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Denys-Drash syndrome
1 OMIM reference -
1 associated gene
7 signs/symptoms
COMMON GENES: 1
Desmoplastic small round cell tumor
2 associated genes
19 signs/symptoms
Denys-Drash syndrome
Desmoplastic small round cell tumor

WT1
(UniProt - OMIM)
 EWSR1
(UniProt - OMIM)
WT1
(UniProt - OMIM)

COMMON
GENES 		WT1


Citations in the biomedical literature:


Denys-Drash syndrome
WT1
Desmoplastic small round cell tumor
EWSR1



Denys-Drash syndrome
Desmoplastic small round cell tumor

Synonym(s):
- Drash syndrome
- Wilms tumor and pseudohermaphroditism
Synonym(s):
- DSRCT
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 abdominal surgical disease
- Rare oncologic disease
Classification (ICD10):
- Diseases of the genitourinary system -
Classification (ICD10):
- Neoplasms -
Epidemiological data:
(no data available)
Epidemiological data:
Class of prevalence: unknown
Average age onset: adolescence / young
Average age of death: -
Type of inheritance: sporadic
External references:
1 OMIM reference -
1 MeSH reference: D030321
External references:
No OMIM references
1 MeSH reference: D058405
Denys-Drash syndrome
Desmoplastic small round cell tumor

Very frequent
- Male pseudohermaphrodism / lack of virilisation
- Nephroblastoma / Wilms tumor
- Nephrotic syndrome
- Proteinuria
- Renal disease / nephropathy

Frequent
- Chronic arterial hypertension

Occasional
- Mixed gonadal dysgenesis


Very frequent
- Acute abdominal pain / colic
- Digestive neoplasm / tumor / carcinoma / cancer
- Lymphadenopathy / polyadenopathies
- Peritoneal diseases
- Soft tissue sarcoma / cancer / tumor / liposarcoma / myosarcoma

Frequent
- Hepatomegaly / liver enlargement (excluding storage disease)
- Intestinal obstruction / ileus
- Mediastinal / hilar adenopathies
- Nausea / vomiting / regurgitation / merycism / hyperemesis
- Weight loss / loss of appetite / break in weight curve / general health alteration

Occasional
- Anaemia
- Ascitis
- Central nervous system / peripheral nerves neoplasm / tumor / carcinoma / cancer
- Lung / bronchopulmonary neoplasm / tumor / carcinoma / cancer
- Nose / nasal sinus neoplasm / tumor / carcinoma / cancer
- Ovary / Fallopian tube neoplasm / tumor / carcinoma / cancer (excl. teratoma / germinoma)
- Pancreatic / pancreas neoplasm / tumor / carcinoma / cancer
- Testis anomalies
- Wasted (excluding lipodystrophy) / poorly muscled build / cachexy