ANDROGENIZED XX HUMAN FETUS: ADRENOGENITAL SYNDROME
For obvious reasons one cannot experimentally manipulate the prenatal environment of human beings to produce cases of hermaphroditism or intersexuality as can be done with rodents or nonhuman primates. However, there are cases of human hermaphroditism (intersexuality) produced by nature, similar to those produced experimentally in animals.
One such form of human intersexuality occurring in XX females is the adrenogenital syndrome. This is a genetic autosomal recessive condition in which the adrenal cortex of the fetus fails to synthesize one of its proper hormones, Cortisol. Instead, it secretes an excess of androgen. In some cases, there is an associated deficit in salt retention. Affected females are born with a hypertrophied clitoris. In some cases masculinization is so complete that instead of an enlarged clitoris, there is a penis and empty scrotum.
In some instances, an XX adrenogenital baby is assigned and reared as a boy. But the more common practice, once the diagnosis has been established, is to institute a program of surgical feminization consistent with assigning and rearing the baby as a girl—even if a sex re-announcement is necessitated.
The endocrine treatment for all babies with the adrenogenital syndrome, discovered in 1950, is therapy with a synthetic form of Cortisol, the missing adrenocortical hormone. This therapy, if begun neonatally and continuously maintained, suppresses excessive postnatal androgen production. Then, at the expected time of puberty, girls with the adrenogenital syndrome develop a feminine physique. Their menses may be late, the time of onset being variable. They will ovulate in adulthood and can expect to conceive pregnancies.
Adrenogenital girls treated since infancy are of special interest to gender identity/role theory because of their history of prenatal androgenization subsequently corrected. Their psychosocial and psychosexual development has been followed longitudinally at The Johns Hopkins Hospital and at Children’s Hospital at Buffalo. The evidence to date is that fetal androgenization does influence subsequent development of behavior, though only to a limited degree.
Behaviorally, girls with the adrenogenital syndrome are described as having a high level of athletic energy as demonstrated by their interest and participation in organized group competitive sports, usually with boys. For example, they become involved in boys’ neighborhood football, basketball, or baseball. They are accepted by the boys because of their superior athletic skills. They do not assert themselves to obtain a high rank in the dominance hierarchy of boys.
Adrenogenital girls do not display much interest in dolls or stereotypic girls’ games. During childhood, they do not show much interest in babies, romance, and marriage, as compared to their unaffected sisters.
Girls with the adrenogenital syndrome show an interest in boys and dating much later in adolescence than do their peers. Divergent from their age-mates in this respect, they often become relatively isolated from them.
Recently it has been possible to obtain the first follow-up information on the psychosexual status of adrenogenital girls, now young adults, who have a history of being hormonally normal as the consequence of cortisone therapy started in infancy. Money and Schwartz (1976) and Schwartz (unpublished Sc.D. dissertation) report findings suggesting a greater incidence of bisexual and homosexual imagery than would be expected by chance. A replication study is needed.
Not all of the 46,XX infants with the adrenogenital syndrome are assigned and reared as girls. In the past, some were assigned and reared as boys, especially those born with a completely formed penis and fused scrotum as a result of extreme fetal androgenization. Although their counterparts who are reared as girls differentiate a female gender identity/ role, these boys differentiate a male gender identity/role, performing sexually as men with women partners.
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