SYNDROME DE DENYS - DRASH : A PROPOS DUN CAS

Denys-Drash syndrome (DDS) consists of a triad of pseudo-hermaphroditism, Wilms tumor and nephropathy. The condition may manifest as a complete triad or an incomplete form, with any or a combination of the above features. The characteristic glomerular abnormality of DDS is diffuse mesangial sclerosis.DDS is a spectrum of diseases with phenotypic heterogeneity. The clinical course is unpredictable despite a similar genotype.There is no single clinical guideline for the optimal management of patients with DDS, due to the impossibility of accurately predicting the course of each affected individual. Similarly, there is no consensus on the advisability of removing native kidneys in DDS patients with nephropathy that has progressed to End Stage Renal Disease. Ultimately, clinical practice is guided by the availability of resources.We report in this work a suspected case of DDS, which was lost within 24 hours of hospitalization.