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胃轻瘫:系统性淀粉样变的一种被低估的表现形式。

Gastroparesis: an under-recognised manifestation of systemic amyloidosis.

机构信息

Internal Medicine, University of California San Francisco, Fresno, California, USA

Internal Medicine, Henry Ford Health System, Detroit, Michigan, USA.

出版信息

BMJ Case Rep. 2023 May 2;16(5):e254634. doi: 10.1136/bcr-2023-254634.

Abstract

Gastrointestinal (GI) amyloidosis can be acquired or genetic and is commonly caused by chronic inflammatory illnesses (AA amyloidosis), haematological malignancies (AL amyloidosis) and end-stage renal disease (beta-2 microglobulin amyloidosis). The accumulation of these aberrant proteins disrupts the structures and functions of many organs; the least common of which is the GI tract. GI presentations depend on the type, location and amount of amyloid deposition. Symptoms can range from nausea and vomiting to fatal GI bleeds. Pathological examination of the involved tissue with characteristic green birefringence under polarised light is used to confirm the diagnosis. Patients should be considered for further evaluation to rule out additional organ involvement, notably cardiac and renal. We present a patient with amyloidosis-induced gastroparesis, an under-recognised presentation of systemic amyloidosis in the gastroenterology system.

摘要

胃肠道(GI)淀粉样变可分为获得性或遗传性,通常由慢性炎症性疾病(AA 淀粉样变)、血液系统恶性肿瘤(AL 淀粉样变)和终末期肾病(β-2 微球蛋白淀粉样变)引起。这些异常蛋白的积累会破坏许多器官的结构和功能;其中最不常见的是胃肠道。胃肠道表现取决于淀粉样蛋白沉积的类型、位置和数量。症状范围从恶心和呕吐到致命的胃肠道出血。通过对受累组织进行特征性的绿色双折射在偏光显微镜下检查,用于确诊。应考虑对患者进行进一步评估以排除其他器官受累,特别是心脏和肾脏。我们介绍了一位因淀粉样变性引起的胃轻瘫患者,这是消化系统中系统性淀粉样变性的一种未被充分认识的表现。

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本文引用的文献

1
Systemic amyloidosis from A (AA) to T (ATTR): a review.系统性淀粉样变,从 A(AA)到 T(ATTR):综述。
J Intern Med. 2021 Mar;289(3):268-292. doi: 10.1111/joim.13169. Epub 2020 Sep 14.
3
Gastroparesis in a Patient with Gastric AL Amyloidosis.胃AL型淀粉样变性患者的胃轻瘫
Case Rep Gastroenterol. 2018 Jun 22;12(2):317-321. doi: 10.1159/000489297. eCollection 2018 May-Aug.
5
Systemic amyloidosis.系统性淀粉样变性。
Lancet. 2016 Jun 25;387(10038):2641-2654. doi: 10.1016/S0140-6736(15)01274-X. Epub 2015 Dec 21.
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Gastrointestinal manifestations of amyloidosis.淀粉样变性的胃肠道表现。
Am J Gastroenterol. 2008 Mar;103(3):776-87. doi: 10.1111/j.1572-0241.2007.01669.x. Epub 2007 Dec 12.

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