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在开始腹膜透析时发生的系统性AA型淀粉样变性,伴有腹膜淀粉样沉积。

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

作者信息

Habuka Masato, Sakurazawa Chihiro, Sakamaki Yuichi, Ogawa Asa, Yamamoto Suguru, Narita Ichiei

机构信息

Division of Nephrology, Niigata Prefectural Shibata Hospital, 1-2-8 Honcho, Shibata City, Niigata, 957-8588, Japan.

Division of Clinical Nephrology and Rheumatology, Kidney Research Center, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

出版信息

CEN Case Rep. 2025 Jun;14(3):408-412. doi: 10.1007/s13730-025-00981-8. Epub 2025 Mar 4.

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.

摘要

淀粉样变性的特征是由疾病特异性前体蛋白形成的不溶性淀粉样纤维在全身各器官的细胞外间质中沉积,导致器官损伤。淀粉样变性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。在管理循环动力学和清除淀粉样纤维的前体蛋白方面,腹膜透析(PD)优于血液透析(HD)。然而,使用淀粉样物质沉积的腹膜进行PD的临床过程尚未见报道。在本文中,我们描述了1例罕见的系统性AA淀粉样变性病例,在开始PD时腹膜有淀粉样物质沉积。PD开始时的腹膜平衡试验(PET)显示转运速率高。透析液暂时改为高糖浓度腹膜透析液,并添加每周1次的体外超滤方法。患者继续进行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并保持在正常范围内。透析液引流中未检测到淀粉样蛋白A。总之,淀粉样物质沉积的腹膜对PD的临床过程没有一致的影响。此外,淀粉样变性治疗可改变伴有淀粉样物质沉积的腹膜功能。然而,未来的研究应调查淀粉样变性治疗改变腹膜功能的确切机制。

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本文引用的文献

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Systemic Amyloidosis and Kidney Transplantation: An Update.系统性淀粉样变与肾移植:最新进展
Semin Nephrol. 2024 Jan;44(1):151496. doi: 10.1016/j.semnephrol.2024.151496. Epub 2024 Mar 14.
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