In 27 (12%) patients, one or several extra-renal abnormalities were reported; these included symptoms suggestive of Denys-Drash syndrome (caused by WT1 gene mutation and characterised by nephropathy, Wilms tumour, and genital abnormalities) and Pierson syndrome (caused by LAMB2 gene mutation and characterised by the occurrence of congenital nephrotic syndrome and ocular anomalies in combination with microcoria). The gene discussed is WT1; the disease is Denys-Drash syndrome.