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Guevedoche is a genetic disorder 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. A genetic disorder, or genetic disease is a disease caused by abnormal expression of one or more genes in a person causing a clinical phenotype. ...
The term Guevedoche is Spanish slang, originated in the American continent. It stands for the vulgar expression güevo a los doce, which translates literally as "cock at twelve". In the US, endocrinologists commonly refer to this disorder as "Penis at Twelve".
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