Imagine being raised as a girl and then, when puberty hits, developing a penis.

Puberty is a difficult time for anyone, but for those who go through this, it can be more difficult than we can imagine.

A BBC2 documentary series, Countdown to Life: The Extraordinary Making of You, has examined children in a small village in the Dominican Republic with this condition. The children there are called “guevedoce,” which translates to “penis at twelve.”

The science of our bodies

The way we develop is a complicated process.

You may remember that the typical human being has 46 chromosomes that come in 23 pairs. One pair is the sex chromosomes: Y for male, X for female.

Men carry both the X and Y chromosomes; women, just the X. If two X chromosomes pair, the fetus develops into a female. If you get an XY pairing, a male develops.

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X and Y chromosomes

Development of external reproductive organs is controlled by something called dihydrotestosterone (DHT).

An enzyme called 5-alpha reductase converts testosterone into DHT. Both males and females start off with the same bits, but if DHT is missing, then genitalia develops into the female parts, with the genital folds remaining unfused. If DHT is present, then the folds enlarge and wrap around the penile urethra, becoming the penis.

‘Penis at 12’

In 1974, Doctor Julianne Imperato, an endocrinologist from Cornell University went to study the children of the Dominican Republic who had been rumoured to have been born with female genitalia but who had developed male genitalia at puberty.

What she discovered was that these children were lacking DHT at birth. But when they reached puberty, they got a surge of the enzyme, which then increased testosterone levels and caused the development of penises. This condition was called 5-alpha reductase deficiency (5-ARD).

That’s not to say that those with 5-ARD have nothing that resembles a male. The children have undescended testicles, and a small penis that looks more like a clitoris as well as an underdeveloped prostate. However, in most cases, these children are raised as girls.

The condition isn’t just relegated to the Dominican Republic. There have been cases around the world including New Guinea, Turkey and the United States.

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