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腹膜淀粉样变作为AL淀粉样变的首发表现

Peritoneal Amyloid as a Presenting Manifestation of AL Amyloid.

作者信息

Acharya Indira, Dominah Gifty A, Xing Dongmei, Allen Elizabeth, Iding Jeffrey S, Haas Christopher J

机构信息

Medstar Health Internal Medicine Residency Program, Baltimore, MD, USA.

Medstar Health, Department of Pathology, Baltimore, MD, USA.

出版信息

J Community Hosp Intern Med Perspect. 2023 May 8;13(3):96-102. doi: 10.55729/2000-9666.1193. eCollection 2023.

Abstract

Amyloid is a systemic disease characterized by extracellular deposition of misfolded protein. Gastrointestinal and peritoneal deposition of light chain (AL) amyloid is an under-recognized manifestation of this systemic disease, usually as a late sequela. Here we present a case of recently diagnosed AL peritoneal amyloid that presented in the context of recurrent, acute onset abdominal discomfort and was found to have bowel obstruction complicated by perforation in the setting of AL-mediated gastrointestinal tract infiltration and dysmotility.

摘要

淀粉样变性是一种全身性疾病,其特征是错误折叠的蛋白质在细胞外沉积。轻链(AL)淀粉样蛋白在胃肠道和腹膜的沉积是这种全身性疾病一种未被充分认识的表现,通常是晚期后遗症。在此,我们报告一例近期诊断的AL腹膜淀粉样变性病例,该病例表现为反复急性发作的腹部不适,在AL介导的胃肠道浸润和动力障碍的情况下,发现存在肠梗阻并伴有穿孔。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d30/10593169/490853ff2276/jchim-13-03-096f1.jpg

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