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      Pamidronate treatment improves bone mineral density in children with Menkes disease.

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          Abstract

          Menkes disease is a severe multisystem disorder due to defective bioavailability and transport of copper at the cellular level. Deficient activity of lysyl oxidase, a copper-dependent enzyme, causes defective collagen cross-linking leading to osteoporosis and pathological fractures in these children. The objective of the study was to evaluate the changes in bone mineral density following pamidronate treatment in children with Menkes disease. The study design was an open observational study of three children with Menkes disease and significant osteoporosis with or without pathological fractures, all of whom received pamidronate treatment for 1 year. There were 34-55% and 16-36% increases in lumbar spine bone mineral content and areal bone mineral density, respectively, following 1 year of treatment with pamidronate. There were no further fractures in two of the three children treated. No adverse effects of pamidronate treatment were noted. Pamidronate treatment was associated with an increase in bone mineral density and may be an effective treatment modality for the management of osteoporosis in children with Menkes disease.

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          Author and article information

          Journal
          J Inherit Metab Dis
          Journal of inherited metabolic disease
          Springer Science and Business Media LLC
          0141-8955
          0141-8955
          Sep 2002
          : 25
          : 5
          Affiliations
          [1 ] Department of Endocrinology and Diabetes, Genetic Health Services Victoria, Royal Children's Hospital, Parkville, Australia.
          Article
          10.1023/a:1020103901969
          12408189
          a1e29d8d-9f1b-4cb0-8b9c-723ca4b67c21
          History

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