Güevedoce

In the Dominican Republic, güevedoces (from güevedoce, from Dominican Spanish güevos a los doce "testicles at twelve") are children with a specific disorder of sex development. Güevedoces are classified as girls when they are born but, around the age of 12, they start developing male genitalia. This is due to a deficiency in the production of 5α-reductase, an enzyme involved in the metabolism of testosterone to dihydrotestosterone. The same phenomenon occurs in Papua New Guinea, where it is called kwolu-aatmwol (literally 'a female thing changing into a male thing') by the Sambia people, and in Turkey. Anne Fausto-Sterling states that güevedoces (as well as people in Papua New Guinea with 5α-reductase deficiency) "are recognised as a third sex" by their cultures, while the cultures "nevertheless recognize only two gender roles". The first scientific investigations of güevedoces occurred in the 1970s, when Julianne Imperato-McGinley, an endocrinologist from Cornell University, traveled to the village of Las Salinas in the Dominican Republic to investigate reports of apparently female children becoming male children at the onset of puberty. The cause was determined to be 5α-Reductase deficiency, and the results were published in the journal Science in 1974. The frequency of the deficiency was found to be unusually high in Las Salinas, with occurrence ratio of 1 güevedoce to every 90 unaffected males. The transformation of a phenotypically female child into a phenotypically male adult at puberty, which is reported to be celebrated in Dominican culture, is the result of a genotypic male (with XY chromosomes) born with a deficiency in the enzyme 5α-reductase. 5α-Reductase is responsible for the reduction of testosterone to dihydrotestosterone. Dihydrotestosterone (DHT) is the hormone responsible for the majority of embryonic development of primary male sexual characteristics such as genital size. However, the oxidized form of the hormone, testosterone, promotes secondary sex characteristics.