Incidence of Radiologically Confirmed Fractures in Adults With Duchenne Muscular Dystrophy.

IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY
Muscle & Nerve Pub Date : 2025-04-01 Epub Date: 2025-01-23 DOI:10.1002/mus.28355
Gavin Langlands, Jennifer McKechnie, Maria E Farrugia, Sze Choong Wong
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引用次数: 0

Abstract

Introduction/aims: An increased risk of low trauma fractures is well documented in children and adolescents with duchenne muscular dystrophy (DMD). There is limited evidence regarding the fracture incidence of adults with DMD. The aim of this study was to examine radiologically confirmed fractures in adults with DMD and review bone health monitoring.

Methods: This was a retrospective review of all adult males ≥ 16 years with DMD under the care of adult physicians in the West of Scotland (2013-2022). Information regarding fractures, bone health monitoring, and bisphosphonate therapy was collected.

Results: Thirty-six men (median age at first visit 18.8 years) with DMD were included. Twelve were taking corticosteroids at first review, and a further 12 had previously been taking corticosteroids that were discontinued in childhood or adolescence. The fracture incidence rate was higher in the corticosteroid group (888.9 per 10,000 person years (95% CI 242.2-2275.9)) than in those not on corticosteroids (156.3 per 10,000 person years (95% CI 32.2-456.6)). Eighteen had lateral spine radiographs for vertebral fracture assessment and 15 had a DXA scan for bone density assessment during the follow-up period.

Discussion: The fracture incidence in adult men with DMD is more than double that of UK men aged 18-49 years old, with an even higher incidence in those treated with corticosteroids. Fewer than half of the study population underwent bone monitoring. There is a need for enhanced clinical guidance for the monitoring and management of osteoporosis during transition and throughout adulthood.

成人杜氏肌营养不良患者影像学证实骨折的发生率。
简介/目的:杜氏肌营养不良症(DMD)儿童和青少年低创伤性骨折的风险增加是有充分证据的。关于成人DMD患者骨折发生率的证据有限。本研究的目的是检查影像学证实的成人DMD骨折,并回顾骨健康监测。方法:回顾性分析2013-2022年苏格兰西部成人医生治疗的所有≥16岁DMD成年男性患者。收集有关骨折、骨健康监测和双膦酸盐治疗的信息。结果:纳入了36名患有DMD的男性(首次就诊时中位年龄18.8岁)。其中12人在第一次检查时正在服用皮质类固醇,另外12人在儿童或青少年时期停用皮质类固醇。皮质类固醇组的骨折发生率(888.9 / 10000人年(95% CI 242.2-2275.9))高于未使用皮质类固醇组(156.3 / 10000人年(95% CI 32.2-456.6))。在随访期间,18名患者进行了侧位脊柱x线片检查以评估椎体骨折,15名患者进行了DXA扫描以评估骨密度。讨论:成年DMD男性的骨折发生率是英国18-49岁男性的两倍多,接受皮质类固醇治疗的发生率更高。不到一半的研究对象接受了骨监测。有必要加强临床指导监测和管理骨质疏松症在过渡期和整个成年期。
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来源期刊
Muscle & Nerve
Muscle & Nerve 医学-临床神经学
CiteScore
6.40
自引率
5.90%
发文量
287
审稿时长
3-6 weeks
期刊介绍: Muscle & Nerve is an international and interdisciplinary publication of original contributions, in both health and disease, concerning studies of the muscle, the neuromuscular junction, the peripheral motor, sensory and autonomic neurons, and the central nervous system where the behavior of the peripheral nervous system is clarified. Appearing monthly, Muscle & Nerve publishes clinical studies and clinically relevant research reports in the fields of anatomy, biochemistry, cell biology, electrophysiology and electrodiagnosis, epidemiology, genetics, immunology, pathology, pharmacology, physiology, toxicology, and virology. The Journal welcomes articles and reports on basic clinical electrophysiology and electrodiagnosis. We expedite some papers dealing with timely topics to keep up with the fast-moving pace of science, based on the referees'' recommendation.
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