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

DIS3L2 WT1
H19
POU6F2
WT1


COMMON
GENES
WT1



Citations in the biomedical literature:


Nephroblastoma
DIS3L2 H19 POU6F2 WT1
Frasier syndrome



Nephroblastoma
Frasier syndrome

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

Synonym(s):
(no synonyms)

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

Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: childhood
Average age of death: any age
Type of inheritance: autosomal dominant
Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: childhood
Average age of death: -
Type of inheritance: autosomal dominant

External references:
5 OMIM references -
1 MeSH reference: D009396
External references:
1 OMIM reference -
1 MeSH reference: D052159

Nephroblastoma
Frasier syndrome

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

Occasional
- Aniridia / iris hypoplasia
- Chronic arterial hypertension
- 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


Very frequent
- Late puberty / hypogonadism / hypogenitalism
- Male pseudohermaphrodism / lack of virilisation
- Primary amenorrhea

Frequent
- Nephrotic syndrome
- Proteinuria
- Renal failure
- Renal glomerular defect / glomerulopathy