How common is intersex? A response to Anne Fausto-Sterling - Sax - 2002 - Article

When a child is born with both female and male genitalia, that is called intersex. Fausto-Sterling suggests that human sexuality is best understood as a continuum. "Deviation from the Platonic ideal" is a criterion for defining intersex created by Fausto-Sterling. However, some people are mosaics - some cells in their bodies have both the male and female chromosomal complements. According to Wiener, a more useful definition of intersex is: (a) the phenotype is not classifiable as either male or female, or (b) chromosomal sex is inconsistent with phenotypic sex. The main intersex conditions are congenital adrenal hyperplasia and complete androgen insensitivity syndrome. In the first condition, a defect in an enzyme involved in the synthesis of adrenal hormones gives rise to excessive production of androgenic hormones.

When a child is born with both female and male genitalia, that is called intersex. Fausto-Sterling suggests that human sexuality is best understood as a continuum. "Deviation from the Platonic ideal" is a criterion for defining intersex created by Fausto-Sterling. However, some people are mosaics - some cells in their bodies have both the male and female chromosomal complements. According to Wiener, a more useful definition of intersex is: (a) the phenotype is not classifiable as either male or female, or (b) chromosomal sex is inconsistent with phenotypic sex. The main intersex conditions are congenital adrenal hyperplasia and complete androgen insensitivity syndrome. In the first condition, a defect in an enzyme involved in the synthesis of adrenal hormones gives rise to excessive production of androgenic hormones.

These androgens will masculinize a female (XX) fetus in utero. In the second case, the individuals are genetically male, but owing to a defect in the androgen receptor, their cells do not respond to testosterone - they do not form male genitalia. Later on in the paper, the non-conditions for ''intersex'' are discussed. In late-onset congenital adrenal hyperplasia, the defect in the enzymatic pathway does not manifest until late adolescence or even later. From a clinician's perspective, LOCAH is not an intersex condition. The most salient features of Turner syndrome are infertility and short stature.

Girls with Turner syndrome can achieve normal adult heights if daily doses of growth hormone are administered. Babies born with Klinefelter syndrome have normal male genitalia. Most men with Klinefelter syndrome are infertile, but an unknown proportion are fertile. Fertile men with Klinefelter syndrome are likely to go completely undetected. The procent of intersex cases is 0.018% - approximately 100 times lower than the number provided by Fausto-Sterling. Szasz suggested that the mental disorder is not a natural phenomenon but a concept created by society. However, this type of thinking is not useful in clinical practice.

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