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Low bone mineral density and fractures are extremely prevalent in pediatric sufferers with spinal muscular atrophy no matter illness severity.
Neuromuscul Disord. 2017 Apr;27(four):331-337
Authors: Wasserman HM, Hornung LN, Stenger PJ, Rutter MM, Wong BL, Rybalsky I, Khoury JC, Kalkwarf HJ
Summary
Sufferers with Spinal Muscular Atrophy (SMA) are in danger for poor bone well being. The prevalence of fractures, low areal bone mineral density (aBMD; Z-score ?-2.zero) of the lateral distal femur and of osteoporosis by SMA subtype just isn’t recognized. We aimed to explain the pure historical past of bone well being in sufferers with SMA previous to bisphosphonate therapy. We reviewed knowledge from 85 eligible sufferers with SMA ages 12 months to 18 years, seen at a single establishment between January 2005 and July 2016. Fracture historical past was reported at annual clinic visits. aBMD was obtained from twin vitality x-ray absorptiometry scans of the lumbar backbone, complete physique, and lateral distal femur. 85% of sufferers had aBMD Z-scores ?-2.zero SD and have been progressively decrease with worsening SMA severity. Longitudinal aBMD Z-scores of the lateral distal femur decreased with age. Fractures occurred in 38% (32/85) of sufferers with the femur being the commonest location (25 of 57 fractures). 13 % of sufferers fulfilled standards for osteoporosis. Low aBMD and femur fractures are extremely prevalent in all SMA subtypes from a younger age; nevertheless, few sufferers met the standards for osteoporosis. Poor bone well being could also be an under-recognized comorbidity of SMA.
PMID: 28258940 [PubMed – indexed for MEDLINE]