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5 OMIM references -
4 associated genes
11 signs/symptoms
PROTEIN INTERACTIONS: 2
1 OMIM reference -
1 associated gene
22 signs/symptoms
Nephroblastoma
ADULT syndrome

DIS3L2 TP63
H19
POU6F2
WT1


INTERACTOME
ASSOCIATIONS

(click on a score value to see the evidence)
H19
WT1
(0.63)
(0.55)
TP63
TP63



Citations in the biomedical literature:


Nephroblastoma
DIS3L2 H19 POU6F2 WT1
ADULT syndrome
TP63



Nephroblastoma
ADULT syndrome

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

Synonym(s):
- Acro-dermato-ungual-lacrimal-tooth syndrome
- Pigment anomaly - ectrodactyly - hypodontia

Classification (Orphanet):
- Rare genetic disease
Classification (Orphanet):
- Rare bone disease
- Rare developmental defect during embryogenesis
- Rare eye disease
- Rare genetic disease
- Rare odontologic disease
- Rare skin disease

Classification (ICD10):
- Neoplasms -
Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -

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: C538052

Nephroblastoma
ADULT 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
- Anomalies of eyelids, eyelashes and lacrimal system
- Anomalies of teeth and dentition
- Chronic skin infection / ulcerations / ulcers / cancrum
- Dry / squaly skin / exfoliation
- Dysplastic / thick / grooved fingernails
- Dysplastic / thick / grooved toenails
- Excessive freckling
- Fine hair
- Nails anomalies
- Oligodactyly / ectrodactyly of toes
- Pigmented naevi / naevus pigmentosus / lentigo
- Syndactyly of fingers / interdigital palm
- Syndactyly of toes
- Thin skin

Frequent
- Alopecia
- Breast tissue / mammary gland absence / aplasia
- Hypoplastic / absent nipples
- Hypotrichosis / atrichia / atrichiasis / scalp hairlessness
- Tooth shape anomaly

Occasional
- Broad nose / nasal bridge
- Face / facial anomalies
- High nasal bridge