Dictionary of Human Evolution and Biology

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XY Female Syndrome

Condition where an individual with a 46,XY chromosome constitution presents with a female phenotype and gonadal dysgenesis, XY type (GDXY). All XY sex-reversed females are sterile. Although sterile, XY females may lead normal lives; many marry (AKA Swyer syndrome). Behaves like an X-linked recessive. Rare (1: 20,000).

Cf. XX male syndrome.

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