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

Authors: Wasserman, Halley M; Hornung, Lindsey N; Stenger, Peggy J; Rutter, Meilan M; Wong, Brenda L; Rybalsky, Irina; Khoury, Jane C; Kalkwarf, Heidi J

Published In Neuromuscul Disord, (2017 Apr)

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.

PubMed ID: 28258940

MeSH Terms: Absorptiometry, Photon; Adolescent; Bone Density/physiology*; Child; Child, Preschool; Female; Fractures, Bone/epidemiology; Fractures, Bone/etiology*; Humans; Infant; Male; Muscular Atrophy, Spinal/complications; Muscular Atrophy, Spinal/epidemiology; Muscular Atrophy, Spinal/physiopathology*; Osteoporosis/diagnostic imaging; Osteoporosis/epidemiology; Osteoporosis/etiology*; Prevalence; Severity of Illness Index