Denys-Drash Syndrome

**Semantic type:** Disease or Syndrome

**Definition:** A rare congenital syndrome caused by mutations in the WT1 gene. It is characterized by the presence of congenital nephropathy (diffuse mesangial sclerosis), Wilms tumor, and intersex disorders.

**Synonyms:** - DDS - DDS - Denys Drash Syndrome - Denys-Drash syndrome - Nephrotic Syndrome Type 4