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Organisation Intersex International

OII
Progestin Induced Virilization (PIV) - Similar to CAH is Progestin-Induced Virilization ('PIV'), which results from an abundance of male hormones in an otherwise normal XX female. PIV is caused by exposure in-utero to progestin that has been taken by the mother during pregnancy. Like individuals with CAH, PIV women will frequently have clitoral hypertrophy. In all other respects, however, they have completely female gonads.  Progestin is a drug which was administered to pregnant women in the 1950's and 1960's and is converted to an androgen by the prenatal XX fetuses' metabolism. Sometimes the genitals of fetuses affected by progestin are virilized with effects ranging from enlarged clitoris to the development of a complete phallus and the fusing of the labia. In all cases ovaries and uterus or uterine tract are present, though in extreme cases of virilization there is no vagina or cervix, the uterine tract being connected to the upper portion of the urethra internally. The virilization only occurs prenatally, and feminizing puberty occurs due to normally functioning ovaries.

Discovery: circa 1970
Usually Diagnosed: as newborns or during puberty
Frequency: unknown
See  http://www.isna.org/faq/progestin.html

DES Exposure - DES is a synthetic estrogen that was given to millions of pregnant women between 1937 and 1971 because the medical profession believed it would help prevent miscarriage and insure a healthy full term pregnancy. A 1957 advertisement claimed desPLEX® would "prevent ABORTION, MISCARRIAGE and PREMATURE LABOR…recommended for routine prophylaxis in ALL pregnancies…" This was only one of numerous brand names that DES (diethystilbestrol) was sold under.

DES did not work. In fact, the unfortunate use of this drug has resulted in severe risks and consequences to the health of many of the women who took the drug, as well, the daughters and sons whom they bore.
Surgical Creation of an Intersexed Condition:  In addition to cases in which intersexed individuals may be assigned a sex that does not comport with their own sexual identity, some persons have had their sexual features altered either accidentally or purposefully. For instance, some individuals have had their penises removed at a young age because they were mistakenly identified as females and the penis was considered an oversized clitoris that required reduction. Although these cases are rare, they are illustrative of the complex nature of sexual identity.

The most famous surgical alteration case involves a male whose penis was accidentally ablated when he was eight months old. The doctors recommended that his genitals be reconstructed to have a female appearance and that he be raised as a girl even though all other sexual factors were congruent and were male. The doctors also recommended that his 'history' as a male be hidden from him.

This surgical alteration case made headlines in 1973. Because the doctors involved in the surgical alteration reported that the child and the parents had successfully adapted to the sex/gender alteration, sociology, psychology, and women's study texts were rewritten to argue, 'This dramatic case . . . provides strong support . . . that conventional patterns of masculine and feminine behavior can be altered. It also casts doubt on the theory that major sex differences, psychological as well as anatomical, are immutably set by the genes at conception.'

For more than twenty years, the scientific literature continued to report that the surgical alteration was successful and the child's sexual identity was female. This case made headlines again in 1997 when Milton Diamond and Keith Sigmundson reported in the Archives of Pediatric & Adolescent Medicine that the boy who was turned into a girl was now living as a man.

According to the Diamond and Sigmundson report, John (a pseudonym) had always thought of himself as different from other girls. As a child, he preferred 'boy' type toys and preferred to mimic his father's rather than his mother's behavior. He also preferred to urinate in a standing position although he had no penis. Because of the cognitive dissonance, Joan (a pseudonym used by the authors to describe John while he lived a female life) often had thoughts of suicide.

At twelve, Joan was put on an estrogen regimen. She rebelled against the regimen and often refused to take the medication. At fourteen, Joan confessed to a doctor that she had suspected that she was a boy since second grade. At that point, the doctors agreed with Joan that she should be remasculinized and become John once more. At age fourteen, Joan/John returned to living as a male. He received male hormone shots and a mastectomy. He underwent surgery to reconstruct a phallus at ages fifteen and sixteen. John was eventually accepted as a boy by his peers. He is now married and helping to raise his wife's children.

John was not born an intersexual. He became an intersexual when doctors removed his penis, constructed external female genitalia and administered female hormones. Despite this intervention, John always felt that he was not a female.

In another recently reported similar case, a child's penis was severely damaged during a circumcision that was performed when the child was two months old. A decision was made to 'turn' the child into a girl. At seven months, surgery was performed to remove the remainder of the male genitalia and from that point on the child was raised as a girl. She was interviewed by a psychiatrist at ages sixteen and twenty-six. The results of these interviews indicate that she self identifies as a bi-sexual female whose recreational and occupational interests are more typically identified with males.

The significance of these two reports is that they exemplify the difficulty law and medicine must confront in defining sex. At birth, these infants' sex factors were congruent and were male. After the original intervention, they were turned into intersexuals but were treated by society as if they were females. They had male chromosomes, ambiguous genitalia, and female gender assignment. As adults, one person self-identifies as a heterosexual male while the other self-identifies as a bi-sexual female.

These studies and other reports about intersexuals have forced the medical and psychiatric communities to question their long-held beliefs about sex and sexual identity. Just as current scientific studies have caused the scientific communities to question their beliefs about sex and sexual identity, the legal community must question its long-held assumptions about the legal definitions of sex, gender, male, and female." Greenberg, Defining Male and Female: Intersexuality and the Collision Between Law and Biology, 41 Ariz. L. Rev. 265, 278-92 (1992).

Swyer Syndrome: Pure gonadal dysgenesis is a condition sometimes referred to as Swyer Syndrome. This syndrome is similar to Turner Syndrome in that individuals with this syndrome will have only streak gonads. In contrast to Turner Syndrome, in which a chromosome is missing (XO), individuals with Swyer Syndrome have XY (male) chromosomes. Although Swyer Syndrome individuals have a Y chromosome, the chromosome may be missing the sex-determining segment. Without this segment, the embryo cannot develop testes and as a result, the masculinizing hormones are also missing. In the absence of the masculinizing hormones, the fetus will take the 'default' female path and will develop a uterus but will not have any ovaries.

This condition is not apparent at birth and the child will be raised as a girl. The syndrome is generally diagnosed at puberty when the absence of a menstruation and breast enlargement causes suspicion.
See http://www.emedicine.com/radio/topic666.htm
Timing Defect:   If all of the proper stages of normal male sex differentiation occur, but the timing is incorrect by just days, errors may arise. The occasional outcome in a 46,XY individual with this timing defect is ambiguous external genitals.
Testicular Dysgenesis Syndrome - that is what it is called - symptoms are cryptorchidism, hypospadias, poor sperm quality (infertility & possible ovarian testicular tissue), testicular cancer.  This is not the same as mixed-gonadal or XY-gonadal dysgenesis or AIS or XXY or any other syndrome - it is what it is, and unlike exposure to DES, it is believed to be the result of exposure to Environmental ECD's.

See:  http://www.mindfully.org/Health/Testicular-Dysgenesis-Skakkebaek.htm
Triplo X (47, XXX): This is a condition affecting females, sometimes also called "hypofemale" or "super female." The physical manifestations of Triplo X are that the woman will usually have larger than average breasts with wider spaced nipples, a narrow (wasp) waist, wider hips than average, and a height of usually more than six feet. Some girls are also born with a "webbed neck" that is easily surgically corrected. This body type is sometimes describes as "Vampira" (the 1950's late night horror movie hostess) or, more recently, like that of the cartoon character Jessica Rabbit from the movie "Who Framed Roger Rabbit." By most standards Triplo X females are quite attractive, proving that a genetic/chromosomal abnormality need not be considered a "deformity." However, learning disabilities or developmental delays are not uncommon.

Discovery: 1959 by various researchers
Usually Diagnosed: any time from birth to adulthood
Frequency: 1 in 1,000 births
See  http://www.triplo-x.org/doc.asp?document_id=1295