Guevedoche
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Guevedoche is an autosomal recessive genetic disorder which is characterized by a phenotypically female child (genotypically male) who at puberty will grow a penis and have testes descent. Ultimately this "once girl" now will appear to have a small penis where the clitoris once was, and this child will also have testes that have descended extremely late. The underlying cause of this dysfunction is a deficiency in the enzyme 5-alpha-reductase-2 which is largely responsible for the conversion of testosterone to dihydrotestosterone. Dihydrotestosterone is the chemical responsible for the development of male external genitalia and secondary sex characteristics. It is hypothesized that rising testosterone levels at the start of puberty (around age twelve) are able to generate sufficient levels of dihydrotestosterone by either the action of 5-alpha-reductase-1 (not normally active for this purpose) or through the action of any small levels of 5-alpha-reductase-2 which may be being produced.
The term Guevedoche or Guevedoces is Spanish slang originated in the Dominican Republic, where more than three dozen cases have occured in the small village of Salinas, all descended from a single individual named Altagracia Carrasco. It stands for the vulgar expression huevo a los doce, which translates literally as "balls at twelve". It is also known locally as 'Machihembras' ('first women, then man'). A similar cluster of cases among the Simbari of the Eastern Highlands of Papua New Guinea has the local name 'Kwolu-aatmwol' ('female thing transforming into male thing'). In the US, endocrinologists commonly refer to this disorder as 5-ARD, a form of male pseudohermaphroditism. See also third sex.
The condition is dealt with in the 1979 BBC documentary "The Fight to be Male" and the 1996 Swiss documentary "Guevote: The Way I Feel Is How I Am" ("Guevote: So wie ich mich fuehle, bin ich") by filmmaker Rolando Sánchez.
