Journal article
A Population‐based Study of Scoliosis among Males Diagnosed with a Dystrophinopathy Identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network ( MD STAR net )
Muscle & nerve, Vol.65(2), pp.193-202
11/17/2021
DOI: 10.1002/mus.27464
PMCID: PMC8752499
PMID: 34787322
Abstract
Introduction/Aims
Scoliosis is a common comorbidity among individuals diagnosed with a dystrophinopathy. We examined associations between clinical predictors and scoliosis in childhood-onset dystrophinopathy.
Methods
The progression and treatment of scoliosis were obtained from data collected by the US population-based Muscular Dystrophy Surveillance, Tracking, and Research Network. Associations between loss of independent ambulation (LoA) and corticosteroid use and scoliosis outcomes (ages at or exceeding Cobb angle thresholds [10°, 20°, 30°]; surgery) were estimated using Kaplan–Meier curve estimation and extended Cox regression modeling.
Results
We analyzed curvature data for 513 of 1054 individuals ascertained. Overall, approximately one-half had at least one radiograph and one-quarter had a curvature of at least 20°. The average maximum curvature was 25.0° (SD = 21.5°) among all individuals and 42.8° (SD = 18.8°) among those recommended for surgery. Higher adjusted hazards ratio of curvature (aHR(curvature) [95% confidence interval]) were found among individuals with LoA compared to those without LoA (aHR(10) = 6.2 [4.4, 8.7], aHR(20) = 15.3 [7.4, 31.7], aHR(30) = 31.6 [7.7, 128.9]), among individuals who did not use corticosteroids compared to those who did (aHR(10) = 1.2 [0.9, 1.7], aHR(20) = 1.8 [1.1, 2.7], aHR(30) = 2.3 [1.3, 4.0]), and among non-ambulatory individuals who used corticosteroids after LoA compared to those who did not (aHR(10) = 1.8 [1.2, 2.8], aHR(20) = 1.6 [1.0, 2.6], aHR(30) = 3.6 [1.6, 7.9]). Scoliosis surgery among individuals with LoA who did not use corticosteroids was more than double compared to those who used (aHR = 2.3 [1.3, 4.2]).
Discussion
Our retrospective observational study suggests corticosteroids may delay spinal curvature progression and need for scoliosis surgery. Continuing corticosteroids after LoA also showed potential benefits of delaying curvature progression, additional studies are needed to confirm this finding or address the magnitude of benefit.
Details
- Title: Subtitle
- A Population‐based Study of Scoliosis among Males Diagnosed with a Dystrophinopathy Identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network ( MD STAR net )
- Creators
- Kristin M Conway - University of IowaAmber Gedlinske - University of IowaKatherine D Mathews - University of IowaSeth Perlman - Seattle Children's HospitalNicholas Johnson - Virginia Commonwealth UniversityRussell Butterfield - University of UtahMan Hung - Roseman University of Health SciencesJerry BounsangaDennis Matthews - Department of Physical Medicine and Rehabilitation University of Colorado and Children's Hospital Denver COJoyce Oleszek - Department of Physical Medicine and Rehabilitation University of Colorado and Children's Hospital Denver COPaul A Romitti - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Muscle & nerve, Vol.65(2), pp.193-202
- DOI
- 10.1002/mus.27464
- PMID
- 34787322
- PMCID
- PMC8752499
- NLM abbreviation
- Muscle Nerve
- ISSN
- 0148-639X
- eISSN
- 1097-4598
- Grant note
- DOI: 10.13039/100000030, name: Centers for Disease Control and Prevention, award: DD000192, DD000191, DD000190, DD000189, DD000187
- Language
- English
- Date published
- 11/17/2021
- Academic Unit
- Neurology; Stead Family Department of Pediatrics; Epidemiology; Iowa Neuroscience Institute; Biostatistics; Neurology (Pediatrics); Internal Medicine
- Record Identifier
- 9984198054402771
Metrics
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