Glomerulocystic kidney disease in a young adult

We report an 18-year old woman who had glomerulocystic kidney disease (GCKD) without a family history of renal disease or hypertension and no known congenital abnormalities. Her renal function was normal. Renal biopsy showed cystic dilatation of the Bowman's spaces and atrophy of the glomerular tufts. Electron microscopy revealed specific changes in the basement membranes of noncystic glomeruli, suggesting a congenital origin for her renal pathology. This relatively rare case contrasts with the usual presentation of GCKD in neonates or children.