The Hip in Muscular Dystrophy
The muscular dystrophies (MD) are a group of inherited conditions characterized by a progressive muscle weakness and increasing disability. The most common and well-known is Duchenne muscular dystrophy (DMD). DMD is essentially the only MD associated with hip problems.
The medical management of DMD has changed over recent years, particularly with the use of steroid medication. This has lengthened life but is associated with other problems that adversely affect quality of life.
Advances have been made in the understanding of dystrophin production in DMD and Becker Muscular Dystrophy. Trials of molecular based therapies including gene replacement via a viral vector have taken place although there are substantial challenges to translation into widespread clinical practice.
Compared to other neuromuscular diseases, hip problems are unusual in DMD and there are no accepted guidelines for the management of hip contractures or hip subluxation/dislocation in this patient group.
KeywordsDuchenne muscular dystrophy Becker muscular dystrophy Dystrophin Hip Scoliosis
- 3.Matthews E, Brassington R, Kuntzer T, Jichi F, Mansur AY. Corticosteroids for the treatment of Duchenne muscular dystrophy. Cochrane Database Syst Rev. 2016;5(5):CD003725.Google Scholar
- 16.Buckon C, Sienko S, Bagley A, Sison-Williamson M, Fowler E, Staudt L, Heberer K, McDonald CM, Sussman M. Can quantitative muscle strength and functional motor ability differentiate the influence of age and corticosteroids in ambulatory boys with Duchenne muscular dystrophy? PLoS Curr. 2016;8:8.Google Scholar
- 19.Griggs R.. Finding the optimum regiment for Duchenne muscular dystrophy (FOR DMD). https://clinicaltrials.gove/ct2/show/NCT01603407. Accessed 20 Apr 2018.
- 20.Bell JM, Shields MD, Watters J, Hamilton A, Beringer T, Elliott M, Quinlivan R, Tirupathi S, Blackwood B. Interventions to prevent and treat corticosteroid-induced osteoporosis and prevent osteoporotic fractures in Duchenne muscular dystrophy. Cochrane Database Syst Rev. 2017;24(1):CD010899.Google Scholar
- 22.Buckner JL, Bowden SA, Mahan JD. Optimising bone health in Duchenne muscular dystrophy. Int J Endocinol. 2015;2015:928385.Google Scholar
- 33.Cheuk DK, Wong V, Wriage E, Baxter P, Cole A. Surgery for scoliosis in Duchenne muscular dystrophy. Cochrane Database Syst Rev. 2015;1(10):CD005375.Google Scholar