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The relationship between celiac disease and juvenile diabetes has long been known. Only a single study in the United States, from Buffalo, New York, has reported the prevalence of celiac disease in a pediatric diabetic population. This study was conducted to determine the prevalence and clinical presentation of celiac disease in children and adolescents with juvenile diabetes in Wisconsin, U.S.A., using serum antiendomysial antibody as a screening test.Two hundred eighteen patients with diabetes (113 males; age range, 4–21 years) and 117 age-and gender-matched control participants were tested for immunoglobulin A endomysial antibody. Patients with positive results were offered a small bowel biopsy. A questionnaire regarding abdominal pain, diarrhea, and growth failure was completed by the parents.Seventeen of 218 diabetic patients (7.7%) had positive endomysial antibody. All control participants had negative results for the endomysial antibody. Small bowel biopsy was performed in 14 patients. Ten patients had villous atrophy. In one patient without villous atrophy, a repeat biopsy 2 years later showed villous atrophy, and two patients had increased intraepithelial lymphocytes without villous atrophy. Seventy percent of the patients with celiac disease were asymptomatic. The reported symptoms were abdominal pain and diarrhea (n = 1) and growth failure (n = 2). Two patients with celiac disease had Down syndrome.The prevalence of celiac disease in children with juvenile diabetes in Wisconsin is at least 4.6%, which is comparable with European and Canadian studies. Because patients without villous atrophy may have latent celiac disease, the prevalence may be even higher. All children with juvenile diabetes should be screened for celiac disease.