Objective. This study was undertaken to investigate the prevalence of celiac disease in children and adolescents with Down syndrome.Material and Methods. Forty-three children and adolescents with Down syndrome were screened for IgAantigliadin antibodies (AGA) and IgA-antiendomysium antibodies (EMA). Patients found to be either AGA-or EMA-positive were investigated further with intestinal biopsy.Results. None of the 43 patients had known celiac disease at entry into the study; 37% (16/43) were found to have AGA levels above normal, and 16% (7/43) to be EMA-positive. Of the 15 patients who underwent biopsy, 8 manifested villous atrophy. Villous atrophy was present in all 7 of the EMA-positive patients, whereas the villi were normal in 7 of the 13 AGA-positive patients who underwent biopsy.Conclusions. EMA is a good immunologic marker for use in screening for celiac disease, and screening is justified in patients with Down syndrome. Pediatrics 1998; 101:272-275; Down syndrome, celiac disease, IgA-antigliadin antibodies, IgA-antiendomysium antibodies.