Human protandry (girls who turn into boys) revisited

I have posted a couple of articles on human protandry and interested readers should probably read the earlier stuff first.  The phenomenon is a marvellous natural experiment proving that “gender” is NOT a “social construct”—as the academic feminists would have it.  The academic feminists themselves are not interested in evidence, of course, but those who have to cope with them may be.  I must say I was amused, though, to see that a lesbian site had one account of the phenomenon posted.  Apparently, the idea of girls turning into boys sounds pretty reasonable from a lesbian perspective!

Anyway, below is an excerpt from a medical source  which explains how human protandry happens:-

“De Vaal (1955) reported 3 brothers who were thought for a time to be girls. The parents and grandparents on one side were first cousins, and the great-grandparents were also related. Simpson et al. (1971) described a family with 3 affected brothers whose parents were double first cousins. Each of the affected sibs had an XY karyotype and ambiguous genitalia, leading to rearing as females. No breast development or menstruation occurred at puberty, and instead typical masculinization was observed.

PPSH can be difficult to distinguish from the incomplete testicular feminization syndrome (ITFS; 300068), especially in the young child. The distinction is obviously important since PPSH is a male-limited autosomal recessive with a recurrence risk of 1 in 8, whereas ITFS is probably X-linked recessive (or autosomal dominant male-limited) as is the complete syndrome. Wilson et al. (1974) chose to refer to PPSH as type 2 familial incomplete male pseudohermaphroditism, type 1 being the Reifenstein syndrome (312300). PPSH resembles the most severe form of type I incomplete male pseudohermaphroditism, but differs from it by the lack of breasts and by its autosomal inheritance. Dihydrotestosterone (DHT) formation is defective in this condition. Testosterone and estrogen levels are normal, hence the lack of gynecomastia. Other evidence as well suggests that DHT is important to external virilization.

In a village in the Dominican Republic, Imperato-McGinley et al. (1974) studied 12 families with 22 male pseudohermaphrodites. The affected males were born with ambiguous genitalia and masculinized at puberty without breast development. The testes were normal histologically. The patients had no mullerian structures, complete wolffian differentiation, small phallus, bifid scrotum, urogenital sinus with perineal hypospadias and blind vaginal pouch. At puberty, they showed male habitus with excellent muscular development, voice change, enlargement of phallus and production of semen, but small prostate and scanty beard. Plasma testosterone was normal; plasma 5-alpha-dihydrotestosterone was low. An abnormally small amount of radioactive testosterone was converted to dihydrotestosterone. One woman studied showed the same biochemical defect.

The disorder has been found in blacks, whites, American Indians, and Latin Americans, as well as in families from Malta, Jordan, and Pakistan. Imperato-McGinley et al. (1991) described a cluster of male pseudohermaphrodites in the Simbari Anga linguistic group in the Eastern Highlands of Papua New Guinea. Their studies revealed a phenotypic and biochemical profile similar to that in patients studied in the Dominican Republic, except for a greater abundance of facial and body hair. DHT is responsible for masculinization of the external genitalia of the fetus and for masculinization at puberty. The virilization at puberty in PPSH may be related to the facts that the reductase is not completely absent and that low levels of DHT are found in plasma”.

Price et al. (1984) presented evidence that high dose androgen therapy may improve virilization, self-image, and sexual performance in patients with alpha-reductase deficiency who have male-gender behavior and in those patients with Reifenstein syndrome (312100) who have normal amounts of a qualitatively abnormal androgen receptor.

A number of male pseudohermaphrodites have married and expressed a desire to father a child. However, a deficiency in dihydrotestosterone production not only impairs differentiation of male external genitalia but also affects the development and secretory function of the prostate and seminal vesicles. Consequently, affected adults have a rudimentary prostate and underdeveloped seminal vesicles, resulting in a highly viscous semen and an extremely low volume of ejaculate, although sperm counts may be normal. Katz et al. (1997) described the use of intrauterine insemination with sperm from a man with this disorder and a history of infertility. The first pregnancy gave rise to a normal son; the second pregnancy produced fraternal twins. All 3 children were heterozygous for the father’s C-to-T mutation in exon 5 of the SRD5A2 gene”.

There is a more general discussion of the hormonal phenomena involved here

Posted by jonjayray on Tuesday, May 17, 2005 at 10:01 AM in No particular place to go
Comments (1) | Tell a friend

Comments:

Next entry: The feminist answer to the poor educational performance of boys

Previous entry: Nordic Beauties?