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Celiac Disease

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Celiac Disease
Dascha C. Weir, MD
Ciaran Kelly, MD

Celiac disease (CD) is an immune-mediated enteropathy secondary to permanent sensitivity to wheat gluten and related proteins in rye and barley. It results in characteristic histologic changes consisting of inflammation, crypt hyperplasia, and villous atrophy of the small intestine in genetically susceptible individuals. Significant variability in the clinical presentation of
CD in the pediatric population complicates recognition of the disease in many patients. Treatment for CD consists of a lifelong strict gluten-free diet (GFD). Adherence to this diet is associated with resolution of most related signs and symptoms and a decreased risk of related complications. With an explosion of new knowledge over the span of two decades, our understanding of
CD has changed dramatically. CD has gone from a rare condition causing gastrointestinal symptoms in children of European origin to a common disorder causing symptoms that affect multiple organ systems in all ages virtually worldwide.1 The overall prevalence of CD is similar in
Europe and North America affecting up to 1% of the population.2,3 A large multicenter study in the United States, using serologic screening with biopsy confirmation to identify cases of
CD, showed a prevalence of CD of 1:133 in individuals with no evident risk factors. Prevalence of CD in symptomatic patients was 1:56. The prevalence of diagnosed CD is much lower, especially in the United States. This reflects underrecognition and underdiagnosis related to the wide clinical spectrum of disease and the presence of silent disease.4 In the United States, the prevalence of CD in people of non-European descent is not known. Limited studies suggest that, in the United States, Hispanic and AfricanAmerican populations have a lower prevalence of CD than non-Hispanic

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