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乳糜泻:不断变化的观点。

Coeliac disease: changing views.

作者信息

Mulder C J J, Cellier C

机构信息

Department Gastroenterology, VU University Medical Center, P.O. Box 7057, 1007 MB, Amsterdam.

出版信息

Best Pract Res Clin Gastroenterol. 2005 Jun;19(3):313-21. doi: 10.1016/j.bpg.2005.01.006.

Abstract

A continuing flow of new scientific developments concerning coeliac disease in the last decade asks for the formulation of new concepts of pathophysiology and clinical considerations. Immunogenetic studies have shown a correlation of the disease to the HLA region on the short arm of chromosome 6, immunological research has led to the concept of a T-cell driven immunologic response of the small intestine, with the identification of highly sensitive and specific antibodies. The understanding of the histopathology of coeliac disease has changed dramatically, initiated by the proposition of a spectrum of gluten sensitive enteropathy by Marsh in 1992. Clinical studies report a significant change in patient characteristics and epidemiology. The incidence of the disease has shifted to a majority of adult coeliacs, the disease may present with less severe symptoms of malabsorption and the screening studies suggest an overall prevalence of up to 1 in 200-300. Histopathology has been standardized; lymphocytic enteritis (Marsh I), lymphocytic enteritis with crypthyperplasia (Marsh II), and villous atrophy, subdivided in partial, subtotal and total (Marsh IIIABC). Special attention is given to a subgroup of 'refractory coeliacs', including the identification of pre-malignant T-cells in the intestinal mucosa. The management of coeliacs primarely consists of monitoring for compliance and complications. Dietetic and medical associations need to establish protocols and offer additional training to undergraduetes, internships, general practitioners and other allied health professionals. It might be relevant to have a low threshold for intestinal biopsies. However, screening asymptomatics may be harmful for individuals. Research is needed to assess the benefits of mass-screening in the future. HLA analysis can contribute towards recognising populations at increased risk.

摘要

在过去十年中,有关乳糜泻的新科学进展不断涌现,这就需要制定新的病理生理学概念和临床考量。免疫遗传学研究表明,该疾病与6号染色体短臂上的HLA区域相关,免疫学研究催生了小肠T细胞驱动免疫反应的概念,并鉴定出了高度敏感和特异的抗体。自1992年马什提出一系列麸质敏感性肠病以来,对乳糜泻组织病理学的认识发生了巨大变化。临床研究报告了患者特征和流行病学的显著变化。该疾病的发病率已转向以成年乳糜泻患者为主,疾病可能表现出不太严重的吸收不良症状,筛查研究表明总体患病率高达200至300人中就有1人。组织病理学已实现标准化;淋巴细胞性肠炎(马什I型)、伴有隐窝增生的淋巴细胞性肠炎(马什II型)以及绒毛萎缩,后者又细分为部分、次全和完全(马什III A、B、C型)。特别关注“难治性乳糜泻”亚组,包括在肠道黏膜中识别癌前T细胞。乳糜泻的管理主要包括监测依从性和并发症。饮食和医学协会需要制定方案,并为本科生、实习生、全科医生和其他相关健康专业人员提供额外培训。对肠道活检设定较低阈值可能是有必要的。然而,对无症状者进行筛查可能对个体有害。未来需要开展研究来评估大规模筛查的益处。HLA分析有助于识别风险增加的人群。

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