ODCs

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1 OMIM reference -
2 associated genes
14 signs/symptoms

COMMON GENES: 2
COMMON SIGNS: 2

3 OMIM references -
3 associated genes
5 signs/symptoms

46,XX ovotesticular disorder of sex development

46,XX testicular disorder of sex development

Citations in the biomedical literature:

46,XX ovotesticular disorder of sex development		46,XX testicular disorder of sex development
	Synonym(s): - 46,XX ovotesticular DSD - True hermaphroditism		Synonym(s): - 46,XX testicular DSD - De la Chapelle syndrome - XX, male syndrome

	Classification (Orphanet): - Rare developmental defect during embryogenesis - Rare endocrine disease - Rare genetic disease - Rare gynecologic or obstetric disease - Rare infertility - Rare urogenital disease		Classification (Orphanet): - Rare developmental defect during embryogenesis - Rare endocrine disease - Rare genetic disease - Rare infertility - Rare urogenital disease

	Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities -		Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities -

	Epidemiological data: Class of prevalence: 1-9 / 100 000 Average age onset: childhood Average age of death: normal Type of inheritance: autosomal recessive		Epidemiological data: (no data available)

	External references: 1 OMIM reference - 1 MeSH reference: D050090		External references: 3 OMIM references - 1 MeSH reference: D058531


COMMON SIGNS	- Abnormal / polycystic ovaries - Ambiguous genitalia

46,XX ovotesticular disorder of sex development		46,XX testicular disorder of sex development
	Very frequent - Bifid scrotum - Horizontal folds on scrotum - Hypoplastic scrotum / hemiscrotum / scrotal ridges - Hypospadias / epispadias / bent penis - Male internal genitalia anomaly / deferent ducts agenesis / ectopy / duplication - Micropenis / small penis / agenesis - Sterility / hypofertility - True hermaphrodism - Undescended / ectopic testes / cryptorchidia / unfixed testes - Urogenital sinus - Uterine / uterus / Fallopian tubes anomalies Occasional - Chromosomal or genetic anomaly		Very frequent - Late puberty / hypogonadism / hypogenitalism - Small / atrophic / hypoplastic testes / monorchism / microorchidism / anorchia - X-linked recessive inheritance