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胃肠道出血与淀粉样变性

Gastrointestinal bleeding and amyloidosis.

作者信息

Levy D J, Franklin G O, Rosenthal W S

出版信息

Am J Gastroenterol. 1982 Jun;77(6):422-6.

PMID:6979925
Abstract

Although gastrointestinal bleeding is well known in patients with amyloidosis, when it occurs as the initial or sole overt manifestation of the disease it is much rarer and frequently causes diagnostic difficulty. This subject is reviewed in the context of a patient who underwent multiple invasive and noninvasive examinations to achieve this diagnosis. Both upper and lower gastrointestinal bleeding of all degrees has been shown to occur with amyloidosis. It appears that diffuse involvement of the gastrointestinal tract is much more common than a specific lesion. Our patient was ultimately found to have multiple waxy amyloid plaques and extremely friable jejunal mucosa at endoscopy. Radiographically, amyloidosis may cause diffuse thickening of the mucosal folds, a finding that carries a broad differential diagnosis. Arteriography, although not used frequently as a diagnostic modality in amyloidosis, may reveal luminal irregularities, truncation, and diffuse attenuation of the vasculature. The treatment of amyloidosis thus far has been unrewarding and difficult. Several new therapeutic measures which appear promising are discussed.

摘要

尽管胃肠道出血在淀粉样变性患者中很常见,但当它作为该疾病的初始或唯一明显表现出现时则更为罕见,并且常常导致诊断困难。本文结合一名接受了多项侵入性和非侵入性检查以确诊的患者对该主题进行了综述。淀粉样变性已被证实会出现各种程度的上消化道和下消化道出血。胃肠道的弥漫性受累似乎比特定病变更为常见。在内镜检查中,我们的患者最终被发现有多个蜡样淀粉样斑块以及极度脆弱的空肠黏膜。在影像学上,淀粉样变性可能导致黏膜皱襞弥漫性增厚,这一表现的鉴别诊断范围很广。血管造影术虽然在淀粉样变性中不常作为诊断手段使用,但可能显示血管腔不规则、截断以及血管弥漫性变细。迄今为止,淀粉样变性的治疗效果不佳且困难重重。文中讨论了几种看起来有前景的新治疗措施。

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