True hermaphroditism

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True hermaphroditism
Classification and external resources
OMIM 235600
DiseasesDB 29664
eMedicine med/1702 
MeSH D050090

True hermaphroditism is a medical term for an intersex condition in which a person is born with both ovarian and testicular tissue.

There may be an ovary on one side and a testis on the other, but more commonly one or both gonads is an ovotestis containing both types of tissue.

External genitalia are often ambiguous, the degree depending mainly on the amount of testosterone produced by the testicular tissue between 8 and 16 weeks of gestation.

This condition is very rare. There are several ways in which this may occur. It can be caused by the division of 1 ova, followed by fertilization of each haploid ova and fusion of the 2 zygotes early in development. Alternately, an ovum can be fertilized by two sperm followed by trisomic rescue in one or more daughter cell.

It is rare for both types of gonadal tissue to function. Encountered karyotypes are 47XXY, 46XX/46XY, or 46XX/47XXY.

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