Pathogenesis of autosomal dominant polycystic kidney disease: role of apoptosis.

Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic disorder with progressive distension of multiple tubular segments, and is manifested by fluid accumulation, growth of epithelial cells, and remodeling of the extracellular matrix, ultimately resulting in renal insufficiency in one half of affected individuals. The process causing the progressive loss of renal tissue is unclear. Recent studies demonstrate that apoptosis is one of the major histopathologic features of ADPKD and may be causally related to the progressive deterioration of the renal function in this population. Further studies are required to elucidate the mechanisms by which some cysts upregulate the process of programmed cell death in the kidney.