Wilson's Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Wilson's Disease, including details on treatment, causes, symptoms, medication.

Bone mineral density of children with Wilson disease: efficacy of penicillamine and zinc therapy.

Selimoglu MA, Ertekin V, Doneray H, Yildirim M

Faculty of Medicine, Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Inonu University, Malatya. ayseselimoglu@hotmail.com

OBJECTIVES: Osteoporosis accompanying chronic liver disease is well known; however, the exact prevalence is unknown. No data on bone mineral density (BMD) of children with Wilson disease (WD) have been published so far. In this study, we aimed to investigate the prevalence of osteoporosis in childhood WD and to observe the probable positive effects of penicillamine and zinc therapy on osteoporosis. METHODS: Thirty-one children with newly diagnosed WD and sex and age-matched 16 healthy children were included. Mean age was 9.0+/-3.2 years (2 to 16 y). Bone mineral content (BMC) and BMD were measured on admission and in 13 cases they were reassessed after 1 year of treatment with penicillamine and zinc. RESULTS: Mean BMD, BMC, and Z scores of the patients were significantly lower than those of healthy children: 0.52+/-0.09 versus 0.72+/-0.09 (P=0.001), 19.27+/-13.01 versus 29.67+/-14.23 (P=0.009), and -2.33+/-1.28 versus -0.12+/-0.31 (P=0.001), respectively. The prevalence of osteopenia and osteoporosis in children with WD was found as 22.6% and 67.7%, respectively. BMD and BMC levels were higher in children with neurologic involvement. The severity of the disease had no effect on the mentioned parameters. One year under treatment with penicillamine and zinc did not significantly alter the mentioned parameters. CONCLUSIONS: In this first study investigating the prevalence of osteoporosis in children with WD, we found an extremely high prevalence. Because of nonbeneficial effect of routine treatment of WD on osteoporosis, we emphasize the necessity of screening of bone mineralization and additional therapeutic approach for those children.

Published 22 January 2008 in J Clin Gastroenterol, 42(2): 194-8.
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