Nephropathy associated with wilms’ tumor (drash syndrome).

ABSTRACT

Nephropathy associated with Wilms’ tumor (Drash syndrome) is an uncommon disease with well-documented clinical course and prognosis regarding kidney function. We report a girl with Drash syndrome who presented with Wilms’ tumor and aniridia at the age of one year. Left nephrectomy with chemotherapy and radiotherapy were given. She remained in good health with normal renal function during the 7 years period of follow up. At the age of 9 years, she developed acute nephrotic syndrome, hypertension, and renal failure (GFR 15ml./min/1.73 square meter). Abdominal ultrasound revealed no evidence of Wilms’ tumor recurrence. Prednisolone, cyclophosphamide, and antihypertensive drugs were given. Renal function returned to normal (GFR 80/ml/min/1.73 square meter) at one month and serum albumin at 3 months after treatment. The case of nephropathy associated with Wilms’ tumor presented here has an atypical clinical manifestation as compared with the literature concerning it may throw some light on the pathogenesis of glomerulopathy in Drash syndrome.