Genetic Disorder Causes Girls To Become Boys At Puberty: Dominican Village

La Salinas, a small village in the Dominican Republic, is home to a rare phenomenon where girls develop male genitalia either after puberty or by the age of 12, along with deeper voices and increased body hair growth, resembling males. Guevedoces are males who appear like girls at birth but develop penises during puberty, translating to ‘penis at twelve’.

This genetic condition is referred to as pseudo-hermaphroditism, where a person is born with the primary sex characteristics of one gender but later develops the primary sex features of the other gender.

According to the report, doctors from Cornell University visited the village to investigate the anomaly present there. Dr. Julianne Imperato-McGinley from Cornell Medical College in New York was among the pioneers in studying this rare condition.

As per their findings, Guevedoces initially lacked male genitalia due to a deficiency in the enzyme 5-alpha-reductase, which converts testosterone into dihydrotestosterone. In medical terms, the sex hormone becomes active approximately eight weeks after conception. For individuals genetically identified as male, the Y chromosome directs the development of testicles in the gonads and initiates the release of testosterone to a specific structure known as a tubercle. This hormone, called dihydro-testosterone, aids in transforming the tubercle into the penis.

However, individuals known as Guevedoces are anticipated to have a deficiency in this process. This deficiency appears to be a genetic condition that is prevalent in the Dominican Republic but exceptionally rare in other regions.

A boy named Johnny was raised as a girl from childhood. However, as he grew older, his gender changed, and he became Johnny. When he transitioned to male, he faced taunts at school, which he often responded to with physical confrontations. People would call him a devil and other derogatory names, frequently leading to fights.

In a conversation with BBC, Johnny said, “I never liked to dress like a girl. When my parents used to buy toys for girls, I never bothered to play with them. Despite being a girl by birth, whenever I saw boys playing, I used to stop to play ball with them.”