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Background We aimed to investigate the frequency of celiac disease in children with β-thalassemia major (B-TM) in Shiraz, southern Iran. Materials and Methods In this study, the prevalence of celiac disease in children with B-TM was evaluated. Children with BTM were screened for celiac disease by ant-tissue transglutaminase (anti-tTG) IgA antibody, IgA level and anti-tTG IgG. A total of 1500 school healthy children in Shiraz with age/sex matched were selected as control group. Results A total of 215 B-TM patients with mean age of 12.7 ± 4.4 years, were included into the study (52.1% was male). None of the patients were positive for anti-tTG IgA. Eight cases were IgA deficient in whom antitTG IgG was investigated but none of them were positive for anti-tTG IgG. The finding in control group has a seroprevalence of 2% and biopsy proven disease of 0.6%. Conclusion Many patients with thalassemia major have multiple non specific symptom that are not justifiable with underlying disease and might be due to atypical celiac disease. We didn't find any case of celiac disease among more than 200 children with β- thalassemia major in Shiraz, southern Iran. So it seems reasonable to screen only those who have features, even not classical, of celiac disease.

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