SANTO DOMINGO, Sept 20 — In the village of Salinas in the Dominican Republic, one in 90 girls possess a rare genetic disorder that causes them to become boys at puberty. Known as “guevedoces”, or “penis at 12”, the children are born with external genitalia that appear female but develop male sex organs once they hit puberty.
The BBC reports that this condition was first studied by Cornell endocrinologist Julianne Imperato in the 1970s and appears to be the result of a 5-alpha-reductase enzyme deficiency that prevents the hormone dihydro-testosterone (DHT) from being formed in the womb. Sex organs are typically developed during the second month of pregnancy. In males, DHT is responsible for the formation of the penis. Females do not produce DHT and thus develop a clitoris. The guevedoces, who are missing the 5-alpha-reductase enzyme, are unable to produce DHT and so appear female when they are born.
According to the US National Library of Medicine, once puberty hits, another surge of testosterone hits the body, and this time the penis and testes form fully. The new organs, though undersized, usually work fine, and individuals tend to live out their lives as men. The condition is rarely seen outside the Dominican Republic, where it is so common that the country now believes there are three sexual categories: male, female, and pseudohermaphrodite. — Reuters
