Fracture in Duchenne Muscular Dystrophy

Author:

Perera Nadia1,Sampaio Hugo2,Woodhead Helen13,Farrar Michelle12

Affiliation:

1. Discipline of Paediatrics, School of Women’s and Children’s Health, UNSW Medicine, University of New South Wales, Sydney, Australia

2. Department of Neurology, Sydney Children’s Hospital, Randwick, Australia

3. Department of Endocrinology, Sydney Children’s Hospital, Randwick, Australia

Abstract

The present study examined the natural history of fracture and vitamin D levels in Duchenne muscular dystrophy patients, who are vulnerable to osteoporosis and fractures. Retrospective analysis of a cohort of 48 Duchenne muscular dystrophy patients revealed that 43% of patients experienced ≥1 fracture. Fracture probabilities at ages 6, 9, 12, and 15 years were 4%, 9%, 31%, and 60% respectively, accelerating around the time of ambulation loss (mean age 11.8 ± 2.7 years). Chronic corticosteroid therapy was utilized in 69% of patients and was associated with all vertebral fractures. A history of vitamin D deficiency occurred in 84%, and 35% were currently deficient. Despite chronic vitamin D supplementation, 38% remained deficient. These results demonstrate that osteoporosis and fracture remain major concerns in Duchenne muscular dystrophy. Bone health should be optimized well before loss of ambulation, however current levels of vitamin D supplementation may be inadequate given high levels of deficiency.

Publisher

SAGE Publications

Subject

Neurology (clinical),Pediatrics, Perinatology and Child Health

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