46,XY complete gonadal dysgenesis

Orpha code: 242OMIM code: 616425

Definition

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.

Disease data
Classification

Malformation syndrome

Synonyms
46,XY CGD
46,XY CGD
46,XY czysta dysgenezja gonad
Zespół Swyera
46,XY pure gonadal dysgenesis
Swyer syndrome
ORPHA code
242
OMIM code
616425
ICD10 code
Q99.1
ICD11 code
LD2A.1

No additional description.

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