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Denys-Drash syndrome

1 OMIM reference -
1 associated gene
7 signs/symptoms

COMMON GENES: 1
COMMON SIGNS: 3

Frasier syndrome

1 OMIM reference -
1 associated gene
7 signs/symptoms

Denys-Drash syndrome

Frasier syndrome

WT1

(UniProt - OMIM)

WT1

(UniProt - OMIM)


COMMON GENES	WT1

Citations in the biomedical literature:

Denys-Drash syndrome		Frasier syndrome
	Synonym(s): - Drash syndrome - Wilms tumor and pseudohermaphroditism		Synonym(s): (no synonyms)

	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 oncologic disease - Rare renal disease - Rare urogenital disease

	Classification (ICD10): - Diseases of the genitourinary system -		Classification (ICD10): - Diseases of the genitourinary system -

	Epidemiological data: (no data available)		Epidemiological data: Class of prevalence: <1 / 1 000 000 Average age onset: childhood Average age of death: - Type of inheritance: autosomal dominant

	External references: 1 OMIM reference - 1 MeSH reference: D030321		External references: 1 OMIM reference - 1 MeSH reference: D052159


COMMON SIGNS	- Male pseudohermaphrodism / lack of virilisation - Nephrotic syndrome - Proteinuria

Denys-Drash syndrome		Frasier syndrome
	Very frequent - Nephroblastoma / Wilms tumor - Renal disease / nephropathy Frequent - Chronic arterial hypertension Occasional - Mixed gonadal dysgenesis		Very frequent - Late puberty / hypogonadism / hypogenitalism - Primary amenorrhea Frequent - Renal failure - Renal glomerular defect / glomerulopathy