Dataset Information

Supported Standing in Boys With Duchenne Muscular Dystrophy.

ABSTRACT: To evaluate the safety, tolerability, and efficacy of supported standing in a small sample of boys with Duchenne muscular dystrophy (DMD).Four 12- to 15-year-old boys with DMD engaged in a home-based supported standing program for 6 to 12 months. A single-subject design was employed to examine muscle length. Bone mineral density was assessed at 4-month intervals using dual-energy x-ray absorptiometry.Upright, sustained supported standing was tolerated in 3 of the 4 boys. Mean weekly stand times ranged from 1.3 to 3.3 hours. Improved hip or knee flexor muscle length was seen in 3 of the 4 boys. No boys showed improved plantar flexor muscle length or increased lumbar bone mineral density.Findings offer preliminary empirical evidence addressing the safety, tolerability, and efficacy of standing in boys with DMD. Additional research with an emphasis on better program adherence is indicated.

SUBMITTER: Townsend EL

PROVIDER: S-EPMC5603175 | biostudies-other | 2016 Fall

REPOSITORIES: biostudies-other

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Supported Standing in Boys With Duchenne Muscular Dystrophy.

Townsend Elise L EL Bibeau Christine C Holmes Tara M TM

Pediatric physical therapy : the official publication of the Section on Pediatrics of the American Physical Therapy Association 20160901 3

<h4>Purpose</h4>To evaluate the safety, tolerability, and efficacy of supported standing in a small sample of boys with Duchenne muscular dystrophy (DMD).<h4>Methods</h4>Four 12- to 15-year-old boys with DMD engaged in a home-based supported standing program for 6 to 12 months. A single-subject design was employed to examine muscle length. Bone mineral density was assessed at 4-month intervals using dual-energy x-ray absorptiometry.<h4>Results</h4>Upright, sustained supported standing was tolera ...[more]

PMID: 27008581

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Project description:BackgroundClinical management of boys with Duchenne muscular dystrophy (DMD) relies on in-depth understanding of cardiac involvement, but right ventricular (RV) structural and functional remodeling remains understudied.PurposeTo evaluate several analysis methods and identify the most reliable one to measure RV pre- and postcontrast T1 (RV-T1) and to characterize myocardial remodeling in the RV of boys with DMD.Study typeProspective.PopulationBoys with DMD (N = 27) and age-/sex-matched healthy controls (N = 17) from two sites.Field strength/sequence3.0 T using balanced steady state free precession, motion-corrected phase sensitive inversion recovery and modified Look-Locker inversion recovery sequences.AssessmentBiventricular mass (Mi), end-diastolic volume (EDVi) and ejection fraction (EF) assessment, tricuspid annular excursion (TAE), late gadolinium enhancement (LGE), pre- and postcontrast myocardial T1 maps. The RV-T1 reliability was assessed by three observers in four different RV regions of interest (ROI) using intraclass correlation (ICC).Statistical testsThe Wilcoxon rank sum test was used to compare RV-T1 differences between DMD boys with negative LGE(-) or positive LGE(+) and healthy controls. Additionally, correlation of precontrast RV-T1 with functional measures was performed. A P-value <0.05 was considered statistically significant.ResultsA 1-pixel thick RV circumferential ROI proved most reliable (ICC > 0.91) for assessing RV-T1. Precontrast RV-T1 was significantly higher in boys with DMD compared to controls. Both LGE(-) and LGE(+) boys had significantly elevated precontrast RV-T1 compared to controls (1543 [1489-1597] msec and 1550 [1402-1699] msec vs. 1436 [1399-1473] msec, respectively). Compared to healthy controls, boys with DMD had preserved RVEF (51.8 [9.9]% vs. 54.2 [7.2]%, P = 0.31) and significantly reduced RVMi (29.8 [9.7] g vs. 48.0 [15.7] g), RVEDVi (69.8 [29.7] mL/m2 vs. 89.1 [21.9] mL/m2 ), and TAE (22.0 [3.2] cm vs. 26.0 [4.7] cm). Significant correlations were found between precontrast RV-T1 and RVEF (β = -0.48%/msec) and between LV-T1 and LVEF (β = -0.51%/msec).Data conclusionPrecontrast RV-T1 is elevated in boys with DMD compared to healthy controls and is negatively correlated with RVEF.Level of evidence1 TECHNICAL EFFICACY: Stage 2.

Dataset Information

Supported Standing in Boys With Duchenne Muscular Dystrophy.

Publications

Supported Standing in Boys With Duchenne Muscular Dystrophy.

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