Male psuedo-intersexuality/hermaphrodite: conflict b/w FA and Kaplan?

[Knicks]

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The 2006 FA says the "most common form is androgen insensitivity syndrome (testicular feminization)", in which testosterone levels are high.

However, Kaplan says the "most common cause is inadequate production of testosterone and MIF by the fetal testes. This is due to a 5alpha-reductace deficiency".


So which is it?

 

[Quizbowler1]

I read in a different source (BRS Phys I think) that androgen insensitivity syndrome is the most common, but that both are exceedingly rare compared to female pseudohermaphritism.

Also, 5a reductase deficiency and androgen insensitivity should present differently I beleive. Since you have functioning testosterone in 5a reductase, testicular descent will occur while it will not in insensitivity. So you should have testicles in the "labia majora"

 

[TRKATK]

I always thought that the androgen insensitivity was more common than 5-alpha.

they do preset differently. In the deficiency of the androgen receptors you have intra abdominal testes, normal external female genitalia, but absence of uterus& cervix as well as pubic/axillary hair. Since DHT is not produced due to receptor deficiency the testes don't descend. You don't have a cervix/uterus because MIF is made by the testes :. ext female genitalia appear and no pubic/axillary hair because you need testosterone for that. High testosterone due to no (-) feedback.

With 5 alpha deficiency you have a male genotype but ambiguous genitalia. the testes descend enlarged penis but selective virilization because it can be a partial enzyme deficiency. And since 5alpha converts it to DHT, and DHT is needed for ext virilization and development of secondary male characteristic @ puberty.

 

[Knicks]

but what is it with regards to testosterone levels? high or low? (refer to the 1st post)

 

[TRKATK]

You should have an elevated level for both. I would think more so for the androgen insensitivity as there is no negative feedback for the testosterone being produced where as 5-alpha testosterone there is a build up of testosterone b/c some of it cant be changed to DHT via 5alhpa deficiency.

 

[Docster]

The 2006 FA says the "most common form is androgen insensitivity syndrome (testicular feminization)", in which testosterone levels are high.

However, Kaplan says the "most common cause is inadequate production of testosterone and MIF by the fetal testes. This is due to a 5alpha-reductace deficiency".


So which is it?

Take a quick look at pages 100-1 of Goljan RR. Testicular feminization is the most common cause of male pseudohermaphroditism, and it is due to androgen-R deficiency. Goljan states that there would be NORMAL levels of testosterone and DHT.

 

[Knicks]

Man, all these sources are saying something different about testosterone levels. 😡

 

[TRKATK]

Take a quick look at pages 100-1 of Goljan RR. Testicular feminization is the most common cause of male pseudohermaphroditism, and it is due to androgen-R deficiency. Goljan states that there would be NORMAL levels of testosterone and DHT.

I don't know if we think about it, her testosterone receptors are absent in the hypothalamus and anterior pituitary so there is nothing feeding back to stop the level of increasing testosterone. But, since she doesn't have any testosterone receptors she develops female genitaila.



maybe someone with more expertise can chime in