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Original Article |
Departments of Pediatrics (M.B., A.M.P., P.M., H.M.D.) and Experimental Medicine and Pathology (F.C.), University of Rome "La Sapienza", 00161 Roma; Department of Pediatrics (L.M.), University of Bologna, 40100 Bologna; and Department of Pediatrics (A.P., G.B.), University of Eastern Piedmont Novara, 28100 Novara, Italy
Address all correspondence and requests for reprints to: Prof. Margherita Bonamico, Istituto di Clinica Pediatrica, Policlinico "Umberto I", Viale Regina Elena 324, 00161 Rome, Italy. E-mail: margherita.bonamico{at}uniroma1.it.
Abstract
A multicenter study of Turner syndrome (TS) patients was carried out to estimate the prevalence of celiac disease (CD) and to detect clinical characteristics and laboratory data of affected patients. Three hundred eighty-nine girls with TS were screened by IgA antigliadin antibodies and/or antiendomysial antibodies. Intestinal biopsy was offered to positive cases. CD was diagnosed in 25 patients. In celiac subjects, anemia, anorexia, and delayed growth (with respect to Italian TS curves) were frequently present; whereas distended abdomen, chronic diarrhea, constipation, and vomiting occurred more rarely. In addition, low serum iron levels, hemoglobinemia, and high values of aminotransferases were observed. Ten patients showed classic CD, 8 showed atypical symptoms, and 7 showed a silent CD. In 11 symptomatic patients, the diagnosis of CD was made at the onset of symptoms, whereas 7 of them showed a median delay of 79 months in diagnosis. Other autoimmune disorders were observed in 40% of the patients. Our study confirms the high prevalence (6.4%) of CD in a large series of TS patients. Moreover, the subclinical picture in 60% of the cases, the diagnostic delay, and the incidence of other autoimmune disorders suggest that routine screening of CD in TS is indicated.
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