Outcome of renal replacement therapy in autosomal dominant polycystic kidney disease.

Abstract

We review our own experience as well as pertinent literature on the outcome of renal replacement therapy (RRT) in autosomal dominant polycystic kidney disease (ADPKD). Due to the virtual absence of data on peritoneal dialysis in ADPKD, we deal only with haemodialysis (HD) and renal transplantation (TP). Special attention is paid to the renal and extrarenal complications of ADPKD. On HD, 5 year survival is 10-15% greater in ADPKD than in non-ADPKD patients, probably because of a lower cardiac mortality of ADPKD patients. After TP, patient as well as graft survival rates of ADPKD patients are similar to those of non-ADPKD patients. On HD, the prevalence of renal pain, gross haematuria and renal infection is significantly greater in ADPKD (36, 36 and 16% respectively) than in non-ADPKD patients (2, 16 and 2% respectively), but these complications are rarely severe. Other than preparation for TP, nephrectomy is required in only 4% of ADPKD patients on HD. With a policy of selective removal of problematic kidneys before TP, complications due to native polycystic kidneys do not frequently occur after TP, leading to post-TP nephrectomy in only 7% of ADPKD patients. There is a mild excess of stroke among ADPKD patients undergoing RRT, the contribution of intracranial aneurysm rupture not being clearly defined. Symptoms related to hepatic cysts are rare and to cardiac valvular abnormalities very rare. In conclusion, RRT is at least as successful in ADPKD as in non-ADPKD patients. Renal complications are frequent but rarely severe. Extrarenal complications are not frequent.