Bone mineral density of children with Wilson disease - Efficacy of penicillamine and zinc therapy

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dc.authorwosidDoneray, Hakan/AAD-8797-2020
dc.contributor.authorSelimoglu, Mukadder Ayse
dc.contributor.authorErtekin, Vildan
dc.contributor.authorDoneray, Hakan
dc.contributor.authorYidirim, Mustafa
dc.date.accessioned2024-08-04T20:30:39Z
dc.date.available2024-08-04T20:30:39Z
dc.date.issued2008
dc.departmentİnönü Üniversitesien_US
dc.description.abstractObjectives: 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.en_US
dc.identifier.doi10.1097/MCG.0b013e318032388d
dc.identifier.endpage198en_US
dc.identifier.issn0192-0790
dc.identifier.issn1539-2031
dc.identifier.issue2en_US
dc.identifier.pmid18209591en_US
dc.identifier.scopus2-s2.0-38349115412en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage194en_US
dc.identifier.urihttps://doi.org/10.1097/MCG.0b013e318032388d
dc.identifier.urihttps://hdl.handle.net/11616/94432
dc.identifier.volume42en_US
dc.identifier.wosWOS:000252862400015en_US
dc.identifier.wosqualityQ2en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherLippincott Williams & Wilkinsen_US
dc.relation.ispartofJournal of Clinical Gastroenterologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectWilson diseaseen_US
dc.subjectosteoporosisen_US
dc.subjectosteopeniaen_US
dc.subjectchildrenen_US
dc.titleBone mineral density of children with Wilson disease - Efficacy of penicillamine and zinc therapyen_US
dc.typeArticleen_US

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