ArticleNeuromuscular disorders : NMD2017
Low bone mineral density and fractures are highly prevalent in pediatric patients with spinal muscular atrophy regardless of disease severity.
Article in Neuromuscular disorders : NMD, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 83 citations in OpenAlex.
- Bone Fragility and Fracture Characteristics in Patients With Spinal and Bulbar Muscular Atrophy.European journal of neurology · 2025Article
- SMN deficiency inhibits endochondral ossification via promoting TRAF6-induced ubiquitination degradation of YBX1 in spinal muscular atrophy.Bone research · 2025Article
- A comparative analysis of bone mineral density in cerebral palsy and spinal muscular atrophy.Bone reports · 2025Article
- Comorbidities in spinal muscular atrophy and their impact on the course of the underlying disease: a real-life observational study.BMC musculoskeletal disorders · 2025Observational
- Padua Days on Muscle and Mobility Medicine, March 25-29, 2025, Hotel Petrarca, Euganean Thermae, Italy: Program and Abstracts.European journal of translational myology · 2025Article
- Assessment of safety and efficacy of risdiplam treatment in adults with spinal muscular atrophy.Frontiers in neurology · 2025Article
- Low bone mineral density and its influencing factors in spinal muscular atrophy without disease-modifying treatment: a single-centre cross-sectional study.BMC pediatrics · 2024Article
- Rehabilitation Strategies for Patients With Spinal Muscular Atrophy in the Era of Disease-Modifying Therapy.Annals of rehabilitation medicine · 2024Article
- Article
- Evaluation of bone health in terms of osteoporosis in adult patients with neuromuscular disease.Turkish journal of medical sciences · 2024Article
- Multiple Lower Limb Fractures in a Child With Spinal Muscular Atrophy Type 2: A Preventable Complication.Cureus · 2024Article
- Bone Health Status of Children with Spinal Muscular Atrophy.Journal of bone metabolism · 2023Article
- Muscle and bone characteristics of a Chinese family with spinal muscular atrophy, lower extremity predominant 1 (SMALED1) caused by a novel missense DYNC1H1 mutation.BMC medical genomics · 2023Article
- A Scoping Review of the Recent Clinical Practice Regarding the Evaluation of Bone Mineral Density in Children and Adolescents with Neuromuscular Diseases.Medicina (Kaunas, Lithuania) · 2023Article
- Bone Mineral Density Assessment by Quantitative Computed Tomography in Glucocorticoid-Treated Boys With Duchenne Muscular Dystrophy: A Linear Mixed-Effects Modeling Approach.Frontiers in endocrinology · 2022Article
- Hybrid versus total sublaminar wires in patients with spinal muscular atrophy undergoing scoliosis surgery.BMC musculoskeletal disorders · 2021Article
- Osteogenesis Imperfecta: Current and Prospective Therapies.Biomolecules · 2021Review
- Bone mineral density and its influencing factors in Chinese children with spinal muscular atrophy types 2 and 3.BMC musculoskeletal disorders · 2021Article
- Sagittal Plane Deformities in Children with SMA2 following Posterior Spinal Instrumentation.Children (Basel, Switzerland) · 2021Article
- Nutritional, Gastrointestinal and Endo-Metabolic Challenges in the Management of Children with Spinal Muscular Atrophy Type 1.Nutrients · 2021Review
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
Patients with Spinal Muscular Atrophy (SMA) are at risk for poor bone health. The prevalence of fractures, low areal bone mineral density (aBMD; Z-score ≤-2.0) of the lateral distal femur and of osteoporosis by SMA subtype is not known. We aimed to describe the natural history of bone health in patients with SMA prior to bisphosphonate treatment. We reviewed data from 85 eligible patients with SMA ages 12 months to 18 years, seen at a single institution between January 2005 and July 2016. Fracture history was reported at annual clinic visits. aBMD was obtained from dual energy x-ray absorptiometry scans of the lumbar spine, total body, and lateral distal femur. 85% of patients had aBMD Z-scores ≤-2.0 SD and were progressively lower with worsening SMA severity. Longitudinal aBMD Z-scores of the lateral distal femur decreased with age. Fractures occurred in 38% (32/85) of patients with the femur being the most common location (25 of 57 fractures). Thirteen percent of patients fulfilled criteria for osteoporosis. Low aBMD and femur fractures are highly prevalent in all SMA subtypes from a young age; however, few patients met the criteria for osteoporosis. Poor bone health may be an under-recognized comorbidity of SMA.
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