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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, Halley M, M.D.
http://orcid.org/0000-0002-8309-6321
http://rave.ohiolink.edu/etdc/view?acc_num=ucin1458299334

Abstract Details

2016, MS, University of Cincinnati, Medicine: Clinical and Translational Research.
Purpose: Fractures and poor bone health due to limited ambulation are significant concerns for patients with Spinal Muscular Atrophy (SMA). However, the prevalence of fractures, low areal bone mineral density (aBMD; Z-score ≤ -2.0) of the lateral distal femur (common fracture location in non-ambulatory children) and of osteoporosis by SMA subtype is not known. Methods: We reviewed data from SMA patients ages 12 months to 25 years, seen at a single institution between January 2005 and January 2015. 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. Results: Median age at initial SMA visit was 1.8 years, but differed by SMA subtype. DXA data were available on 69% of the sample: of these, 90% had a BMD Z-score ≤-2.0 SD at time of first DXA. aBMD Z-scores at all sites was lower with worsening SMA severity, decreasing over time at the lateral distal femur. Fractures occurred in 36% of patients with the femur being the most common location (25 of 53 total fractures). Median age at first fracture was significantly younger with worsening SMA severity. 13% of patients had multiple fractures. Only 8.5% of patients fulfilled criteria for osteoporosis. Conclusion: Low BMD is highly prevalent in SMA patients at the time of first DXA. Fracture frequency is also high with a predominance of femur fractures in all subtypes. However, few patients met ISCD diagnostic criteria for osteoporosis. Our data suggests poor bone health is a significant concern for SMA patients, but may be under-recognized using the 2013 International Society for Clinical Densitometry criteria for diagnosis of osteoporosis in children.
Erin Nicole Haynes, Dr.P.H. (Committee Chair)
Philippe F. Backeljauw, M.D. (Committee Member)
Heidi J. Kalkwarf, Ph.D. (Committee Member)
Jane Khoury, M.S. Ph.D. (Committee Member)
26 p.
Surgery
Osteoporosis; Children; spinal muscular atrophy; Fractures

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Citations

  • Wasserman, M.D., H. M. (2016). Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity [Master's thesis, University of Cincinnati]. OhioLINK Electronic Theses and Dissertations Center. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1458299334

    APA Style (7th edition)

  • Wasserman, M.D., Halley. Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity. 2016. University of Cincinnati, Master's thesis. OhioLINK Electronic Theses and Dissertations Center, http://rave.ohiolink.edu/etdc/view?acc_num=ucin1458299334.

    MLA Style (8th edition)

  • Wasserman, M.D., Halley. "Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity." Master's thesis, University of Cincinnati, 2016. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1458299334

    Chicago Manual of Style (17th edition)

ucin1458299334
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This open access ETD is published by University of Cincinnati and OhioLINK.