A little help w/ hermaphroditism...

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So I'm not seeing a clear distinction between male pseudohermaphroditism and testicular feminization syndrome. Both are 46 XY, both are 'normal' appearing females w/ female genitalia and both have testes present. I know the mechanisms behind them are different (androgen insensitivity vs defective DHT receptor) but is there any other difference that I'm just not seeing? Do they present the same & can you distinguish them clinically?

Thanks!
2 days, 8 hours & counting!

 

[jakstat33]

hey- i expect ?'s about these 2 malformations to involve a basic understanding of the cause (male PH: no MIF/tes vs. AIS: as you said, defective receptor)... ummm, i also remember that with ais patients have a blind ending vagina, i am not sure if you see this with male pseudo (ambiguous genitalia).

then again, what do i know? i'll tell you if this shows up on my test Wed night, g'luck

So I'm not seeing a clear distinction between male pseudohermaphroditism and testicular feminization syndrome. Both are 46 XY, both are 'normal' appearing females w/ female genitalia and both have testes present. I know the mechanisms behind them are different (androgen insensitivity vs defective DHT receptor) but is there any other difference that I'm just not seeing? Do they present the same & can you distinguish them clinically?

Thanks!
2 days, 8 hours & counting!

 

[barone ROCKS]

look at it this way

if ur 46xy pseudoherm, you dont have mif so both female and male internal genitalia develop. the key here is that you still have testicles...and no ovarian tissue which seperates this from true herms

in receptor insensitivity you dont get female internal genitalia since mif is present and you dont get male internal genitalia due to receptor insensitivity. you do get female external genitalia ...2/3 vagina ending in a blind pouch.

my usual disclaimer...this is all from memory so if someone could confirm or correct my explaination...ty

 

[Idiopathic]

Male pseudohermaphroditism includes both androgen receptor deficiency/testicular feminization and 5-a-reductase deficiency (no DHT).

I think one huge difference is that LH will be off the charts in the first case, and not so in the second case.