kiranmayi
Forum Guru
Topics: 237
Posts: 407
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what happens in 5 alpha reductase deficiency?
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| gr_calif
Forum Junior
Topics: 3
Posts: 51
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The condition 5-alpha-reductase deficiency (5-ARD) is an autosomal recessive sex-limited condition resulting in the inability to convert testosterone to the more physiologically active dihydrotestosterone (DHT).
Because DHT is required for the normal masculinization of the external genitalia in utero, genetic males with 5-ARD are born with ambiguous genitalia (ie, male pseudohermaphroditism
The uterus and fallopian tubes are absent due to the normal secretion of the müllerian-inhibiting factor. Testes are intact, as are wolffian structures (epididymis, vas deferens, seminal vesicles). Male internal ducts are present but terminate either in a blind pseudovaginal pouch or on the perineum
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| usmleasr
Forum Guru
Topics: 105
Posts: 970
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good explanation 
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| peekay
Forum Guru
Topics: 102
Posts: 588
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isn't right also that these patient have female identity before puberty but after puberty they are more males and can have normal sex and reproductive.mostly seen cases are in Dominican Republic, a caribbean Island.
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| asmi
Forum Hero
Topics: 1043
Posts: 4,609
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to add some more info..
* @ puberty these people will demonstrate striking virilization due to increased T HT ratio...which is diagnostic .( normal is 5, these pts will show 20 -60)
*In genotypic XY fetus DHT is essential for the development of penis ,scrotum (external genitalia) and prostate gland , which are underdeveloped in these pts .
*they have normal epididymis , ductus deferens, seminal vesicle , ejaculatory ducts .
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