Conditions / Syndrome
Denys-Drash syndrome
info · Syndrome
A syndrome that is characterized by the association of diffuse mesangial sclerosis (DMS), male pseudohermaphroditism with a 46,XY karyotype, and nephroblastoma that derives_from an abnormality in the WT1 gene (Wilms' tumor suppressor gene).
Signs and symptoms
- Epicanthus
- Wide anterior fontanel
- Septate vagina
- Male pseudohermaphroditism
- Uterus didelphys
- Focal segmental glomerulosclerosis
- Posterolateral diaphragmatic hernia
- Enlarged kidney
- Neonatal respiratory distress
- Stage 5 chronic kidney disease