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乳糜泻的新见解。

New insights in celiac disease.

作者信息

Branski David

机构信息

Hadassah University Hospital, The Hebrew University Hadassah Medical School, Jerusalem, Israel; Editor-in-Chief, Journal of Pediatric Gastroenterology and Nutrition, Europe.

出版信息

Rambam Maimonides Med J. 2012 Jan 31;3(1):e0006. doi: 10.5041/RMMJ.10073. Print 2012 Jan.

Abstract

Celiac disease (CD) is an autoimmune disorder occurring in genetically susceptible subjects. The incidence of CD is around 1%, and it is much more common in first-degree relatives of CD patients, 10%-18%. However, the pattern of the genetic inheritance is still obscure. Environmental factors are undoubtedly affecting the disease's clinical presentation, time at presentation, and may have an effect on the characteristics of the disease. The clinical presentation of CD has shifted during the previous decades from the classical presentation in which the toddler suffers from diarrhea, constipation, vomiting, failure to thrive, abdominal distension, etc., to the child with a monosymptomatic presentation, such as anemia, as well as an enlarged list of extra-intestinal disorders. The diagnosis of CD is being established by symptoms consistent with CD and positive serology. The ultimate diagnosis should be made upon histological evaluation of the small bowel mucosa. The treatment of CD is a lifelong, strict gluten-free diet (GFD). Compliance with a GFD is quite difficult. Therefore, new strategies for prevention and treatment modalities other than GFD are greatly needed. Recently several promising therapeutic modalities have been developed; these include resuming traditional baking techniques. Another methodology is using probiotic-driven prolylendopeptidase. Another pathway to tackle the therapeutic option in CD is by down-regulation of the activity of zonulin-the active pump enabling gluten to enter the enterocytes. We are facing an era where other modalities beyond a GFD might allow CD patients to be able to tolerate occasionally a small amount of gluten in their diet.

摘要

乳糜泻(CD)是一种发生在遗传易感性个体中的自身免疫性疾病。CD的发病率约为1%,在CD患者的一级亲属中更为常见,为10%-18%。然而,遗传遗传模式仍不明确。环境因素无疑会影响疾病的临床表现、发病时间,并且可能对疾病特征产生影响。在过去几十年中,CD的临床表现已从幼儿出现腹泻、便秘、呕吐、生长发育迟缓、腹胀等典型表现,转变为以单一症状表现的儿童,如贫血,以及一系列肠道外疾病。CD的诊断依据与CD相符的症状和血清学阳性结果来确立。最终诊断应基于小肠黏膜的组织学评估。CD的治疗是终身严格的无麸质饮食(GFD)。遵守GFD相当困难。因此,迫切需要除GFD之外的预防和治疗方式的新策略。最近已开发出几种有前景的治疗方式;这些包括恢复传统烘焙技术。另一种方法是使用益生菌驱动的脯氨酰内肽酶。解决CD治疗选择的另一条途径是下调zonulin的活性,zonulin是使麸质能够进入肠细胞的活性泵。我们正面临一个时代,除了GFD之外的其他方式可能使CD患者能够偶尔耐受饮食中少量的麸质。

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