Fracturas en la atrofia muscular espinal

Febrer, Anna; Vigo, M.; Rodrı́guez, Natalia; Medina, J.; Colomer, Jaume; Nascimento, A.

doi:10.33588/rn.5705.2013174

Cited by 4 publications

(1 citation statement)

References 20 publications

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“…Children with SMA have a high incidence of fragility fractures. But only 5% (2/40) of children with SMA in our study had fractures, which was significantly lower than the fracture rate of 36-46% reported by other studies [22,30,31]. The low rate of fractures in our study patients may be related to their decreased participation in outdoor activities and rehabilitation.…”

Section: Discussioncontrasting

confidence: 76%

Bone mineral density and its influencing factors in Chinese children with spinal muscular atrophy types 2 and 3

Peng

et al. 2021

BMC Musculoskelet Disord

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Background Patients with spinal muscular atrophy (SMA) are at risk of decreased bone mineral density (BMD). The bone health status of Chinese patients with SMA has been poorly studied. We aimed to evaluate the BMD of children with SMA types 2 and 3 in mainland China and investigate its influencing factors. Methods Forty patients with a mean age of 5.5 years affected by SMA types 2 and 3 (n = 22 and n = 18, respectively) were enrolled between September 2017 and May 2019. Total body less head (TBLH) BMD, lumbar spine (LS) BMD, and body composition were measured using dual-energy X-ray absorptiometry (DXA). Serum bone metabolism markers and complete spinal radiographs were assessed. We utilized a linear regression model to explore the correlations between BMD and its related factors. Results A total of 67.5% (27/40) of patients were diagnosed with low BMD and 2.5% (1/40) were diagnosed with osteoporosis. The TBLH BMD and LS BMD Z-scores in children with SMA type 2 were significantly lower than those with SMA type 3. Both TBLH and LS BMD Z-scores tended to increase with the change of SMA subtypes from 2a-3b. Vitamin D insufficiency and deficiency were found in 37.5% (15/40) of the patients. Serum Ca, phosphorus (P), alkaline phosphatase (ALP) and parathormone (PTH) levels were normal. There were no significant differences among the four subtypes in terms of all the serum bone metabolism markers. Phenotype was significantly associated with TBLH BMD and LS BMD Z-scores, and serum PTH levels were significantly associated with TBLH BMD Z-scores. Conclusions Low BMD and osteoporosis were highly prevalent in mainland Chinese children with SMA types 2 and 3. Phenotype and serum PTH level might be the influencing factors of BMD. Regular monitoring of BMD by DXA scan and taking active interventions aim to SMA children with different types are important.

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Section: Discussioncontrasting

confidence: 76%

Bone mineral density and its influencing factors in Chinese children with spinal muscular atrophy types 2 and 3

Peng

et al. 2021

BMC Musculoskelet Disord

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Enfermedades neuromusculares

Febrer¹

2015

Rehabilitación Domiciliaria

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Low bone mineral density and fractures are highly prevalent in pediatric patients with spinal muscular atrophy regardless of disease severity

Wasserman

Hornung

Stenger

et al. 2017

Neuromuscular Disorders

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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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Fracturas en la atrofia muscular espinal

Cited by 4 publications

References 20 publications

Bone mineral density and its influencing factors in Chinese children with spinal muscular atrophy types 2 and 3

Bone mineral density and its influencing factors in Chinese children with spinal muscular atrophy types 2 and 3

Enfermedades neuromusculares

Low bone mineral density and fractures are highly prevalent in pediatric patients with spinal muscular atrophy regardless of disease severity

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