An end-stage kidney disease patient undergoing dialysis treatment for 50 years with dialysis-related amyloidosis in multiple organs.

IF 0.7 Q4 UROLOGY & NEPHROLOGY
CEN Case Reports Pub Date : 2025-08-01 Epub Date: 2025-06-01 DOI:10.1007/s13730-025-01003-3
Toru Ito, Takashi Kato, Suguru Yamamoto, Hisaki Shimada, Hideyuki Kabasawa, Shin Goto, Riuko Ohashi, Ichiei Narita
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Abstract

Advancements in dialysis treatment have led to an increase in the number of patients undergoing long-term dialysis. These patients often experience a decline in activities of daily living (ADL) owing to various chronic kidney disease-related conditions such as mineral and bone disorders, dialysis-related amyloidosis (DRA), and cardiovascular diseases. A male patient began hemodialysis at the age of 22 years because of end-stage kidney disease caused by chronic glomerulonephritis and continued dialysis treatment for 50 years. The patient died at 72 years of age from sepsis, ischemic colitis, and pneumonia. During the course of treatment, the patient frequently underwent surgeries for carpal tunnel syndrome and destructive spondyloarthropathy, which led to a decline in ADL. An autopsy revealed β2-microglobulin amyloid deposits in multiple organs, including the heart, lungs, and intestines. Long-term dialysis treatment results in ADL impairment owing to osteoarticular disorders caused by DRA, and systemic organ deposits are potentially related to organ failures such as ischemic colitis and pneumonia. Elucidating the pathophysiology of DRA and developing more effective treatments may improve ADL and prognosis in patients undergoing long-term dialysis.

终末期肾病患者透析治疗50年多器官与透析相关的淀粉样变性。
透析治疗的进步导致接受长期透析的患者数量增加。这些患者通常会经历日常生活活动(ADL)的下降,原因是各种慢性肾脏疾病相关疾病,如矿物质和骨骼疾病、透析相关淀粉样变性(DRA)和心血管疾病。一位男性患者因慢性肾小球肾炎引起的终末期肾病,22岁开始血液透析,并持续透析治疗50年。患者死于败血症、缺血性结肠炎和肺炎,享年72岁。在治疗过程中,患者频繁因腕管综合征和破坏性脊椎关节病接受手术,导致ADL下降。尸检显示β2-微球蛋白淀粉样蛋白沉积在多个器官,包括心脏、肺和肠。由于DRA引起的骨关节紊乱,长期透析治疗导致ADL损伤,而系统性器官沉积可能与缺血性结肠炎和肺炎等器官衰竭有关。阐明DRA的病理生理学和开发更有效的治疗方法可以改善长期透析患者的ADL和预后。
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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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