Renal and extrarenal signs of autosomal dominant polycystic kidney disease

Abstract: The aim of the study was to evaluate the clinical impact of renal and extrarenal manifestations of ADPKD (Autosomal Dominant Polycystic Kidney Disease). For this purpose, prospective and retrospective analyses of patients with ADPKD in different populations were performed.Records of 26 patients CAPD (Continous Ambulatory Peritoneal Dialysis) treatment in 1988-1996 were reviewed and dialysis efficacy and complications were evalated in comparison with 26 contemporary controls. The incidence of peritonitis and herniations or the properties of the peritoneal membranes were not different.The post-transplant course was recorded in 114 kidney transplant patients with ADPKD and 114 control patients matched for sex, age and donor type. Specific features of the ADPKD patients were enlarged kidneys, however relevant only before transplantation, requirement of more phlebotomies due to erythrocytosis, and diverticulitis in four patients versus none of the controls, with perforation occurring in two.Thirty patients with ADPKD who had undergone coronary angiography on clinical indication were identified. Control patients with other renal diagnoses investigated by coronary angio-graphy, were matched for age, sex and relation to transplantation. The angiograms were reviewed. The prevalence of coronary aneurysms and minor dilatations was increased and three ADPKD patients but no controls died of aortic aneurysms. The the receiving vein of arterio-venous fistulas created for hemodialysis in 19 ADPKD patients and matched control patients were measured by ultrasound. The maximum diameter of the fistulas in ADPKD patients was significantly wider.An echocardiographic investigation of 21 renal transplant patients with ADPKD was performed and compared with that of a control group of 21 transplant patients with other diagnoses, matched for sex and time after transplantation. Valvular anomalies were infrequent. Aneurysmal formation in the aorta or signs of dilated cardiomyopathy were not observed. Conclusion: The studies suggest that patients with ADPKD have a limited risk of specific complications to CAPD or transplantation related to original disease, but the wider veins of the arterio-venous fistulas and the increased number of dilatations of coronary arteries emphasize the systemic nature of the disease, particularly with respect to vessel involvement.