Glomerulocystic kidney: proposed etiology and pathogenesis

Abstract

Glomerulocystic kidney, an unusual lesion seen only in infancy or early childhood, involves cystic dilatation of Bowman's space without evidence of urinary obstruction. This disease may result from intrarenal medullary obstruction in the third trimester of pregnancy because 1) it is similar to the cystic kidney lesion produced by urethral obstruction, 2) patients with glomerulocystic kidney have no evidence of extrarenal urinary tract obstruction and 3) most patients with glomerulocystic kidney have evidence of inflammation and fibrosis in the renal medulla. A prenatal drug history of prolonged maternal ingestion of phenacetin in an infant with glomerulocystic kidney prompted us to submit a possible etiologic source for the hypothesis of intrarenal obstruction.