Intersex Conditions

XX male syndrome, AIS:Androgen Insensitivity Syndrome and others
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  • XX Male Syndrome
    Female spirit, biology, bone structure, skull, jaw etc with penis and testicles. A recessive (both parents must have the mutation) trait passed on to children.
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  • Androgen Insensitivity Syndrome
    Males with testicular feminisation, testicles never fully descended. Vagina but no uterus usually. Another recessive trait that can be passed on to children. Generational male to female, automatic transgenders.
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  • Congenital Adrenal Hyperplasia
    Two hormones are critical in normal sex differentiation. The testes of normal 46,XY males secrete both Mí¼llerian Inhibiting Substance (also known as MIS or antimí¼llerian hormone) and masculinizing androgenic hormones, while the ovaries of a normal 46,XX female secrete neither. In CAH, the absence of a critical enzyme allows a 46,XX fetus to produce androgens, resulting in ambiguous external genitals. A CAH individual may have an oversized clitoris and fused labia. -pbs.org
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  • Testosterone Biosynthetic Defects
    In a condition related to CAH, some 46,XY individuals do not have the properly functioning enzymes needed to convert cholesterol to testosterone. When such enzymes prove completely incapable of creating testosterone, the genitals appear female; when the enzymes function at a low level, ambiguous genitals form. -pbs.org
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  • Gonadal Dysgenesis
    In gonadal dysgenesis, the androgen receptors are intact while the androgen-secreting testes are not. Complete Gonadal Dysgenesis, in which neither androgens nor MIS are produced, yields female genitals and Mí¼llerian duct formation, despite a genetic profile suggesting maleness. With estrogen treatment, female puberty can be achieved. Partial Gonadal Dysgenesis results in ambiguous genitals and duct development, as some androgens and MIS are produced. Like PAIS, the choice of hormone treatments determines the physical gender of the adult with Partial Gonadal Dysgenesis. -pbs.org
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  • 5-Alpha Reductase Deficiency
    5-Alpha Reductase is the enzyme that facilitates the conversion of testosterone to another hormone, dihydrotestosterone (DHT). When a genetic male is deficient in 5-Alpha Reductase, the powerful DHT hormone is not produced. While testes and Wolffian ducts do exist, the male external genitals are similar in size to those of a normal female. If left intact, an adult 5-Alpha Reductase Deficiency individual will appear generally male but with small genitals and no facial hair. -pbs.org
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  • Klinefelter syndrome—One in 1,000 births
    Sometimes chromosomes join but do not form standard 46,XX or 46,XY combinations. Individuals with Klinefelter Syndrome are genetically 47,XXY and live as men. Small penis and testes, low androgen secretion, and possible female breast development are characteristics of this syndrome. -pbs.org
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  • Turner syndrome
    Like Klinefelter Syndrome, Turner Syndrome is marked by an abnormal karyotype, 45,XO. While Turner women have female external genitals, the individuals lack properly formed ovaries. Without estrogen treatment, no breast growth occurs. Other possible features of Turner Syndrome include short stature, webbing of the neck, and misshapen internal organs.
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