Denys-Drash syndrome

The Denys-Drash syndrome is an autosomal recessive congenital disorder characterized by the triad of progressive renal disease, male pseudohermaphroditism, and Wilms tumor. The disorder is caused by heterozygous germline missense mutations in the zinc-finger motife (mainly exon 8 or 9) of the WT1 gene.

Systematic

Wilms tumor
	Aniridia-Wilms-tumor syndrome
	Denys-Drash syndrome
		WT1
	Somatic nephroblastoma
	WAGR syndrome