CELIAC-DISEASE AND TURNER-SYNDROME

Background: Short stature is one of the features of Turner syndrome an d a form of presentation of monosymptomatic celiac disease. Methods: T he recognition of celiac disease in two antiendomysium antibody-positi ve Turner syndrome girls who did not respond to growth hormone treatme nt led us to perform as a screening for celiac disease IgA and IgG ant igliadin antibodies and antiendomysium antibodies determination in oth er 35 Turner syndrome patients. Intestinal biopsy was proposed to the antiendomysium antibodies-positive girls; in the former, subtotal vill ous atrophy was found; in the latter, one parent's consent for intesti nal biopsy was not obtained. Results: The prevalence of celiac disease in Turner syndrome patients observed in the present study (8.1 if we consider 3 villous atrophy, 10.8 if we consider 4 antiendomysium antib ody-positive) is quite high and seems to indicate that the association of these two disorders could not be coincidental. As to the clinical picture, celiac disease appeared atypical in one case, typical in anot her one and as a silent form in the third case. Of the 3 cases with vi llous atrophy on gluten-free diet growth hormone therapy was not effec tive in two girls, who were older than 16 years, while in the younger patient, detected by the screening, a significant increment of height velocity and height Standard Deviation Score for Chronological Age acc ording to Turner references was observed. Conclusions: This study sugg ests that celiac disease can be associated with Turner syndrome and ev en responsible for a failure of growth hormone therapy. Therefore we p ropose to perform in Turner syndrome patients antiendomysium antibody determination as a screening followed by intestinal biopsy in positive cases. This would be advisable at least before starting growth hormon e treatment.