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Individuals with a deficiency of the aforementioned enzyme present with ambiguous genitalia at birth, making it difficult to determine their true sex, until the secondary sexual characteristics develop at puberty.

These individuals possess the genotype of a male (XY) but dress and act as a female would, as they were brought up that way, and, therefore, have a different perception of themselves than what their actual genotypes dictate.

Articles that discussed pseudo-hermaphroditism were marked, and then reviewed by two reviewers for relevance and content.As a result of this, individuals present with ambiguous external genitalia, and do not have a defined idea of what their true identity is.One such example is present in Las Salinas, in the south of the Dominican Republic, where there is a growing minority of pseudo-hermaphrodites (1 in every 50 births), known as "guevedoces" which literally translates into "penis at 12".Due to the lack of relevant information in the online databases, much effort went into finding information from non-catalogued sources in the libraries of Dr. Padre Billini Teaching Hospital, in Santo Domingo, Dominican Republic, where we have affiliations.Only pertinent data was extracted and included in the review.




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