Optimizing Bone Health in Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder characterized by progressive muscle weakness, with eventual loss of ambulation and premature death. The approved therapy with corticosteroids improves muscle strength, prolongs ambulation, and maintains pulmonary function. However,...

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Main Authors: Jason L. Buckner, Sasigarn A. Bowden, John D. Mahan
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:International Journal of Endocrinology
Online Access:http://dx.doi.org/10.1155/2015/928385
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author Jason L. Buckner
Sasigarn A. Bowden
John D. Mahan
author_facet Jason L. Buckner
Sasigarn A. Bowden
John D. Mahan
author_sort Jason L. Buckner
collection DOAJ
description Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder characterized by progressive muscle weakness, with eventual loss of ambulation and premature death. The approved therapy with corticosteroids improves muscle strength, prolongs ambulation, and maintains pulmonary function. However, the osteoporotic impact of chronic corticosteroid use further impairs the underlying reduced bone mass seen in DMD, leading to increased fragility fractures of long bones and vertebrae. These serious sequelae adversely affect quality of life and can impact survival. The current clinical issues relating to bone health and bone health screening methods in DMD are presented in this review. Diagnostic studies, including biochemical markers of bone turnover and bone mineral density by dual energy X-ray absorptiometry (DXA), as well as spinal imaging using densitometric lateral spinal imaging, and treatment to optimize bone health in patients with DMD are discussed. Treatment with bisphosphonates offers a method to increase bone mass in these children; oral and intravenous bisphosphonates have been used successfully although treatment is typically reserved for children with fractures and/or bone pain with low bone mass by DXA.
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spelling doaj-art-2631f72961b04e1fb8e09e00cfdcb4b02025-02-03T06:05:52ZengWileyInternational Journal of Endocrinology1687-83371687-83452015-01-01201510.1155/2015/928385928385Optimizing Bone Health in Duchenne Muscular DystrophyJason L. Buckner0Sasigarn A. Bowden1John D. Mahan2Division of Endocrinology, Department of Pediatrics, Nationwide Children’s Hospital, The Ohio State University College of Medicine, Columbus, OH 43205, USADivision of Endocrinology, Department of Pediatrics, Nationwide Children’s Hospital, The Ohio State University College of Medicine, Columbus, OH 43205, USADivision of Nephrology, Department of Pediatrics, Nationwide Children’s Hospital, The Ohio State University College of Medicine, Columbus, OH 43205, USADuchenne muscular dystrophy (DMD) is an X-linked recessive disorder characterized by progressive muscle weakness, with eventual loss of ambulation and premature death. The approved therapy with corticosteroids improves muscle strength, prolongs ambulation, and maintains pulmonary function. However, the osteoporotic impact of chronic corticosteroid use further impairs the underlying reduced bone mass seen in DMD, leading to increased fragility fractures of long bones and vertebrae. These serious sequelae adversely affect quality of life and can impact survival. The current clinical issues relating to bone health and bone health screening methods in DMD are presented in this review. Diagnostic studies, including biochemical markers of bone turnover and bone mineral density by dual energy X-ray absorptiometry (DXA), as well as spinal imaging using densitometric lateral spinal imaging, and treatment to optimize bone health in patients with DMD are discussed. Treatment with bisphosphonates offers a method to increase bone mass in these children; oral and intravenous bisphosphonates have been used successfully although treatment is typically reserved for children with fractures and/or bone pain with low bone mass by DXA.http://dx.doi.org/10.1155/2015/928385
spellingShingle Jason L. Buckner
Sasigarn A. Bowden
John D. Mahan
Optimizing Bone Health in Duchenne Muscular Dystrophy
International Journal of Endocrinology
title Optimizing Bone Health in Duchenne Muscular Dystrophy
title_full Optimizing Bone Health in Duchenne Muscular Dystrophy
title_fullStr Optimizing Bone Health in Duchenne Muscular Dystrophy
title_full_unstemmed Optimizing Bone Health in Duchenne Muscular Dystrophy
title_short Optimizing Bone Health in Duchenne Muscular Dystrophy
title_sort optimizing bone health in duchenne muscular dystrophy
url http://dx.doi.org/10.1155/2015/928385
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