Systemic AA amyloidosis with amyloid deposition in the peritoneum at the time of initiating peritoneal dialysis.

IF 1 Q4 UROLOGY & NEPHROLOGY
Masato Habuka, Chihiro Sakurazawa, Yuichi Sakamaki, Asa Ogawa, Suguru Yamamoto, Ichiei Narita
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Abstract

Amyloidosis is characterized by the deposition of insoluble amyloid fibrils formed by disease-specific precursor proteins in the extracellular interstitium of various organs throughout the body, resulting in organ damage. Patients with amyloidosis often develop end-stage kidney disease (ESKD), which can be managed with dialysis or kidney transplantation. Peritoneal dialysis (PD) is advantageous over hemodialysis (HD) in managing the circulatory dynamics and removing the precursor proteins of amyloid fibrils. However, the clinical course of PD using an amyloid-deposited peritoneum has not been reported. In this paper, we describe a rare case of systemic AA amyloidosis with amyloid deposition in the peritoneum at the beginning of PD. The peritoneal equilibrium test (PET) at PD initiation revealed a high transport rate. The dialysis solution was temporarily changed to a high-glucose concentration peritoneal dialysate, and a weekly extracorporeal ultrafiltration method was added. The patient continued with PD treatment without any complications. The PET category changed from "high" to "high average" during the subsequent PD treatment course. The serum amyloid A levels improved post-nephrectomy and remained in the normal range. Amyloid A was not detected in the dialysate drainage. In conclusion, the amyloid-deposited peritoneum has no uniform effect on the clinical course of PD. Moreover, amyloidosis therapy can alter the peritoneal function with amyloid deposition. However, future studies should investigate the exact mechanism of the alteration of peritoneal function with amyloidosis therapy.

开始腹膜透析时腹膜淀粉样沉积的系统性AA淀粉样变性。
淀粉样变性的特点是由疾病特异性前体蛋白形成的不溶性淀粉样原纤维沉积在全身各器官的细胞外间质中,导致器官损伤。淀粉样变患者常发展为终末期肾病(ESKD),可通过透析或肾移植进行治疗。腹膜透析(PD)在控制循环动力学和去除淀粉样原纤维前体蛋白方面优于血液透析(HD)。然而,使用淀粉样蛋白沉积腹膜的PD的临床过程尚未报道。在本文中,我们描述了一个罕见的系统性AA淀粉样变性与淀粉样沉积在腹膜在PD开始。腹膜平衡试验(PET)在PD开始时显示高转运率。暂时将透析液改为高糖浓度腹膜透析液,并每周加入体外超滤方法。患者继续PD治疗,无任何并发症。在随后的PD治疗过程中,PET类别从“高”变为“高平均”。血清淀粉样蛋白A水平在肾切除术后改善,维持在正常范围内。透析液引流液未检出淀粉样蛋白A。综上所述,淀粉样蛋白沉积的腹膜对PD的临床病程没有统一的影响。此外,淀粉样变性治疗可通过淀粉样蛋白沉积改变腹膜功能。然而,未来的研究应探讨淀粉样变治疗改变腹膜功能的确切机制。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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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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