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Denys-Drash syndrome

1 OMIM reference -
1 associated gene
7 signs/symptoms

PROTEIN INTERACTIONS: 1

Split hand-split foot malformation

6 OMIM references -
5 associated genes
9 signs/symptoms

Denys-Drash syndrome

Split hand-split foot malformation

WT1	(UniProt - OMIM)		BTRC	(UniProt - OMIM)
			FBXW4	(UniProt - OMIM)
			SHFM1	(UniProt - OMIM)
			TP63	(UniProt - OMIM)
			WNT10B	(UniProt - OMIM)


INTERACTOME ASSOCIATIONS (click on a score value to see the evidence)	WT1	(0.55)	TP63

Citations in the biomedical literature:

Denys-Drash syndrome		Split hand-split foot malformation
	Synonym(s): - Drash syndrome - Wilms tumor and pseudohermaphroditism		Synonym(s): - Ectrodactyly - Lobster-claw deformity - SHFM - Split hand foot malformation

	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 bone disease - Rare developmental defect during embryogenesis - Rare genetic disease

	Classification (ICD10): - Diseases of the genitourinary system -		Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities -

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

	External references: 1 OMIM reference - 1 MeSH reference: D030321		External references: 6 OMIM references - No MeSH references

Denys-Drash syndrome		Split hand-split foot malformation
	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 - Autosomal dominant inheritance - Oligodactyly / ectrodactyly of fingers Frequent - Syndactyly of fingers / interdigital palm Occasional - Aniridia / iris hypoplasia - Autosomal recessive inheritance - Hand agenesis / absence - Sensorineural deafness / hearing loss - Trident hand / split hand / abnormal median ray - X-linked recessive inheritance