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 testicular feminization  



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Author10 Posts
  #1

SXR :idea:

Q : what will be the levels of testosterone/DHT/ LH in serum in a patient with testicular femnization??




  #2

Deficient androgen receptors.
Testosterone unable to form "SEED"
{via mesonephric duct(Wolffian duct)}
Sem.vesicles, Ejaculatory duct, Epididymis, Ductus deferens
ext.genitalia are female, rudimentary vagina(testis in inguinal canal/labia
majora
*testosterone, DHT, and FSH are normal, LH is increased
*LH doesnt respond to the negative feedback of testosterone


  #3

excellent i agree with you. 8)

but FA 2003 says that testosterone levels are high

i don't agree with FA b/c the feminization is due to unopposed action of estrogens.testosterone is normal but not-functional receptors unfortunately..

what do you think ?


  #4

I think it is called OVARIZATION :-)


  #5

On a more serious note...
testosteone induces production of Inhibin,... so if no stimulus to testicles .. no inhibin...FSH goes up...testorone goes up.


  #6

huh??? test & dht are up or normal but has no effect... mif is also secreted that's why there are no internal genitalia vagina in a blind pouch... this has nothing to do with estrogen.... and presents as 14 year old with primanry amen and no breast development.... "vagina ending in blind pouch"

a def of 5 alpha reductase would cause a high test:dht ratio and that is how u distinguish between type 1 and 2 male pseudoherm.


  #7

adeelmd is on the right!
"In this ambiguous area the labs are ambiguous"
I looked in Harrisons. In complete and incomplete testicular feminization
testosterone , estrogen and LH are all increase. Elevated testosterone is due to a high mean plasma LH. (no negative feedback) LH also causes increased estrogen. Gonadotropic hormones are even higher.
(by the way these are x-linked recessive disorders) DHT and inhibin are not mentioned.
Also in 5 alpha reductase def. testosterone and estrogen are normal and LH is normal or high
Lastly in 5 enzyme def. testosterone is normal or decreased, estrogen is variable and LH is high
(innormal men estrogen is derived from peripheral formation of circulating androgens but when theplasmaLH is elevated, the testis secrete increase estrogen into circulation.
As far as the lab parameters we know that DHT is formed via 5 alpha reductase and testosterone.
*Also DHT is responsible directly for masculinization of external genitalia and of urogenital sinus. Wheras testosterone :arrow:" Wolf duct"

In this case much to my surprise looks like "1st Aid is Correct and Goljan is
at best ambiguous or incorrect" He incorrectly states testosterone is normal!

***The main Pathophysiology is impairment of DHT formation in a male embryo would be expected to cause the phenotype in this disorder.
**Remember "normal Wolf with defective masculinization of external genitalia.

**Overall there is ambiguity and I doubt that the usmle would ask an ambiguous question on ambiguous labs. But I could see them asking a structural or anatomical question.
Last thoughts-21,11, 17,3 hydroxlase deficiencies would be good distractors.


  #8

a xy with a vagina and NO internal female organs can only be eith tf (type 1) or 5 alpha (type 2) male psudo... if test is much greater than dht, than it's type 2... if not it's type 1. 17 def would have htn and increased corts thrown into the mix right??


  #9

"mjl1717" wrote:



In this case much to my surprise looks like "1st Aid is Correct and Goljan is
at best ambiguous or incorrect" He incorrectly states testosterone is normal!

.



thanx tó this spectacular forum the area is cleared.who is right or not smiling face this is also a little surprise for me too
thanx guys smiling face


  #10

Yeah, Mars thats the great part of the forum . We can share ideas and information to reach a solution. Great!!

Adeelmd - As far as 17 OH def. -BP is increased, decreased aldo. but increased DOC.,adrenal and testicular androgens are decreased and estrogen is decreased. Contrary to what you say the cortisol is decreased.
Actually the cortisol is decreased in all 3( 21,11, and 17 hydroxylase enzyme deficiencies)
*I find this as one of the more challenging areas for me and wouldnt be surprised if we constantly see qs about this.





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