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乳糜泻

Celiac disease.

作者信息

Rodrigo Luis

机构信息

Gastroenterology Service, Hospital Universitario Central de Asturias, c/Celestino Villamil s. n . 33.006. Oviedo, Spain.

出版信息

World J Gastroenterol. 2006 Nov 7;12(41):6585-93. doi: 10.3748/wjg.v12.i41.6585.

Abstract

Celiac disease (CD) is a common autoimmune disorder, induced by the intake of gluten proteins present in wheat, barley and rye. Contrary to common belief, this disorder is a protean systemic disease, rather than merely a pure digestive alteration. CD is closely associated with genes that code HLA-II antigens, mainly of DQ2 and DQ8 classes. Previously, it was considered to be a rare childhood disorder, but is actually considered a frequent condition, present at any age, which may have multiple complications. Tissue transglutaminase-2 (tTG), appears to be an important component of this disease, both, in its pathogenesis and diagnosis. Active CD is characterized by intestinal and/or extra-intestinal symptoms, villous atrophy and crypt hyperplasia, and strongly positive tTG auto-antibodies. The duodenal biopsy is considered to be the "gold standard" for diagnosis, but its practice has significant limitations in its interpretation, especially in adults. Occasionally, it results in a false-negative because of patchy mucosal changes and the presence of mucosal villous atrophy is often more severe in the proximal jejunum, usually not reached by endoscopic biopsies. CD is associated with increased rates of several diseases, such as iron deficiency anemia, osteoporosis, dermatitis herpetiformis, several neurologic and endocrine diseases, persistent chronic hypertransami-nasemia of unknown origin, various types of cancer and other autoimmune disorders. Treatment of CD dictates a strict, life-long gluten-free diet, which results in remission for most individuals, although its effect on some associated extraintestinal manifestations remains to be established.

摘要

乳糜泻(CD)是一种常见的自身免疫性疾病,由摄入小麦、大麦和黑麦中的麸质蛋白所诱发。与普遍看法相反,这种疾病是一种具有多种表现的全身性疾病,而非仅仅是单纯的消化改变。CD与编码HLA-II抗原的基因密切相关,主要是DQ2和DQ8类。以前,它被认为是一种罕见的儿童疾病,但实际上被视为一种常见病症,可出现在任何年龄,且可能有多种并发症。组织转谷氨酰胺酶-2(tTG)似乎是这种疾病发病机制和诊断中的一个重要组成部分。活动性CD的特征是肠道和/或肠道外症状、绒毛萎缩和隐窝增生,以及tTG自身抗体呈强阳性。十二指肠活检被认为是诊断的“金标准”,但其实施在解读方面有显著局限性,尤其是在成人中。偶尔,由于黏膜改变呈斑片状,它会导致假阴性,而且黏膜绒毛萎缩在空肠近端通常更严重,而内镜活检通常无法到达此处。CD与多种疾病的发病率增加相关,如缺铁性贫血、骨质疏松症、疱疹样皮炎、多种神经和内分泌疾病、不明原因的持续性慢性转氨酶升高、各种类型的癌症以及其他自身免疫性疾病。CD的治疗要求严格的终身无麸质饮食,这会使大多数患者病情缓解,尽管其对一些相关肠道外表现的影响仍有待确定。

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