For some individuals, determination of biological sex can be difficult. Intersex refers to genetically determined differences of the reproductive system. This can include differences in internal reproductive organs, external genitalia, or karyotype. Mild intersex conditions include, in males, a condition in which the urethra opens on the underside of the penis or, in females, an enlarged clitoris. Female intersexuals (karyotype 46XX) (also called female pseudohermaphrodites) have normal ovarian tissue, and have either male or ambiguous genitalia. This is usually a result of a change in the fetal adrenal glands, leading to production of abnormally high levels of androgens. The androgens produce some masculine features in female infants: ovaries and uterus form, but the external genitalia appear male-like. This accounts for about two-thirds of intersex states.

Male intersexuals (karyotype 46XY) (also called male pseudohermaphrodites) have normal testes with female or ambiguous genitalia. They most often result from several different genetic alterations in pathways of testosterone synthesis and metabolism. For example, males who have a mutation in the gene that converts testosterone to dihydroxytestosterone have normal testes but have a very small penis and a vaginal pouch. In gonadal dysgenesis the testes fail to secrete androgens or Müllerian-inhibiting hormone, leading to formation of female genitalia. With estrogen treatment, however, these individuals will grow into females. A condition called micropenis results from lack of androgens later in fetal life; testosterone treatment can stimulate masculizing puberty in these individuals.

Androgen insensitivity syndrome (AIS) occurs when a male produces cells that cannot respond to androgen. The defect is in a gene on the X chromosome that produces the androgen receptor. Individuals may have complete or partial androgen insensitivity. In complete AIS the testes develop in the embryo, and produce testosterone and the hormone that inhibits development of female internal reproductive organs (Fig. 4). However, because the cells do not respond to testosterone, female genitals develop, which may be incomplete. The newborn appears to be a female and develops external female characteristics at puberty. Lacking internal female reproductive organs, though, the individual with AIS does not menstruate and is infertile. In incomplete AIS, individuals may appear male or female, but there may be abnormalities in the external genitalia. Maria Patino, a Spanish runner with complete AIS, was not allowed to compete in the 1985 World University Games in Kobe, Japan because she failed the gender test. (See the Sex and Gender video.) Because of such difficulties in determining sex, the International Olympic Committee abolished gender testing in 1999.

Ethics of Intersex Treatment

Common medical treatments of intersex babies include:

    1. Assignment of gender based on a variety of clinical tests
    2. Surgery to remove internal gonads that might become cancerous
    3. Reconstruction of external genitalia appropriate for the assigned sex
    4. If necessary, treatment with appropriate hormones

Unless a penis is present, most intersex babies are assigned female because it is not possible to construct a fully functional penis. The gender assignment and the surgery are usually done immediately after birth, with possible additional surgery after puberty. Today, some physicians such as Eric Vilain (featured in the video) recommend allowing the child to make his or her own surgical decisions later in life. However, most parents choose the surgery earlier because they are uncomfortable with the ambiguity.

The Intersex Society of North America believes that intersex is not an abnormality but rather "an anatomical variation from the standard male and female types."3 The Society also believes that the decision regarding treatment, if any, should be made by the individual when he or she is capable of informed consent. The Society has two objections to treatment:

1) treatment assumes that intersexuality is a disease, and 2) surgery often damages sexual function, while still failing to produce anatomically normal genitals. They claim that physicians have traditionally failed to communicate to parents the basis for the assignment of gender, which is not always (and in the case of XY individuals without a penis, never) made based on biological sex. They also claim that some physicians have failed to inform parents of alternatives and presented elective surgery as essential for health.

Physicians were once guided by the idea that infants are gender neutral at birth, and that normal gender development would follow from the environment of the child based on the sex assigned to the child.4 They believed that gender came from "nurture" rather than "nature." In recent years individuals, especially intersexuals, have protested that the environment does not control gender and, given that the treatments are irreversible, they should be delayed until the child determines his or her true gender. Others worry, however, that children with ambiguous genitals will be ridiculed, causing permanent psychological damage that could be avoided.




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