46,XX Sex Reversal 1

**Semantic type:** Congenital Abnormality|Disease or Syndrome

**Definition:** Presence of testes in an individual with a 46,XX karyotype associated with translocation of the SRY gene, encoding the transcription factor sex-determining region Y protein (SRY-positive).

**Synonyms:** - SRXX1 - XX Male, SRY-Positive