Guevedoces: individuals reported to apparently change gender naturally at puberty, from female to male. These people have been observed in an isolated village in the Dominican Republic and in the eastern highlands of Papua New Guinea. Infants with female appearance at birth begin to develop physical characteristics of a male during puberty. Affected individuals are born with male (XY) chromosomes. Because of a deficiency of the 5 a-reductase enzyme, testosterone is not converted to the biologically active form, dihydrotestosterone (DHT). In the absence of DHT, the infant’s external genitalia appear to be an ambiguous clitoris and labia.
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