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Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is the fourth leading cause of progressive kidney disease leading to end-stage kidney disease (ESKD) in Japan.Total kidney volume (TKV) serves as a surrogate marker for disease severity until patients reach ESKD.While numerous clinical studies have focused on the pre-ESKD phase, research on clinical outcomes and kidney volume changes after ESKD remains limited.Methods: A total of 69 ADPKD patients with ESKD (40 males, 29 females) were enrolled to assess long-term clinical outcomes, including survival after ESKD onset.We also performed repeated computed tomography (CT) and magnetic resonance imaging (MRI) to measure and compare TKV and the rate of TKV growth.In a subset of patients, evaluations were conducted stratified by the specific PKD1/2 pathogenic variants. Results:The median age at which patients reached to ESKD was 52.1 years (range, 21-77 years).When stratified by PKD1/2 pathogenic variants, the median age at ESKD followed the order of PKD1-truncated, PKD1 non-truncated, and PKD2: 49.5 years, 59.0 years, and 65.5 years, respectively (p < 0.01 for PKD1-tr vs PKD2).Renal replacement therapy (RRT) chosen after ESKD onset consisted of hemodialysis (HD) in 56 patients (81%), peritoneal dialysis (PD) in 10 patients (14%), and living-donor kidney transplantation in 3 patients (4%).The long-term survival rates after ESKD onset were favorable, with 5-year and 10year survival rates of 95% and 93%, respectively.The rate of TKV change significantly decreased from +10.3%/year in the pre-ESKD phase to -0.5%/year after ESKD (p<0.01).However, the PD group demonstrated a significantly higher rate of TKV change compared to the HD group (+14.6%/year vs. -1.3%/year,p<0.01). Conclusion:The long-term prognosis for ADPKD patients after reaching ESKD is relatively favorable.While the change in TKV slows down after dialysis initiation compared to the pre-ESKD phase, there is a risk for TKV to increase in peritoneal dialysis (PD) patients, highlighting the importance of careful, individualized case observation.I have no potential conflict of interest to disclose.I did not use generative AI and AI-assisted technologies in the writing process.
Published in: Kidney International Reports
Volume 11, Issue 4, pp. 105757-105757