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Coeliac disease (British English) or celiac disease (American English) is a long-term autoimmune disorder, primarily affecting the small intestine, where individuals develop intolerance to gluten, present in foods such as wheat, rye and barley. [10]
This condition is known as refractory coeliac disease (RCD), defined as malabsorption due to gluten-related enteropathy (villous atrophy or elevated intraepitheal lymphocytes) after initial or subsequent failure of a strict gluten-free diet (usually 1 year) and after exclusion of any disorder mimicking coeliac disease. [106] [107]
[41] [42] [33] As occurs in people with coeliac disease, the treatment is a gluten-free diet (GFD) strict and maintained, without making any dietary transgression. [37] Whereas coeliac disease requires adherence to a strict lifelong gluten-free diet, it is not yet known whether NCGS is a permanent, or a transient condition.
Celiac disease affects ~1% of the population in most parts of the world. [3] Ninety to one hundred percent of patients with coeliac disease have inherited genes at the HLA-DQ locus that encode HLA-DQ2 and/or HLA-DQ8 serotype proteins. [12] About 2–3% of individuals who inherit these HLA-DQ2 and/or HLA-DQ8 serotypes develop coeliac disease. [10]
CD affects approximately 1–2% of the general population, [11] but most cases remain unrecognized, undiagnosed and untreated, and at risk for serious long-term health complications. [11] [45] [58] [59] People may suffer severe disease symptoms and be subjected to extensive investigations for many years, before a proper diagnosis is achieved. [55]
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Coeliac disease with "classic symptoms", which include gastrointestinal manifestations such as chronic diarrhea and abdominal distention, malabsorption, loss of appetite, and impaired growth, is currently the least common presentation of the disease and affects predominantly small children generally younger than two years of age. [29] [31]
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