encyclopedia of medical concepts
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46, XY Disorders of Sex Development
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Definition
: Congenital conditions in individuals with a male karyotype, in which the development of the gonadal or anatomical sex is atypical.
Examples
Androgen-Insensitivity Syndrome
;
Denys-Drash Syndrome
;
Frasier Syndrome
;
Gonadal Dysgenesis, 46,XY
;
Kallmann Syndrome
;
WAGR Syndrome
Other names
Pseudohermaphroditisms, Male; Pseudohermaphroditism, Male; Male Pseudohermaphroditisms; DSDs, 46,XY; DSD, 46,XY; 46,XY DSDs; Male Pseudohermaphroditism; 46,XY Disorders of Sex Development; 46,XY DSD; 46, XY DSD
Substance
CAS Registry & name
Categories
Source
17-
Hydroxysteroid Dehydrogenase Deficiency
0
*Gynecomastia
*Steroid Metabolism, Inborn Errors
*46, XY Disorders of Sex Development
17-Hydroxysteroid Dehydrogenases/deficiency.
Lipoid congenital adrenal hyperplasia
0
*Adrenal Hyperplasia, Congenital
*46, XY Disorders of Sex Development.
Persistent Mullerian duct syndrome
0
*46, XY Disorders of Sex Development.
Urioste Martinez-
Frias syndrome
0
*Abnormalities, Multiple
*Cryptorchidism
*Polydactyly
*Craniofacial Abnormalities
*46, XY Disorders of Sex Development
Mullerian Ducts/abnormalities.
Nivelon Nivelon Mabille syndrome
0
*Osteochondrodysplasias
*46, XY Disorders of Sex Development.
Pseudovaginal perineoscrotal hypospadias
0
*Hypospadias
*Steroid Metabolism, Inborn Errors
*46, XY Disorders of Sex Development
3-Oxo-5-alpha-Steroid 4-Dehydrogenase/deficiency.
Male pseudohermaphroditism/mental retardation syndrome, Verloes type
0
*Abnormalities, Multiple
*Intellectual Disability
*46, XY Disorders of Sex Development.
Male pseudohermaphroditism due to defective LH molecule
0
*46, XY Disorders of Sex Development
Luteinizing Hormone/deficiency.
