Incident renal events and risk factors in ADPKD: A population and family based cohort followed for 22 years.

摘要

To determine the incidence of renal events in Autosomal Dominant Polycystic Kidney Disease (ADPKD) we identified all patients with ADPKD attending nephrology/urology clinics in the province in 1981, and followed members of 18 families at 50% risk of inheriting ADPKD prospectively for 22 years, including research clinics at 6 year intervals. Time to hypertension treatment, Stage 3 chronic kidney disease (CKD), endstage renal disease (ESRD) and death were measured, and the impact of genotype, gender, gender of parent transmitting PKD, family, family history of essential hypertension, parity and oral contraceptive pill were assessed.; Nine (50%) families had PKD1, 4 (22%) PKD2 and 1 family had both PKD1 and PKD2. The number of family members with PKD1 was 136 and with PKD2 60. In PKD1 median age to hypertension treatment was 46 years, to CKD Stage 3 50 years, to ESRD 53 years and to death 67 years. In PKD2 median age to hypertension treatment was 51 years, to CKD Stage 3 66 years, to death 71 years, and ESRD was infrequent. Although the incidence of CKD was later and ESRD occurred infrequently in PKD2 compared to PKD1, early onset of hypertension occurred and life expectancy was compromised. Genotype, family, and proteinuria were identified as risk factors for incident renal events. Gender, gender of parent transmitting PKD, family history of essential hypertension, multi parity, and use of the oral contraceptive pill were not identified as risk factors for renal events in ADPKD.