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Pseudohermaphroditism or pseudo-hermaphroditism, is a name used to describe people born with secondary sex characteristics[1] or a phenotype[2] which is different from what would be expected based upon the gonadal tissue (ovary or testis).
In some cases, the external sex organs look intermediate between the typical clitoris or penis. In other cases, the external sex organs have an appearance that does not look intermediate, but rather has the appearance that would be expected to be seen with the “opposite” gonadal tissue. Because of this, pseudohermaphroditism is sometimes not identified until puberty.
The term “male pseudohermaphrodite” is used when a testis is present, and the term “female pseudohermaphrodite” is used when an ovary is present.[3] The term “true” hermaphrodite is reserved for the very rare cases where both ovarian and testicular tissue is present. (Whether or not that term would be appropriate when ovotestes are found, or only when distinct ovaries and testes are found, is not well defined.)
It is possible for the condition to be undetected until adulthood.[4]
Associated conditions in males include 5-a-reductase deficiency[5] from a deficiency in the male chromosome (46 XY).[6][7]
Surgery has sometimes been performed to alter the appearance of the genitals.[8][9] Modern approaches often involve delaying surgery when possible until the child matures enough to express a preference.
Use of the term “pseudohermaphroditism” can be problematic. The term “pseudohermaphroditism” was created by Edwin Klebs in 1876, [10][11] long before the genetic roles of the X chromosome and Y chromosome and the social components of gender identity were well characterized, which is why the term is usually used to describe the dissonance between gonadal histology and external genital appearance.
The term “intersexuality” was introduced by Richard Goldschmidt in 1923. [10][12] However, the term “intersex” has also been challenged; the Lawson Wilkins Pediatric Endocrine Society and the European Society for Pediatric Endocrinology have adopted a nomenclature system based on disorders of sex development which covers “congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical” and thus replaces many disparate terms, including but not limited to those based on “hermaphrodite.”[13][14]
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