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NBME 21 Answers

nbme21/Block 1/Question#30 (44.3 difficulty score)
A newborn has female external genitalia and a ...

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submitted by sinforslide(49),
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leaM nnraietl eagtniail t&-;g tntIac SRY , s,esett nda

No emafel enlnarti lgatnaiei tg&-; senerePc fo IMF na(unmielrtial o)onmher dan ctatin Selroit lcel ntfioncu.

leFmae lentxrae lntageiai &;gt- oN aogdnenr esetnrp, hhicw is derqurie rof lame trnaxeel ianaieglt mtainoro.f

d_holles  Not sure I understand why T is wrong, but DHT is correct. +1  
d_holles  I thought about this some more -- DHT forms external genitalia while T forms 'male genital ducts'. That's why the correct answer is DHT, not T, since the PT had +ext genitalia, but -internal genitalia. I was thinking that the PT had CAIS, but that would lead to testes only w/o male genital ducts. See FA2019 p608. +18  
d_holles  *I meant -ext genitalia, +int genitalia +  
adong  T is wrong because you still need T to make the internal male organs which he has based off the MRI +2  

submitted by mcl(586),
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erP 680p in AF ,9021 RSY no Y omocsehmor susterl in vetdmelpeno fo te.sset HDT lsusret ni eoetmvdnpel fo mlea arlentxe aiegntlia n(ad hte et.sprtoa)

mrsmac  No sertoli cells or lack of mullerian inhibitory factor makes more sense. bc there is both male and female internal genitalia but only male external genatalia. and karyotype would show 46XY. First Aid 2018 pg. 604 - the "Sexual Differentiation" charge delineates exactly this. If it were 5areductase deficiency the child would have testicles and scrotum, which in this case is absence. Hope this makes sense. Please let me know if you disagree and why. Thanks. +  
mixmasta  I believe the tricky part is that they don't mention the status of the Male external genitalia. Pg. 605 from FA ( bottom portion) shows the external development of the Male/Female genitalia; you see DHT is need for male. Furthermore, pg. 604 (SEXUAL DIFFERENTIATION) DHT is also needed for Male external development. +  
niboonsh  My understanding of this is that the diagnosis is 5alpha reductase deficiency because the newborn has female external (aka ambiguous) with male internal (aka "male genital ducts"). According to FA, leydig cells produce testosterone, which can either stimulate the mesonephric duct to form the INTERNAL male genitals (as see in the pt). Testosterone can also be acted on by 5alpha reductase to become Dihydrotestosterone, which forms the male EXTERNAL genitalia. Since this kid has "female" genitals, but has male insides and is 46XY, id say this is a simple case of 5alpha reductase deficiency. No sertoli cells or no MIF would present as both female and male internal (because MIF typically inhibits differentiation of female internal) and male external genitalia (bcuz leydig cells are unaffected) +15  

submitted by basic_pathology(8),

Y chromosome --> SRY gene --> Testis --> Sertoli and Leydig cells.

Sertoli cells: AMH --> involution of mullerian ducts --> no female internal organs

Leydig cells:

  • Testosterone: development of Wolffian duct (SEED) (seminal vesicles, Epididymis, Ejaculatory Duct, Ductus Deferens).
  • 5a-reductase --> DHT: male external genitalia (penis, prostate)

Everything is normal, except for external genitalia. DHT signalling is missing.

submitted by yo(79),
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nagD thsi quneitso rehwt em fof beueacs it aids mleefa inatligae ehwn I htoghut it dlwou be aoumbgsui at tbr.ih THD iltsl mesak hte toms .nsese nakhsT.

submitted by et-tu-bromocriptine(114),
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nEghrityev ash eben cvodree ,edalary btu e'hrse to hte ialsuv slaerrne - ts'i yase to see eth troinseiphla wbeenet HTD and estn.otesretom8od/3.:ga/Fihptt/McY/smurm

(rnoieg hte ewloh lbakc vrsues ebul cpitemcoasl gntihs dna 'mI sure I modicolres o)re.emshew

submitted by frijoles(13),

I chose E) because I was thinking androgen insensitivity syndrome. However, I realize the wording of the question is which HORMONE would be reduced and in AIS, testosterone levels would not actually be reduce.

To be clear though, AIS could have the EXACT same described presentation, yes? (46.XY with male genital ducts & female external genitalia) Just want to make sure there is nothing else that could have tipped me off. Thanks.

daddyusmle  Yup you got it. AIS is a defect with the androgen receptor in target tissues, not with the hormones. AIS has the same presentation: Genotypically XY but has female external genitalia and male internal genitalia. This is most likely 5-alpha reductase deficiency. +