Xj. Zhou et G. Kukes, PATHOGENESIS OF AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY-DISEASE - ROLE OF APOPTOSIS, Diagnostic molecular pathology, 7(2), 1998, pp. 65-68
Autosomal dominant polycystic kidney disease (ADPKD) is a common monog
enic 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 r
enal insufficiency in one half of affected individuals. The process ca
using the progressive loss of renal tissue is unclear. Recent studies
demonstrate that apoptosis is one of the major histopathologic feature
s of ADPKD and may be causally related to the progressive deterioratio
n of the renal function in this population. Further studies are requir
ed to elucidate the mechanisms by which some cysts upregulate the proc
ess of programmed cell death in the kidney.