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Denys-Drash syndrome

1 OMIM reference -
1 associated gene
7 signs/symptoms

PROTEIN INTERACTIONS: 1

Bladder exstrophy

1 OMIM reference -
1 associated gene
14 signs/symptoms

Denys-Drash syndrome

Bladder exstrophy

(UniProt - OMIM)

(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		Bladder exstrophy
	Synonym(s): - Drash syndrome - Wilms tumor and pseudohermaphroditism		Synonym(s): - Classic exstrophy of the bladder

	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 abdominal surgical disease - Rare developmental defect during embryogenesis - Rare genetic disease - Rare renal disease - Rare urogenital 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: normal Type of inheritance: multigenic/multifactorial

	External references: 1 OMIM reference - 1 MeSH reference: D030321		External references: 1 OMIM reference - 1 MeSH reference: D001746

Denys-Drash syndrome		Bladder exstrophy
	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 - Anus / rectum anomalies - Bladder / cloacal exstrophy - Clitoris / labia majora / labia minora / female external genitalia hypoplasia - Hypospadias / epispadias / bent penis - Micropenis / small penis / agenesis - Umbilical hernia - Vesicorenal / vesicoureteral reflux Frequent - Inguinal / inguinoscrotal / crural hernia - Recurrent urinary infections - Urethral anomalies / stenosis / posterior urethral valves / megalocystis Occasional - Encopresis / fecal incontinence - Intestinal / gut / bowel malrotation - Omphalocele / exomphalos - Short bowel