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
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A large center in New York City reported that African-Americans comprised 1.3% of their patients with biopsy-proven CD.6 This is likely partially because of genetic differences in these populations. However, given the increasing recognition of CD in non-Caucasian populations internationally, serious consideration should be
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given to the possibility of underdiagnosis in minority populations in the United States.
CD is being increasingly diagnosed in other parts of the world, involving populations that were not traditionally thought to be affected by the disease. Reports from Mexico, South America,
North India, the Middle East, Turkey, and North
Africa have changed previous assumptions that
CD is a European affliction.7–15 CD is rare in people of Japanese, Chinese, and purely AfricanCaribbean descent.16
CD is associated with several autoimmune conditions and genetic disorders and often presents atypically or silently in these populations. In children, it is associated with autoimmune thyroid disease and with type I diabetes mellitus with a 7.8 and 4.5% prevalence of CD in affected children.17,18 Up to 7% of patients with a selective IgA deficiency also have CD.19 Patients with
Trisomy 21, Turner syndrome, and Williams syndrome have also been found to be at higher

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