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1 OMIM reference -
1 associated gene
7 signs/symptoms
COMMON GENES: 1
COMMON SIGNS: 1
5 OMIM references -
4 associated genes
11 signs/symptoms
Denys-Drash syndrome
Nephroblastoma

WT1 DIS3L2
H19
POU6F2
WT1


COMMON
GENES
WT1



Citations in the biomedical literature:


Denys-Drash syndrome
WT1
Nephroblastoma
DIS3L2 H19 POU6F2



Denys-Drash syndrome
Nephroblastoma

Synonym(s):
- Drash syndrome
- Wilms tumor and pseudohermaphroditism

Synonym(s):
- Renal embryonic tumor
- Wilms tumor

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 genetic disease

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

Epidemiological data:
(no data available)
Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: childhood
Average age of death: any age
Type of inheritance: autosomal dominant

External references:
1 OMIM reference -
1 MeSH reference: D030321
External references:
5 OMIM references -
1 MeSH reference: D009396


COMMON
SIGNS
- Chronic arterial hypertension


Denys-Drash syndrome
Nephroblastoma

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

Occasional
- Mixed gonadal dysgenesis


Very frequent
- Acute abdominal pain / colic
- Kidney / renal neoplasm / tumor / carcinoma / cancer
- Neoplasms / tumors

Occasional
- Aniridia / iris hypoplasia
- Fever / chilling
- Hematuria / microhematuria
- Hepatic / liver neoplasm / tumor / carcinoma / cancer
- Lung / bronchopulmonary neoplasm / tumor / carcinoma / cancer
- Lymphadenopathy / polyadenopathies
- Weight loss / loss of appetite / break in weight curve / general health alteration