Bone mineralization in children with Wilson's disease.

Abstract

The goal of this study was to determine bone mineralization in children with Wilson's disease (WD). Twenty-seven patients (16 males) and two age- and gender-matched healthy children for each patient were enrolled in the study. Bone mineral content (BMC, grams) and density (BMD, g/cm(2)) at lumbar 1-4 vertebrae were measured by dual-energy X-ray absorptiometry. Urinary calcium excretion was calculated in 19 patients. The effect of cirrhosis and hypercalciuria on BMC and BMD was also evaluated in WD patients. There was no statistically significant difference between patients and healthy controls regarding mean BMC (33.0 ± 13.9 vs. 35.8 ± 13.8 g) (p = 0.940) and mean BMD values (0.66 ± 0.16 vs. 0.71 ± 0.18 g/cm(2)) (p = 0.269), respectively. Nine (47.4 %) patients had hypercalciuria. Hypercalciuric patients had statistically significant lower BMC and BMD values than those without hypercalciuria. A significant difference continued to be present after age, weight, height, and pubertal stage adjustment was done, but disappeared after weight, height, follow up duration, and pubertal stage adjustment was done. The presence of cirrhosis did not affect BMC and BMD significantly in WD patients. BMC and BMD in children with WD were normal. The presence of hypercalciuria but not cirrhosis may affect BMC and BMD negatively in the patients.