What are the other forms of intersexuality?

• Progestin-induced androgenization

  Caused by prenatal exposure to exogenous androgens, most commonly progestin. Progestin is a drug which was administered to prevent miscarriage in the 50's and 60's and it is converted to an androgen (virilizing hormone) by the prenatal XX persons metabolism. If the timing is right, the genital anlagen is virilized with effects ranging from enlarged clitoris to the development of a complete phallus and the fusing of the labia. The virilization only occurs prenatally and the endocrinological functionality is unchanged, ie. feminizing puberty occurs due to normally functioning ovaries.

  In other words, XX people affected in-utero by virilizing hormones can be born into a continuum of sex phenotype which ranges from "normal female with large clitoris" to "normal male with no testes". It is noteworthy that the use of progestin is not effective in the prevention of miscarriage.

• Congenital Adrenal Hyperplasia

  Caused when an anomaly of adrenal function (21-hydroxylase or 11-hydroxylase deficiency) causes the synthesis and excretion an androgen precursor, initiating virilization of a XX person in-utero. Because the virilization originates metabolically, masculinizing effects continue after birth. As in PIA, sex phenotype varies along the same continuum, with the possible added complication of metabolic problems which upset serum sodium balance. The metabolic effects of CAH can be counteracted with cortisone. The scenario for medical intervention for intersex is similar... but CAH people have an increased likelihood of early detection due to metabolic imbalances (Salt Losing Form).

• Androgen Insensitivity/Partial Insensitivity Syndrome

  In AIS/PAIS the cellular metabolism of an XY person is such that the cells do not respond to the effects of androgens. Endocrinological function is normal... but the cells ability to bind androgens, due to compromised receptor site metabolism, causes a partial or complete lack of response to virilization. PAIS produces effects similar to CAH or PIA in a neonate... genital ambiguity. With complete AIS a neonate shows no indication that they are intersexed as the external genitalia are completely phenotypically female. Internal female structures do not develop however because Mullerian Inhibiting Hormone is present and prevents the formation of a female genital tract (oviducts, cervix, uterus, part of the vagina).

  Another form of AIS is present in individuals with 5-alpha reductase deficieny. During the formation of the male genital tract from the Wolffian duct portion of the genital anlagen, the target tissues are not repondent to testosterone, another form, hydrotestosterone is required at this stage. The required enzyme, 5-alpha reductase, is missing so these people may be assigned and reared as girls. However, since pubertal genital tissue is sensitive to the effects of testosterone... such a child could experience masculinizing puberty and genital growth - assuming that their gonads have not been removed.

• Turner's Syndrome

  Turner's Syndrome children are born with an XO karyotype and, in the absence of gonads, develop without any endocrinological influenceing sexual phenotype. What this means however, is that they are phenotypically female and raised as such.

It is significant that these events... the discovery and "management" of our medical conditions occur as neonates, children or teenagers ie. during puberty. Our say in which physical "sex" we are intended to be ranges from very little to none.

Additional FAQS include:

• Intersexuality Frequently Asked Questions
• Parents of Intersexed Children FAQ

next: Sexual Development - Glossary of Terms