Department of Nephrology, Hasheminejad Kidney Center, Iran University of Medical Sciences (IUMS), Tehran, Iran.
Iran J Kidney Dis. 2023 May;17(3):141-149.
Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary kidney disease that can affect several organs. The clinical course of the disease varies among patients; some never become symptomatic, and others reach end-stage kidney disease (ESKD) in the 5th decade of their life.
This historical cohort study was conducted on ADPKD patients to investigate kidney and patient survival rates and related risk factors in Iran. Survival analysis and risk ratio calculation were performed using the Cox proportional hazards model, Kaplan- Meier method, and log-rank test.
Among the 145 participants, 67 developed ESKD, and 20 died before the end of the study period. Developing chronic kidney disease (CKD) at the age of ≤ 40, baseline serum creatinine level (SCr) of more than 1.5 mg/dL, and cardiovascular disease increased the risk of ESKD by 4, 1.8, and 2.4 times; respectively. Patient survival analysis revealed a fourfold increase in mortality if the glomerular filtration rate (GFR) declined more than 5 cc/min annually and if CKD was diagnosed at the age of ≤ 40. Vascular thrombotic events or ESKD in the course of disease increased the risk of death by approximately 6- and 7-fold, respectively. Kidney survival was 48% by the age of 60 and 28% by the age of 70. Patient survival was 86.05% at the age of 60 and 67.99% at the age of 70. Additionally, men had a significantly better renal function and survival than women.
Elevated baseline SCr and cardiovascular disease can increase ESKD risk in ADPKD patients. A rapid decline in GFR, ESKD development, and vascular thrombotic events increase the risk of death, but early CKD can affect both. DOI: 10.52547/ijkd.7551.
常染色体显性遗传性多囊肾病（ADPKD）是一种遗传性肾病，可影响多个器官。该病患者的临床病程存在差异；有些患者从未出现症状，而有些患者在 50 岁时就进入终末期肾病（ESKD）。
本历史队列研究对伊朗的 ADPKD 患者进行了调查，以研究其肾脏和患者生存率及相关危险因素。采用 Cox 比例风险模型、Kaplan-Meier 法和对数秩检验进行生存分析和风险比计算。
在 145 名参与者中，有 67 人发展为 ESKD，有 20 人在研究结束前死亡。在≤40 岁时发展为慢性肾脏病（CKD）、基线血清肌酐（SCr）水平>1.5mg/dL 和心血管疾病，分别使 ESKD 的风险增加了 4 倍、1.8 倍和 2.4 倍。患者生存分析显示，如果肾小球滤过率（GFR）每年下降超过 5cc/min，或者 CKD 在≤40 岁时诊断，死亡率会增加 4 倍。疾病过程中的血管血栓事件或 ESKD 使死亡风险增加约 6 倍和 7 倍。到 60 岁时，肾脏的存活率为 48%，到 70 岁时为 28%。到 60 岁时，患者的生存率为 86.05%，到 70 岁时为 67.99%。此外，男性的肾功能和生存率均优于女性。
基线 SCr 升高和心血管疾病会增加 ADPKD 患者发生 ESKD 的风险。GFR 快速下降、ESKD 发生和血管血栓事件会增加死亡风险，但早期 CKD 也会影响这两个方面。DOI：10.52547/ijkd.7551.
