Articles have been appearing in a number of online newspapers and journals over the past couple of days about one of the more unusual tricks that Mother Nature has up her sleeve: children who appear to be girls but who change into boys upon reaching puberty.
The popular term for this is guevedoce, a word coined in the Dominican Republic where a small village has gained some fame for the prevalence there of this condition. The word literally means "penis-twelve" (guebo is slang for penis and doce means 12 in Spanish). In science, the proper term is 5-alpha-reductase deficiency, describing the lack of an enzyme (AR5) that is necessary for normal development of male genitalia.
The condition only affects genetic male children. It's a complicated and very science-y thing but the condensed version goes like this: a mutation in one gene means the enzyme above is not produced naturally. This prevents development in the fetus of typical male genitalia (penis and testes) so the infant is born with what appears to be a vagina and visually missing the penis and testicles.
Unsurprisingly, parents assume that their new baby is a little girl and they raise her accordingly. Around the time puberty sets in, powerful hormones kick in and set off the process of male development, belatedly causing the testes to appear and descend and transform what was believed was a clitoris into a penis. The effect: the little girl rapidly becomes visibly an adolescent boy.
It is a very rare condition, found so far only in the Dominican Republic, Turkey and Papua New Guinea. However, in the tiny Dominican village of Las Salinas, it is common enough to have the previously mentioned name as well as for parents to be rather unsurprised when this dramatic change happens to what they thought was their daughter. As we all remember from science classes, genetic mutations can become concentrated in an isolated population or where marriage/reproduction is limited to a small group (for example, the hemophilia that became prevalent within Europe's intermarrying royal families).
Interestingly, parents seem to often know ahead of time what is in store for their child. Many report that "she" was always more interested in athletics and stereotypical "boyish" activities than traditional feminine roles and play; in other words, the child self-identifies as male at an early age, regardless of genital appearance.
An interesting side effect of this has been its role in the discovery of a drug used by millions of men to treat enlarged prostate glands as well as male pattern baldness. Back in the 1970s, an endocrinologist from Cornell University, Julianne Imperato, traveled to the Dominican Republic to investigate the guevedoces. Her research eventually led to the development of finasteride (Proscar) which essentially blocks the AR5 enzyme much as the genetic condition does for the pre-adolescent guevedoces.
Traditionalists of the conservative/religious variety often insist that matters of sexuality and gender identity are clear and simple and those of us who are LGBTQI are just being obstinate and complicating things that aren't complicated at all. This certainly won't convince them otherwise but it is rather striking evidence that nature itself isn't as neat and tidy about such matters as they might like to believe.
Following are a few links to some of the interesting articles on this subject:
'Guevedoces': Rare Medical Condition Hides Child's Sex Until Age 12
The Guevedoces: Gender metamorphosis at work
In this remote village, some boys don’t grow a penis until they’re 12