A study of long-term morbidity associated with autosomal recessive polycystic kidney disease

Abstract

tion has not been reported. Ectatic bile ducts may leadto recurrent cholangitis.Blyth and Ockenden in 1971 [1] separated autosomal The survival of patients with ARPKD is not asrecessive polycystic kidney disease (ARPKD) from bleak as previously understood. The mortality is highother cystic diseases, describing four sub-groups (peri- (9–24%) during the first year [6–9], but the likelihoodnatal, neonatal, infantile and juvenile) on the basis of survival beyond 15 years is reported to be as highof age-at-presentation, clinical symptoms and patho- as 79% [7]. As more children with ARPKD now reachlogical findings. They speculated that diVerent genes adulthood and require renal replacement therapy, theremight be responsible for each group as aVected children is a need to report their clinical course following thein one family always had similar disease. Subsequent development of end-stage renal failure particularlyreviews, however, showed marked clinical variability given the recognized co-morbidity of hepatic dysfunc-within a single kindred which appeared to refute this tion. In Australia and New Zealand there are currentlyhypothesis [2,3]. Zerres