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Denys-Drash syndrome
1 OMIM reference -
1 associated gene
7 signs/symptoms
PROTEIN INTERACTIONS: 1
B-cell chronic lymphocytic leukemia
6 OMIM references -
7 associated genes
No signs/symptoms info
Denys-Drash syndrome
B-cell chronic lymphocytic leukemia

WT1
(UniProt - OMIM)
 ARL11
(UniProt - OMIM)
ATM
(UniProt - OMIM)
CCND1
(UniProt - OMIM)
IGHG1
(UniProt - OMIM)
IGHV3-21
()
POT1
(UniProt - OMIM)
TP53
(UniProt - OMIM)

INTERACTOME
ASSOCIATIONS
(click on a score value to see the evidence)
WT1
(0.86)
TP53


Citations in the biomedical literature:


Denys-Drash syndrome
WT1
B-cell chronic lymphocytic leukemia
ARL11 ATM CCND1 IGHG1 IGHV3-21 POT1
TP53



Denys-Drash syndrome
B-cell chronic lymphocytic leukemia

Synonym(s):
- Drash syndrome
- Wilms tumor and pseudohermaphroditism
Synonym(s):
- B-CLL
- B-cell chronic lymphoid leukemia
- Lymphoplasmacytic leukemia
- Lymphoplasmacytoid immunocytoma
- Small lymphocytic lymphoma
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 hematologic disease
- Rare oncologic disease
Classification (ICD10):
- Diseases of the genitourinary system -
Classification (ICD10):
- Neoplasms -
Epidemiological data:
(no data available)
Epidemiological data:
Class of prevalence: 1-5 / 10 000
Average age onset: adulthood
Average age of death: -
Type of inheritance: sporadic
External references:
1 OMIM reference -
1 MeSH reference: D030321
External references:
6 OMIM references -
1 MeSH reference: D015451
Denys-Drash syndrome

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

Frequent
- Chronic arterial hypertension

Occasional
- Mixed gonadal dysgenesis


B-cell chronic lymphocytic leukemia

(no data available)