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1.
Neuromuscul Disord ; 27(8): 723-729, 2017 Aug.
Article in English | MEDLINE | ID: mdl-28648683

ABSTRACT

The North Star Ambulatory Assessment measures motor performance in ambulatory boys with Duchenne muscular dystrophy, a hereditary and degenerative muscle disorder. To use the North Star Ambulatory Assessment in Brazilian boys, we performed the cross-cultural adaptation to the Portuguese language spoken in Brazil and evaluated the reliability and validity of the instrument. Cross-cultural adaptation included: independent translations, synthesis, committee review, pre-testing in 12 boys, back-translation and comparison with the original instrument. Thirty-five boys with Duchenne muscular dystrophy and 38 healthy age-matched controls were recruited for further analyses. Reliability was assessed by internal consistency and reproducibility. Validity studies included face, content, construct and known-groups analyses. Cross-cultural adaptation resulted in an adequate instrument. Reliability studies demonstrated high internal consistency (Cronbach's alpha = 0.935) and adequate intra and inter-rater reproducibility (intraclass correlation coefficient = 0.988 and 0.962). Validation analyses indicated satisfactory content, face and convergent construct validities, with positive correlations with the Motor Function Measure total score (r = 0.863) and the 6-minute walk test (r = 0.433). The known group validity was demonstrated by higher scores in younger boys with Duchenne muscular dystrophy (p = 0.005). North Star Ambulatory Assessment in Brazilian Portuguese is a reliable and valid instrument to measure functional capacity in boys with Duchenne muscular dystrophy.


Subject(s)
Motor Activity , Muscular Dystrophy, Duchenne/diagnosis , Adolescent , Brazil , Child , Child, Preschool , Cross-Cultural Comparison , Follow-Up Studies , Humans , Male , Reproducibility of Results , Translating , Walk Test
2.
Article in Portuguese | LILACS | ID: lil-583300

ABSTRACT

Objetivo: O objetivo foi avaliar portadores de distrofia muscular de Duchenne (DMD) e Becker (DMB) por meio de escalas e análise postural. Metodologia: Foram avaliados 13 pacientes, idade 16,75 (± 6,9) sendo 9 DMD, 4 DMB, 7 cadeirantes, 6 não cadeirantes, nas escalas: Índice de Barthel e EK (Egen klassifikation). A avaliação postural sentada foi feita no software SAPO. Resultados: Os dados revelam que: aumento da idade (p<0.01), dependência de cadeira de rodas (p<0.01) e uso de ventilador (p<0.01) indicaram menor independência. Na avaliação postural tanto dos não cadeirantes quanto dos cadeirantes, verificou-se que aumentam os agrupamentos nos cadeirantes, remetendo às limitações impostas pela postura. Conclusão: Esses achados mostram que pacientes com DMD e DMB têm sua funcionalidade e atividades de vida diária debilitadas com o avanço da idade, dependência de cadeira de rodas e uso de ventilador. Assim, associação de escalas com avaliações convencionais e análise postural são ferramentas essenciais para a investigação.


Objective: The objective was to evaluate patients with Duchenne muscular dystrophy (DMD) and Becker (BMD) in scales and postural analysis. Methods: We evaluated 13 patients, age 16.75 (± 6.9) and 9 DMD, BMD 4, 7 wheelchair, wheelchair not 6, on the scales: Barthel Index and EK (Egen Klassifikation). The evaluation was performed in sitting posture software SAPO. Results: Data show that: increasing age (p <0.01), dependence on a wheelchair (p <0.01) and use of ventilator (p <0.01) showed less independence. Postural assessment of both the wheelchair and not the wheelchair, it was found that increase in the wheelchair groups, referring to the restrictions imposed by posture. Conclusion: These findings show that patients with DMD and BMD have its functionality and activities of daily living impaired with advancing age, dependence on a wheelchair and ventilator use. Thus, association of scales with conventional assessments and postural analysis are essential tools for research.


Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Young Adult , Muscular Dystrophy, Duchenne/complications , Postural Balance , Functional Status , Respiration, Artificial , Wheelchairs , Cross-Sectional Studies , Prospective Studies , Age Factors
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