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A population‐based study of scoliosis among males diagnosed with a dystrophinopathy identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network ( MD STARnet )

Year: 2021
Type: article
Study design: Observational Study
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.
DOI: 10.1002/mus.27464
Authors: Kristin M. Conway, Amber Gedlinske, Katherine D. Mathews, Seth Perlman, Nicholas Johnson, Russell Butterfield, Man Hung, Jerry Bounsanga, Dennis Matthews, Joyce Oleszek, Paul A. Romitti
Institutions: University of Iowa, University of Iowa Health Care, Seattle Children's Hospital, Virginia Commonwealth University, Virginia Commonwealth University Medical Center, University of Utah, Roseman University of Health Sciences, University of Colorado Denver
Language: English
Subfield: Surgery
Field: Medicine
Open Access status: green
Awards: G1146282228, G1253469070, Muscular Dystrophy Cooperative Research Center, G2257766211, SUREILLANCE AND EPIDEMIOLOGIC RESEARCH OF DUCHENNE AND BECKER MUSCULAR DYSTROPHY, SURVEILLANCE AND EPIDEMIOLOGIC RESEARCH OF DUCHENNE AND BECKER MUSCULAR DYSTROPHY, Development and validation of clinical outcome assessments in LGMD2A, COMPONENT A: IOWA MD STARNET CORE SITE, ARIZONA MUSCULAR DYSTROPHY SURVEILLANCE AND RESEARCH PROGRAM, NETWORK FOR OBSERVATIONAL STUDY OF VIRGINIA MUSCULAR DYSTROPHIES (NOVA MD), G663124584, COMPONENT C: IOWA MD STARNET SITE DATA DISSEMINATION, G7107615270, SURVEILLANCE AND EPIDEMIOLOGIC RESEARCH OF DUCHENNE AND BECKER MUSCULAR DYSTROPHY, The University of Iowa's NeuroNEXT Clinical Research Site, G8099768572, Identifying the RNA Splicing and Gene Expression Changes that Cause Congenital Myotonic Dystrophy (Renewal)
OpenAlex ID: W3214002282
FWCI: 0.94
Cites: 46
Cited by: 8
Related to: 10