatsu rcesseive in rtehi atecnireihn tpr,tneas hihcw fit hwit eth esmt wr'ee eving ie( XX sldiiniudva cna eb Ofce ndeetf)a ngtih thta UW tates that I od not eahv an aainntlxpoe orf: het uludbpi fo anriogc asidc bihtiin teh earu clyce nheec teh ecnsriea ni aoamnmi enona(y noskw hwo tshi a?np)phse +

aaftabsethi1 anC eenmsoo lelt me yhw tno SLAME ? +1

jaramaiha @sthaiafteab cebeasu lal fo teh ilsgbsin owlud oshw smeo potysmsm if it reew to be a ionmtridclaoh .aisesde hWihc sih hetorbr si ,lehhayt dan stiser was tlfr-umel but eddi deu to endsueficip suasc.e +

utap2001 Why aisodsci scuea iylgepmcyaoH nad sseKoti dan irohmnypaema? In dasoc(iishcsu alcitc i&sotg)-dsic;a tdciinea yobd regeyn csiisr g;t-& n.ci ,slcgoiyysl cpe-&ago;ityymlgH cin. toeinrp k,ewdrnboa mHanraoypime g;-&t e.dc CTA ccley, oeKists +

forkyeaa osew.c/u.n///r/lte7anggcsni/Cpwg.t2w23Pbl:t1hi/hrc.mii6/pFfMvm28i +

+8 upvote downvote
submitted by ∗powerfulgarbage(17)

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uJts to dda ot wtha emos steorh evah said, isht si in srtiF Adi sa .ellw AF 0220 paeg 58:

Oiacgrn sacdmeaii msot onmmylco etpners ni yifacnn wtih ropo fdnei,ge gi,tomniv pnoayto,hi a ighh nnoia gap ilcboemat iiosad,sc y,maehotlpgea nda rzse.iues

hTe coigran dcai nuuciloacamt dseo 2 :isnhtg

ncrOgai dicsa itiibhn ogesenlgcioesun, eligdan to nstiafg loayeicyhgmp chwhi tenh csesua ioo,saictksde asngcui a hghi GA lioacmetb acsiosdi
gncaOri dasic ihnibit eht aeru cecly, nlideag ot ohmaaeemmprniy

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forkyeaa hits nicb lothcarwf swohs hwo teh ccesly are hnibeitid. tmghi p.leh i7ahfFlcPccnrwp/eii.b/8nwitttngevr.3/2uwslimh//2CM.p2go/mg./16s:/ +

+2 upvote downvote
submitted by ∗sattanki(82)

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aCn’t ephl uchm on eht xaect snginearo yhw, ubt hrete aer a few rUodWl suiesotnq on hsti eherw fi a enonaet has oiamceyylghp, esoikst and iyneaaep,ommmhr a nrciaog cdai osddierr sdlhou be ssecedput cirpoo(nip iadc or lymoenchimalt .)dcai Lsse ipuciusoss of an RTA ecsau ieyhomgpacyl is not tircaitcacsrhe of .htta

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sweetmed mI gamsiusn cbeuase aaact-uetNymltelgt si na siearcllto acovrtait fo CPS I eneedd ni euar e.yclc dan ANG- is emda of metuatalg dan elatyc .ocA So ni ncrigao ,iescmaida all teh aeclty AoC is iegnb dseu to akem tnkeoes for ernyge sneci cgnsulieeneosog is sdmees p.u oS rUae ceycl tsendo owkr as elwl and NH3 cuualamsetc +4

+1 upvote downvote
submitted by passispass(1)

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yM seagnnroi on siht niossqtue aws emrplsi - I hktin tehy nadtwe us ot kicp na aloamtuso ieeesrcsv eissaed esdba no paettrn fo kids efdecfta oh(tb emslfea ear eftfdeca - tanc' eb ynnigtha dkln-)ei;X yob aws yhtaehl - lure tuo nmdotoic.ihrla hWit rset - rleu otu bseaeuc fo rnsieceda iomaamn adn leatinnif e.rntoispenat lnyO ocairgn adic ltmaimsobe irdodesr ltfe

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submitted by ∗forkyeaa(1)

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.3./v87cFe/c/oCti/m:wl.2/ehrPMaiw1/gc/b2ihifgsplniuwgntns6.pr2m/t

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-2 upvote downvote
submitted by ∗abhishek021196(119)

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hitnireOn srtaysaaecmnlabr detoeycf. cisniM nommoc euar lceyc rdo.disre ielXkdn- evcesisre vs( ethor urae yccel ezymen ecdifee,isnic ichhw era amtusoola sce.sirev)e

efsrIreent twih hte osy’bd alytiib ot nimeitela nmoa.iam

tnOef ieetdvn ni het itfsr fwe dyas of ie,fl utb may npretse t.lare sExecs aombyalcr ohsetpaph is ttdcoenrve o ciootr ciad atp(r fo the yempdiniir nsstyeish )tw.ahayp

Fsng:inid rotsneidIeocrc a ciad ni oldbo dna eir,un asUeB,dreeDcN smmyspot fo iehmnroammpe.ya lot esamgblcoaNi aiaenm (sv itrooc a)i.ruicda

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passispass naC ti be na inlXdke- sedasie if tboh lesfaem rea fceafdet adn yob si ton? I ktnih knlyleui +

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