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继发于超敏性血管炎的胃肠道淀粉样变性伴肠假性梗阻。

Gastrointestinal amyloidosis secondary to hypersensitivity vasculitis presenting with intestinal pseudoobstruction.

作者信息

Hiramatsu K, Kaneko S, Shirota Y, Matsuda M, Kaji K, Kitano Y, Ikeda N, Terasaki S, Kawai H, Shimoda A, Yokoyama H, Matsushita E, Urabe T, Kobayashi K

机构信息

First Department of Internal Medicine, Kanazawa University School of Medicine, Ishikawa, Japan.

出版信息

Dig Dis Sci. 1998 Aug;43(8):1824-30. doi: 10.1023/a:1018856324810.

Abstract

A 22-year-old woman developed sudden hepatic encephalopathy and severe intestinal bleeding. She was diagnosed with acute fatty liver and hypersensitivity vasculitis and was successfully treated with whole plasma exchange, methylprednisolone pulse therapy, and transcatheter arterial embolization. Twenty-seven months later, she began complaining of lower abdominal fullness, tenderness, and nausea and vomiting. Histologic examination showed that she had developed gastrointestinal and renal amyloidosis with intestinal pseudoobstruction and proteinuria. The immunohistochemical study of the stomach, rectum, and kidney with anti-amyloid A fluorescent antibody showed that the systemic amyloid deposit was secondary to her underlying disease. This is the first report of amyloidosis occurring secondary to hypersensitivity vasculitis.

摘要

一名22岁女性突发肝性脑病和严重肠道出血。她被诊断为急性脂肪肝和过敏性血管炎,并通过全血浆置换、甲泼尼龙冲击疗法和经导管动脉栓塞术成功治疗。27个月后,她开始抱怨下腹部胀满、压痛以及恶心和呕吐。组织学检查显示她已发展为胃肠道和肾淀粉样变性,伴有肠道假性梗阻和蛋白尿。用抗淀粉样蛋白A荧光抗体对胃、直肠和肾脏进行免疫组织化学研究表明,全身性淀粉样沉积物是其基础疾病的继发表现。这是首例关于过敏性血管炎继发淀粉样变性的报道。

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