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Denys-Drash syndrome

1 OMIM reference -
1 associated gene
7 signs/symptoms

COMMON GENES: 1
COMMON SIGNS: 1

46,XY partial gonadal dysgenesis

5 OMIM references -
7 associated genes
9 signs/symptoms

Denys-Drash syndrome

46,XY partial gonadal dysgenesis

WT1	(UniProt - OMIM)		GATA4	(UniProt - OMIM)
			MAP3K1	(UniProt - OMIM)
			NR0B1	(UniProt - OMIM)
			NR5A1	(UniProt - OMIM)
			SRY	(UniProt - OMIM)
			WT1	(UniProt - OMIM)
			WWOX	(UniProt - OMIM)


COMMON GENES	WT1

Citations in the biomedical literature:

Denys-Drash syndrome		46,XY partial gonadal dysgenesis
	Synonym(s): - Drash syndrome - Wilms tumor and pseudohermaphroditism		Synonym(s): - 46,XY PGD - 46,XY partial testicular dysgenesis

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

	Classification (ICD10): - Diseases of the genitourinary system -		Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities -

	Epidemiological data: (no data available)		Epidemiological data: Class of prevalence: unknown Average age onset: neonatal/infancy Average age of death: any age Type of inheritance: sporadic

	External references: 1 OMIM reference - 1 MeSH reference: D030321		External references: 5 OMIM references - No MeSH references


COMMON SIGNS	- Mixed gonadal dysgenesis

Denys-Drash syndrome		46,XY partial gonadal dysgenesis
	Very frequent - Male pseudohermaphrodism / lack of virilisation - Nephroblastoma / Wilms tumor - Nephrotic syndrome - Proteinuria - Renal disease / nephropathy Frequent - Chronic arterial hypertension		Very frequent - Ambiguous genitalia - Female pseudohermaphrodism / virilisation / clitoridomegaly - Hypospadias / epispadias / bent penis - Micropenis / small penis / agenesis - Testis anomalies Occasional - Cortico-adrenal hypoplasia / insufficiency - Ovary / Fallopian tube neoplasm / tumor / carcinoma / cancer (excl. teratoma / germinoma) - Testicular / seminal neoplasm / tumor / carcinoma / cancer (excl. teratoma / germinoma)