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46,XY difference of sex development due to 5-alpha-reductase 2 deficiency

ORPHA:753DiseaseAutosomal recessiveAdolescent, Childhood, Infancy, Neonatal

Ассоциированные гены (1)

SRD5A2
steroid 5 alpha-reductase 2
Disease-causing germline mutation(s) (loss of function) in
OMIM: 607306

Фенотипы (10)

Очень частый (80–99%)10
HP:0000028Cryptorchidism
HP:0000033Ambiguous genitalia, male
HP:0000046Small scrotum
HP:0000048Bifid scrotum
HP:0000051Perineal hypospadias
HP:0000062Ambiguous genitalia
HP:0000144Decreased fertility
HP:0000818Abnormality of the endocrine system
HP:0008736Hypoplasia of penis
HP:0100779Urogenital sinus anomaly

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы