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Denys-Drash syndrome

1 OMIM reference -
1 associated gene
7 signs/symptoms

PROTEIN INTERACTIONS: 1

Multiple endocrine neoplasia type 1

1 OMIM reference -
5 associated genes
13 signs/symptoms

Denys-Drash syndrome

Multiple endocrine neoplasia type 1

WT1	(UniProt - OMIM)		CDKN1A	(UniProt - OMIM)
			CDKN1B	(UniProt - OMIM)
			CDKN2B	(UniProt - OMIM)
			CDKN2C	(UniProt - OMIM)
			MEN1	(UniProt - OMIM)


INTERACTOME ASSOCIATIONS (click on a score value to see the evidence)	WT1	(0.63)	MEN1

Citations in the biomedical literature:

Denys-Drash syndrome		Multiple endocrine neoplasia type 1
	Synonym(s): - Drash syndrome - Wilms tumor and pseudohermaphroditism		Synonym(s): - MEN 1 - Wermer syndrome

	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 endocrine disease - Rare gastroenterologic disease - Rare genetic disease - Rare oncologic disease

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

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

	External references: 1 OMIM reference - 1 MeSH reference: D030321		External references: 1 OMIM reference - 1 MeSH reference: D018761

Denys-Drash syndrome		Multiple endocrine neoplasia type 1
	Very frequent - Male pseudohermaphrodism / lack of virilisation - Nephroblastoma / Wilms tumor - Nephrotic syndrome - Proteinuria - Renal disease / nephropathy Frequent - Chronic arterial hypertension Occasional - Mixed gonadal dysgenesis		Very frequent - Anomalies of the endocrine glands - Anomaly of pancreatic hormones - Autosomal dominant inheritance - Hypercalcemia - Hyperparathyroidy - Hypothalamic-hypophyseal axis anomalies / hypothalamus / pituitary anomalies - Neoplasms / tumors - Parathyroids anomalies - Structural anomalies of the pancreas Frequent - Cortico-adrenal hyperplasia / hypersecretion - Epigastralgia / heartburn / gastric / duodenal ulcer / gastritis - Thyroid anomalies - Xanthomas / lipomas