Denys drash syndrome

a syndrome that consists of ambiguous genitalia, Wilms tumour and progressive nephropathy with renal failure. The syndrome appears to be generally sporadic, although familial cases have been described. The syndrome has been linked to mutations in the gene coding for the tumour suppressor gene, WT1. Renal involvement manifests as a progressive nephropathy, usually in the first year of life. Renal biopsy typically shows diffuse or sometimes focal mesangial sclerosis. The imaging findings are those of a glomerulonephritis (see glomerulonephritis). The other major renal manifestation is Wilms' tumour, which usually presents within the first two years of life. The imaging findings of Wilms' tumour in the Denys Drash syndrome are the same as Wilms' tumour in general (see Wilms tumour).

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