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46,XY повна дисгенезія гонад

ORPHA:242· ICD-10 Q99.1· 46,XY complete gonadal dysgenesis

Визначення(English summary)

A rare disorder/difference of sex development (DSD) associated with absence in gonadal development that results in the presence of female appearing external and internal genitalia in presence of a 46,XY karyotype.

Поширеність
Unknown
Успадкування
Autosomal dominant, Autosomal recessive, X-linked recessive, Y-linked
Вік початку
Adolescent, Adult