In humans sex is determined by the X and Y chromosomes: Female XX and male XY. In a remote community in the Dominican Republic a it was discovered that some children were born looking like girls, with no testes and what appears to be a vagina. Once these children reach puberty then the penis grows and testicles descend. These children have been called "Guevedoces" which literally means "penis at 12".
It was discovered that Guevedoces children have a deficiency of the 5-alpha-reductase which is an enzyme that converts testosterone to dihydrotestosterone (DHT), a more potent androgen. This deficiency is a genetic condition resulting from a mutation in the 5-alpha-reductase 2 gene located on chromosome 2. DHT and testosterone are essential for the normal development of the male sex characteristics. DHT directs the development of the external male genitalia before birth, thus its absence results in an XY child being born with ambiguous genetalia and often mistaken for a girl. However, at puberty the individual will receive a second dose of testosterone which will promote the growth of the penis.
Read the stories of some of these children
Extraordinary case of the Guevedoces
Guevedoces: Rare medical condition
Curious case of the children whose penises don't appear until puberty
Sex really is a spectrum, and there are literally hundreds of different ways to be intersex. And as this is a fact of nature, all societies have found ways for their intersex members to be recognized and incorporated. Guevedoce means 'a penis at twelve’ and refers to male children who are born with no visible male organ and what looks like a clitoris until they attain the age of puberty when they develop male organs,and recognize that in later days and is also called ‘machihembras’ which means ‘first a woman, then a man’.The male ‘equipment’ seems to work fine and most of them live out the rest of their lives as men, though some remain females and also it is discovered that the condition end up having small prostrates.
ReplyDeleteand this led to the development of the best-selling drug, Finasteride, which ‘blocks the action of the 5-alpha-reductase, mimicking the lack of dihydro-testosterone seen in this and the drug is an effective way to cure benign prostrate enlargement, a problem of many men as they grow older.As they say, ‘One man’s loss always is another man’s gain’. although these boys were brought up as girls, almost all Guevedoce showed strong heterosexual preferences. Gemma Nierman, PhD, says ”the Dominican Republic republic communities that quietly accept their citizens with the 5-alpha-reductase deficiency are a model to the world on how to deal with genetic differences are showing us how to help these folks have a full and rewarding life despite their genetic disorder.”
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ReplyDeleteSome children with a rare genetic condition appear female at birth but later develop a penis and testes around the time puberty begins. Children with the condition are genetically male, meaning they have one X and one Y chromosome. However, as they develop in the womb, a genetic mutation prevents their bodies from converting the male hormone testosterone into a hormone called dihydrotestosterone, or DHT. Because DHT is responsible for the development of male sex organs, the lack of DHT means the male organs don't develop properly, according to the National Institutes of Health. When children with the condition are born, their external genitals look female, or in some cases, appear ambiguous, the NIH says. But during puberty, Guevedoces, like typical males, experience an increase in testosterone. The body appears to respond to these higher levels of testosterone — the penis and scrotum grow larger, and secondary sex characteristics — such as increased muscle mass and a deep voice — can also appear. Men with this condition, officially called 5-alpha reductase deficiency, are usually infertile, and often do not develop much facial or body hair. Although most children with this condition are raised as girls, about half identify themselves as male after puberty, the NIH says. [7 Diseases You Can Learn About From a Genetic Test]
ReplyDeleteWhen babies are conceived normally have a pair of X chromosomes if you are to become a girl and a set of XY chromosomes if you are destined to be male. If you're female and you don't make dihydro-testosterone then your tubercle becomes a clitoris. This deficiency seems to be a genetic condition, quite common in this part of the Dominican Republic, but vanishingly rare elsewhere. So the boys, despite having an XY chromosome, appear female when they are born. At puberty, like other boys, they get a second surge of testosterone. This time the body does respond and they sprout muscles, testes and a penis. When Imperato-McGinley investigated the Guevedoces she discovered the reason they don't have male genitalia when they are born is because they are deficient in an enzyme called 5-alpha-reductase, which normally converts testosterone into dihydro-testosterone. He described that these boys, despite being brought up as girls, almost all showed strong heterosexual preferences.
ReplyDeleteGenetics may also play a part in the hormones not being as effective or the baby being more sensitive to hormonal imbalances that might cause Gender Issues and the child to become Transgender. So two of the three factors are in some way hormonally based and prenatal hormones can be affected by stress, medications, pollutants, disease and numerous other things. Certainly some people may come to the realization later in life but even then many of them had other gender nonconforming behaviors before that such as occasional cross dressing. The ages for transition can be at any age but the ages for when they knew they felt more like a girl (or boy) tends to average much lower. In many people like myself, as low as early childhood and as young as 6.
Men with this condition, officially called 5-alpha reductase deficiency, are usually infertile, and often do not develop much facial or body hair,In addition to the Dominican Republic, cases of 5-alpha reductase deficiency have been found in Papua New Guinea, Turkey and Egypt, according to the NIH.
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ReplyDeleteGuevedoces is a gentic disorder that both sexes appear at maturity time. in case of girls they grow normally until before they puberty when reaching this stage they grow with male reproductive organs along with there. because of 5-alpha-reductase enzyme that converts testosterone to Dihydro dehydrogenase. Due to this condition they appear like Guevedoces. This was discovered under Dominican republic. Guevedoces were infertile.
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ReplyDeleteThe deficiency of DHT and testosterone is a genetic condition resulting from a mutation in the 5-alpha-reductase 2 gene located on chromosome 2. DHT and testosterone are essential for the normal development of the male sex characteristics. Due to the absence of DHT male external genitalia is not formed during the birth. But later at the puberty if the receive a dosage of DHT then they develop the growth of male external genitalia. This condition is called Guevedoces which is also called as penis at 12. Which is commonly seen in a remote community of Dominican Republic.
GUEVEDOCES is a rare condition found in Dominican republic which literally means “penis at 12” arises due to mutation in the 5-alpha-reductase 2 gene located on chromosome 2 results in the deficiency of 5-alpha-reductase which is an enzyme that converts testosterone to dihydrotestosterone (DHT), a more potent androgen. DHT directs the development of the external male genitalia before birth, thus its absence results in an XY child being born with ambiguous genetalia and often mistaken for a girl. However, at puberty the individual will receive a second dose of testosterone which will promote the growth of the penis eventually the gender changes from girl to boy.
ReplyDeleteGuevedoces is a genetic disorder of the enzyme 5-alpha-reductase. It is due to a mutation in the 5-alpha-reductase 2 gene located on chromosome 2. The enzyme is responsible for the conversion of testosterone to dihydrotestosterone (DHT), which are necessary for male sex characteristics. The DHT is needed for the growth of the genetalia, which is not observed in children without the enzyme. However, genetalia will grow at the age of 12 in boys, which is around the starting age for puberty.
ReplyDeleteIn an isolated village in the Dominican Republic, an estimated one in fifty children are born appearing to be girls but grow male genitalia during puberty. This is known as Guevedoces, which literally means "penis at 12".
ReplyDeleteIt was discovered that Guevedoces children have a deficiency of the 5-alpha-reductase which is an enzyme that converts testosterone to dihydrotestosterone (DHT), a more potent androgen. This was also common in the Sambian villages of Papua New Guinea, where the locals called the children “turnims”, meaning “expected to become men”.
The Sambians view these children as flawed males; the children are rejected and humiliated by their families and society. On the other hand, in the Dominican Republic, the birth of a pseudohermaphrodite is fully accepted and during puberty, the child’s physical transformation into a male is marked by joyous celebration.
Also the observation that Guevedoces tended to have small prostates led to the development of a drug that has helped millions of men, according to the BBC.
Guevedoces are also sometimes called “machihembras” meaning “first a woman, then a man”. When they’re born they look like girls with no testes and what appears to be a vagina. It is only when they near puberty that the penis grows and testicles descend.Children with the condition are genetically male, meaning they have one X and one Y chromosome. However, as they develop in the womb, a genetic mutation prevents their bodies from converting the male hormone testosterone into a hormone called dihydrotestosterone, or DHT. Because DHT is responsible for the development of male sex organs, the lack of DHT means the male organs don't develop properly.Men with this condition, officially called 5-alpha reductase deficiency, are usually infertile, and often do not develop much facial or body hair. Although most children with this condition are raised as girls, about half identify themselves as male after puberty.
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