Federation of international societies of pediatric gastroenterology, hepatology, and nutrition consensus report on celiac disease
Federation of international societies of pediatric gastroenterology, hepatology, and nutrition consensus report on celiac disease
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DOI:
10.1097/mpg.0b013e318181afed
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发表时间:
2008-08-01
影响因子:
2.9
通讯作者:
Phillips, A.
中科院分区:
文献类型:
--
作者:
Fasano, A.;Araya, M.;Phillips, A.
Celiac disease (CD) is a gluten-sensitive, immunemediated chronic enteropathy with a wide range of manifestations of variable severity. It is triggered by the ingestion of gliadin fractions of wheat gluten and similar alcohol-soluble proteins (prolamines) of barley and rye in genetically susceptible subjects. The subsequent immune reaction leads to small bowel inflammation and villous atrophy. Adherence to a gluten-free diet (GFD) is followed by amelioration or normalization of the villous architecture. CD represents a ‘‘unique’’autoimmune disease in that the environmental factor triggering the immune response (gluten) is known. CD not only affects the gut but is also a systemic disease that may cause injury to extraintestinal organs as well. Human leukocyte antigen (HLA) status appears to be the strongest genetic determinant of risk for celiac autoimmunity, because of the role that specific HLA class II alleles play in the presentation of gluten to T cells. Of the affected individuals, 95% have either DQ2 (HLA-DQA1Ã05-DQB1Ã02) or DQ8 (HLADQA1Ã03-DQB1Ã0302), in comparison with the general population in which about 30% to 35% have either DQ2 or DQ8 (1, 2). Besides HLA II class genes, there is evidence for involvement of other genes located on chromosomes 2 (2q33), 5 (5q31-q33), and 19 (19p13. 1), and in the region harboring interleukin-2 (IL-2) and IL-21 (3–6).ThetrueprevalenceofCDisdifficulttoestimatebecause of its variable clinical presentation, and many patients can have few or no symptoms. With a better appreciation of its clinical complexity and the availability of sensitive and specific screening tests, CD is now considered a public health problem worldwide. CD affects as much as 0.5% to 1.0% of European or European ancestry populations, but most cases remain undiagnosed (7–11).