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淀粉样变与胃肠道。

Amyloid and the GI tract.

机构信息

National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, UCL Medical School, Royal Free Hospital Campus, Rowland Hill Street, London NW3 2PF, UK.

出版信息

Expert Rev Gastroenterol Hepatol. 2009 Dec;3(6):615-30. doi: 10.1586/egh.09.59.

Abstract

Amyloidosis is characterized by the extracellular deposition of an abnormal fibrillar protein, which disrupts tissue structure and function. Amyloid may be localized to a single organ, such as the GI tract, or be systemic where the amyloid type is defined by the respective fibril precursor protein. Among patients with systemic amyloidosis, histological involvement of the gastrointestinal (GI) tract is very common but often subclinical. The presence and pattern of GI symptoms varies substantially, not only between the different amyloid types but also within them. GI presentations are frequently nonspecific and include macroglossia, dyspepsia, hemorrhage, a change in bowel habit and malabsorption. Endoscopic and radiological features of amyloidosis are also nonspecific, with the small intestine most commonly affected. In the absence of specific treatments for GI amyloidosis, therapy is aimed at reducing or eliminating the supply of the respective fibril precursor protein. Supportive measures such as nutritional support and antidiarrheal agents should be instigated while awaiting the clinical improvement associated with a successful reduction in the abundance of the fibril precursor protein. GI tract surgery should be performed only if the benefits clearly outweigh the risks, as there is a risk of decompensation of organs affected by amyloid.

摘要

淀粉样变性的特征是细胞外异常纤维状蛋白的沉积,这会破坏组织结构和功能。淀粉样物质可能局限于单个器官,如胃肠道,也可能是系统性的,其中淀粉样类型由相应的纤维原蛋白定义。在系统性淀粉样变性患者中,胃肠道(GI)受累非常常见,但通常为亚临床。GI 症状的存在和模式有很大差异,不仅在不同的淀粉样变性类型之间,而且在同一类型内也有很大差异。GI 表现通常是非特异性的,包括巨舌、消化不良、出血、排便习惯改变和吸收不良。淀粉样变性的内镜和影像学特征也不具有特异性,小肠最常受累。由于缺乏针对 GI 淀粉样变性的特定治疗方法,治疗旨在减少或消除相应纤维原蛋白的供应。在等待与纤维原蛋白丰度成功减少相关的临床改善的同时,应采取支持性措施,如营养支持和止泻药。只有当获益明显超过风险时,才应进行胃肠道手术,因为淀粉样物质会使受累器官失代偿的风险增加。

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