Generation of percentile curves for strength and functional abilities for boys with Duchenne muscular dystrophy.

Autor: Leon MAS; Department of Health Science, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil., Roza DLD; Department of Epidemiology, School of Public Health, University of São Paulo, São Paulo, Brazil., Davoli GBQ; Department of Health Science, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil., Baptista CRJA; Department of Health Science, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil., Sobreira CFDR; Department of Neurosciences and Behavior, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil., Mattiello-Sverzut AC; Department of Health Science, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil.
Jazyk: angličtina
Zdroj: Muscle & nerve [Muscle Nerve] 2023 Aug; Vol. 68 (2), pp. 198-205. Date of Electronic Publication: 2023 Jun 15.
DOI: 10.1002/mus.27921
Abstrakt: Introduction/aims: Considering the heterogeneity of the clinical manifestations of Duchenne muscular dystrophy (DMD), it is important to describe their various clinical profiles. Thus, in this study we aimed to develop percentile curves for DMD using a battery of measures to define the patterns of functional abilities, timed tests, muscle strength, and range of motion (ROM).
Methods: This retrospective data analysis was based on the records of patients with DMD using the Motor Function Measure (MFM) scale, isometric muscle strength (IS), dorsiflexion ROM, 10-meter walk test (10 MWT), and 6-minute walk test (6 MWT). Percentile curves (25th, 50th, and 75th percentiles) with MFM, IS, ROM, 10 MWT, and 6 MWT on the y axis and patient age on the x axis were constructed using the generalized additive model for location, scale, and shape, with Box-Cox power exponential distribution.
Results: There were records of 329 assessments of patients between 4 and 18 years of age. The MFM percentiles showed a gradual reduction in all dimensions. Muscle strength and ROM percentiles showed that the knee extensors were the most affected from 4 years of age, and dorsiflexion ROM negative values were noted from the age of 8 years. The 10 MWT showed a gradual increase in performance time with age. For the 6 MWT, the distance curve remained stable until 8 years, with a subsequent progressive decline.
Discussion: In this study we generated percentile curves that can help health professionals and caregivers follow the trajectory of disease progression in DMD patients.
(© 2023 Wiley Periodicals LLC.)
Databáze: MEDLINE
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