Kwon John H, Farrell Richard J
Division of Gastroenterology and Hepatology, Johns Hopkins University, Baltimore, Maryland, USA.
Paediatr Drugs. 2006;8(6):375-88. doi: 10.2165/00148581-200608060-00005.
Celiac disease (CD) is an autoimmune condition occurring in genetically susceptible individuals characterized by inflammatory injury to the mucosa of the small intestine after the ingestion of wheat glutens or related barley and rye products. Originally thought to be highly prevalent only in Northern European populations, growing evidence indicates a much higher prevalence in many other regions, including the US as well as South America, North Africa, and Asia. The growing awareness that pediatric patients may present with quite diverse and protean manifestations and the significant impact of CD on childhood development has prompted efforts to increase CD awareness for the early diagnosis and treatment of this disease. The current diagnostic criteria for CD requires characteristic histologic findings in small bowel biopsies and clinical remission when placed on a gluten-free diet. Serologic testing for CD can provide additional support for the diagnosis of CD or a means to assess efficacy and adherence to a gluten-free diet. The mainstay of treatment remains the institution of a gluten-free diet. However, patients with refractory CD may require treatment with immunosuppressant medications. With the increased identification of specific gluten epitopes and understanding of the pathogenesis of CD, future therapies may rely on genetically altering gluten proteins, immunization techniques, or therapies focused on either the development of specific immune tolerance or regulation of mucosal inflammation.
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,其特征是摄入小麦麸质或相关的大麦和黑麦产品后,小肠黏膜发生炎性损伤。最初认为该病仅在北欧人群中高度流行,但越来越多的证据表明,在包括美国、南美洲、北非和亚洲在内的许多其他地区,其患病率要高得多。人们越来越意识到儿科患者可能表现出非常多样和多变的症状,以及乳糜泻对儿童发育的重大影响,这促使人们努力提高对乳糜泻的认识,以便对该疾病进行早期诊断和治疗。目前乳糜泻的诊断标准要求小肠活检有特征性组织学表现,且采用无麸质饮食时临床症状缓解。乳糜泻的血清学检测可为乳糜泻的诊断提供额外支持,或作为评估无麸质饮食疗效和依从性的一种手段。治疗的主要方法仍然是采用无麸质饮食。然而,难治性乳糜泻患者可能需要使用免疫抑制药物进行治疗。随着对特定麸质表位的识别增加以及对乳糜泻发病机制的了解,未来的治疗可能依赖于对麸质蛋白进行基因改造、免疫技术,或侧重于发展特定免疫耐受性或调节黏膜炎症的治疗方法。