Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity

Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity

Bibliographic Details
Main Author: Wasserman, Halley M., M.D.
Language:English
Published: University of Cincinnati / OhioLINK 2016
Subjects:
Surgery
Osteoporosis
Children
spinal muscular atrophy
Fractures
Online Access:http://rave.ohiolink.edu/etdc/view?acc_num=ucin1458299334
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spelling ndltd-OhioLink-oai-etd.ohiolink.edu-ucin14582993342021-08-03T06:35:04Z 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. Surgery Osteoporosis Children spinal muscular atrophy Fractures 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. 2016-06-28 English text University of Cincinnati / OhioLINK http://rave.ohiolink.edu/etdc/view?acc_num=ucin1458299334 http://rave.ohiolink.edu/etdc/view?acc_num=ucin1458299334 unrestricted This thesis or dissertation is protected by copyright: all rights reserved. It may not be copied or redistributed beyond the terms of applicable copyright laws.
collection NDLTD
language English
sources NDLTD
topic Surgery
Osteoporosis
Children
spinal muscular atrophy
Fractures
spellingShingle Surgery
Osteoporosis
Children
spinal muscular atrophy
Fractures
Wasserman, Halley M., M.D.
Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity
author Wasserman, Halley M., M.D.
author_facet Wasserman, Halley M., M.D.
author_sort Wasserman, Halley M., M.D.
title Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity
title_short Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity
title_full Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity
title_fullStr Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity
title_full_unstemmed Low bone mineral density and fractures are highly prevalent in pediatric patients with Spinal Muscular Atrophy regardless of disease severity
title_sort low bone mineral density and fractures are highly prevalent in pediatric patients with spinal muscular atrophy regardless of disease severity
publisher University of Cincinnati / OhioLINK
publishDate 2016
url http://rave.ohiolink.edu/etdc/view?acc_num=ucin1458299334
work_keys_str_mv AT wassermanhalleymmd lowbonemineraldensityandfracturesarehighlyprevalentinpediatricpatientswithspinalmuscularatrophyregardlessofdiseaseseverity
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