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...
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Some genetically male children do not grow penises until they are 12 years old. In the Dominican Republic, these children are recognized as Guevedoces (which literally means “penis at 12”). Throughout their childhood, they look, for the most part, like little girls. However, when they hit puberty, they begin to develop more male features, including a penis and testes.
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IFLScience reports Sept. 21 that the phenomenon is due to what is medically known as 5-alpha-reductase deficiency, a condition that affects male sexual development pre-birth and into puberty. Genetically, children with the deficiency are male but their bodies have not produced enough dihydrotestosterone (DHT), which impedes the development of external sexual organs.
BBC News reports about a child -- once named Felicita, now named Johnny -- who, like many other Guevedoces from his small village, has been brought up as a girl. Johnny’s transition has been a rough one. When he was younger, he never liked wearing girl clothes or doing girl things after he turned 7. He wanted to dress like and play with the boys. Then, when fellow students began bullying him because of the change he went through at puberty, he felt that he had to take action: “They used to say I was a devil, nasty things, bad words and I had no choice but to fight them because they were crossing the line," he said.
In the village of Salinas, there are many children going through similar transitions. It is estimated that 1 in 90 genetically male children have been brought up as girls until puberty when their external organs begin to develop. These children, when born, have what looks like vaginas. Until puberty, it is difficult to tell the difference.
British television journalist Dr. Michael Mosely writes that a team filming for the BBC2 series Countdown to Life, a program that “looks at the consequences of normal, and abnormal, developments in the womb,” found Johnny.
One of the first people to study the deficiency was Dr. Julianne Imperato, an endocrinologist at Cornell University. She had heard rumours about girls turning into boys in small villages of the Dominican Republic. She traveled there and learned that the phenomenon was due to a deficiency in 5-α-reductase which are enzymes involved in steroid metabolism and which normally convert testosterone into DHT. In the case of Guevedoces, until their bodies develop more testosterone during puberty they are also not fully developed as males.
Mosely writes that “Apart from being slightly undersized everything works and the Guevedoces normally live out their lives as men, albeit with wispy beards and small prostates.”
By chance, Merck, an American pharmaceutical company, found Dr. Imperato’s research and have developed a drug called finasteride. The drug, which blocks 5-α-reductase, is used to treat non-cancerous prostate enlargement and male pattern baldness.
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