POLYCYSTIC KIDNEY-DISEASE - A TRULY PEDIATRIC PROBLEM

Polycystic kidney disease (PKD) represents the most common inherited c ause of chronic renal failure. PKD is a relatively uncommon cause of c hronic renal failure or mortality in childhood and adolescence, but is nevertheless often responsible for symptoms of renal disease. Current research into the pathogenesis of PKD suggests that disturbance of th e normal regulation of growth and development of tubular epithelium is intrinsic to cyst formation and growth. Features of cystic epithelium that are analogous to earlier stages of renal development include alt ered composition of the extracellular matrix, abnormal cell proliferat ion, and the persistence of a secretory pattern of fluid and electroly te transport. The potential for early diagnosis and intervention in PK D makes it an area of great interest to the pediatric nephrologist. An imal and in vitro studies have achieved modification of cyst growth by reduction of dietary protein, use of amiloride and its analogs, antag onism of the epidermal growth factor receptor system, antiinflammatory therapy, and most recently with the use of taxol, an agent that inhib its microtubule assembly. PKD may represent an area in which childhood diagnosis and intervention will have a significant impact on the prev alence of chronic renal failure in adult life.