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

DIS3L2 WT1
H19
POU6F2
WT1


COMMON
GENES
WT1



Citations in the biomedical literature:


Nephroblastoma
DIS3L2 H19 POU6F2 WT1
Meacham syndrome



Nephroblastoma
Meacham syndrome

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

Synonym(s):
- Meacham-Winn-Culler syndrome
- Rhabdomyomatous dysplasia - cardiopathy - genital anomalies

Classification (Orphanet):
- Rare genetic disease
Classification (Orphanet):
- Rare developmental defect during embryogenesis
- Rare endocrine disease
- Rare genetic disease
- Rare gynecologic or obstetric disease
- Rare urogenital 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:
(no data available)

External references:
5 OMIM references -
1 MeSH reference: D009396
External references:
1 OMIM reference -
No MeSH references

Nephroblastoma
Meacham 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
- Ambiguous genitalia
- Diaphragmatic hernia / defect / agenesis
- Hypoplastic lungs / pulmonary hypoplasia / agenesis
- Uterine / uterus / Fallopian tubes anomalies
- Vagina anomalies / atresia / hydrometrocolpos / hymen imperforation

Frequent
- Absent lobe / lung / segmentation defect / abnormal lobulation / sequestration
- Death in infancy
- Hypoplastic left heart / ventricle
- Micropenis / small penis / agenesis
- Stillbirth / neonatal death
- Structural and functional anomalies of the spleen
- Undescended / ectopic testes / cryptorchidia / unfixed testes

Occasional
- Anomalous pulmonary venous return
- Asplenia / polysplenia / spleen lobulation / accessory spleen
- Atrial septal defect / interauricular communication
- Dextrocardia / abnormal heart position / cardiac heterotaxia / situs inversus
- Ectopic / horseshoe / fused kidneys
- Hypoplastic aorta / coarctation / stenosis / anomaly / aortic arch interruption
- Patent ductus arteriosus
- Tetralogy of Fallot / trilogy of Fallot
- Transposition of great vessels
- Ventricular septal defect / interventricular communication