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胃肠道孤立性淀粉样变。

Isolated amyloidosis of the gastro-intestinal tract.

机构信息

Gastroenterologist in training, University hospital of Ghent.

Department of anatomopathology Sint-Andries hospital Tielt.

出版信息

Acta Gastroenterol Belg. 2022 Jan-Mar;85(1):80-84. doi: 10.51821/85.1.8499.

DOI:10.51821/85.1.8499
PMID:35304997
Abstract

Amyloidosis is a very rare condition, which, due to its rarity, is often missed or diagnosed in an advanced stage of the disease, causing significant morbidity and mortality. In this review we describe the existing types of amyloidosis focusing on the gastro-intestinal tract. Amyloidosis occurs when abnormal protein fibrils (amyloid) deposit in the muscularis mucosae. This can cause an array of symptoms ranging from (in order of occurrence): gastro-intestinal bleeding, heartburn, unintentional weight loss, early satiety, constipation, diarrhea, nausea, vomiting and fecal incontinence (1). Treatment is focused on the underlying condition (if any) causing the production and deposition of the abnormal fibrils, in combination of symptomatic treatment.

摘要

淀粉样变性是一种非常罕见的病症,由于其罕见性,常被漏诊或在疾病晚期才被诊断,导致发病率和死亡率显著升高。在本综述中,我们描述了现有的淀粉样变性类型,重点关注胃肠道。淀粉样变性是指异常蛋白纤维(淀粉样物质)在黏膜肌层沉积。这会引起一系列症状,按发生频率依次为:胃肠出血、胃灼热、非故意体重减轻、早饱、便秘、腹泻、恶心、呕吐和大便失禁(1)。治疗侧重于引起异常纤维产生和沉积的潜在疾病(如有),并结合对症治疗。

相似文献

1
Isolated amyloidosis of the gastro-intestinal tract.胃肠道孤立性淀粉样变。
Acta Gastroenterol Belg. 2022 Jan-Mar;85(1):80-84. doi: 10.51821/85.1.8499.
2
Gastric amyloidosis presenting with severe weight loss.以严重体重减轻为表现的胃淀粉样变性
BMJ Case Rep. 2010 Aug 13;2010:bcr0120102650. doi: 10.1136/bcr.01.2010.2650.
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Massive intestinal hemorrhage associated with intestinal amyloidosis. An investigation of underlying pathologic processes.与肠道淀粉样变性相关的大量肠道出血。潜在病理过程的研究。
Gen Diagn Pathol. 1995 Oct;141(2):147-54.
4
Small bowel amyloidosis.小肠淀粉样变性
Curr Gastroenterol Rep. 2013 Oct;15(10):350. doi: 10.1007/s11894-013-0350-4.
5
Gastrointestinal amyloidosis: a case of chronic diarrhoea.胃肠道淀粉样变性:一例慢性腹泻病例
Eur Rev Med Pharmacol Sci. 2009 Mar;13 Suppl 1:45-50.
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Gastrointestinal Manifestations in Hereditary Transthyretin Amyloidosis associated with Glu89Gln Mutation.遗传性转甲状腺素蛋白淀粉样变性相关Glu89Gln 突变患者的胃肠道表现。
J Gastrointestin Liver Dis. 2019 Dec 9;28(4):421-426. doi: 10.15403/jgld-362.
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Intestinal amyloidosis: Clinical manifestations and diagnostic challenge.肠道淀粉样变性:临床表现和诊断挑战。
Adv Clin Exp Med. 2021 May;30(5):563-570. doi: 10.17219/acem/133521.
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Endoscopic and histopathological features of gastrointestinal amyloidosis.胃肠道淀粉样变性的内镜及组织病理学特征
World J Gastrointest Endosc. 2011 Aug 16;3(8):157-61. doi: 10.4253/wjge.v3.i8.157.
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Recurrent diffuse gastric bleeding as a leading symptom of gastrointestinal AL amyloidosis.反复弥漫性胃出血作为胃肠道AL型淀粉样变性的主要症状。
Z Gastroenterol. 2017 Dec;55(12):1318-1322. doi: 10.1055/s-0043-120924. Epub 2017 Dec 6.
10
[Amyloidosis of the intestinal tract (author's transl)].[肠道淀粉样变性(作者译)]
Leber Magen Darm. 1981 Jun;11(3):105-11.

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