Renal Cystic Disease : Classification and Pathogenesis *

Abstract

Among the principal types of hereditary and developmental renal cystic disease (Table I), polycystic kidney disease (PKD) stands out because of its frequency and medical importance. The two principal categories of PKD are autosomal dominant (AD-PKD) and autosomal recessive (AR-PKD). Cystic kidney disease, often indistinguishable from classic PKD, is also inherent in several well-defined syndromes, among them tuberous sclerosis and von Hippel-Lindau disease.