syndrome de Denys-Drash

CONT: Le syndrome de Denys-Drash est une maladie rare caractérisée par l'association d'une néphropathie glomérulaire [...], d'anomalies génitales [...] et d'une prédisposition aux tumeurs de Wilms [...]

CONT: Denys–Drash syndrome (DDS) is a rare disorder consisting of the triad of congenital nephropathy, Wilms tumor, and intersex disorders resulting from mutations in the Wilms tumor suppressor (WT1) gene. Nephropathy is a constant feature; in the incomplete forms of the syndrome, the nephropathy is present with either Wilms tumor or intersex disorders, but the vast majority of patients with Denys–Drash syndrome are destined to develop Wilms tumor in any residual renal tissue.