Development of the Brazilian version of the Performance of Upper Limb scale for children and adolescents with Duchenne muscular dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is one of the most common and disabling childhood genetic diseases. The course of DMD involves progressive muscular degeneration and weakness, leading to functional decline. The Performance of the Upper Limb scale (PUL) is a specific instrument designed to assess the upper limb function of patients with DMD. OBJECTIVE: To adapt the PUL cross-culturally to Brazilian Portuguese (PUL-Br) and assess the convergent validity, structural validity, inter-rater reliability, and internal consistency for Brazilian patients with DMD METHODS: The cross-cultural adaptation involved six steps: translation to Brazilian Portuguese, Brazilian Portuguese translation synthesis, back-translation to English, back-translation synthesis, an expert committee review, and a pre-final version test (n = 12). The convergent validity of the PUL-Br was evaluated by examining its correlation to the Motor Function Measure scale (MFM) using 30 patients with DMD. Confirmatory factor analysis was conducted to assess structural validity. Intraclass correlation coefficient (ICC) verified the PUL-Br interrater reliability. Cronbach's alpha was calculated to verify internal consistency. RESULTS: The PUL was cross-culturally adapted to Brazilian Portuguese. A strong and positive correlation was found between the PUL-Br total score and the total score on the MFM (r = 0.83; 95% CI: 0.67, 0.91). The PUL-Br showed a satisfactory fit of the data to the three-factor model, excellent inter-rater reliability (ICC: 0.94), and good internal consistency (Cronbach's: 0.91). CONCLUSION: The PUL-Br is valid and reliable for assessing the upper limb function of Brazilian patients with DMD.

Cross-cultural adaptation and concurrent validity of the children's OMNI scale of perceived exertion for Arm-Crank activity in Spina Bifida.

PURPOSE: To perform a cross-cultural adaptation of the OMNI verbal descriptors to Brazilian-Portuguese and examine the validity of the Brazilian version for arm-crank activity. MATERIALS AND METHODS: Cross-cultural adaptation stages were: permission, translation, synthesis, back translation, expert committee review, pretesting, and submission and appraisal. For the concurrent validity, a Brazilian OMNI-Wheel scale was used to obtain rating of perceived exertion for the overall body (RPEOverall) and arms (RPEArms) in participants (n = 9, 10-17 years) with spina bifida. Cardiopulmonary exercise test was used to measure heart rate (HR) and oxygen uptake (VO2). Repeated Measures Correlation (rrm) was used to examine the scale validity. RESULTS: The cross-cultural adaptation produced equivalence between English and Brazilian-Portuguese verbal descriptors based on successful translation and pretesting. The Brazilian OMNI-wheel was validated based on strong associations of RPEOverall with VO2 (rrm (35) = 0.86, 95% CI [0.93, 0.73], p < 0.001) and HR (rrm (35) = 0.89, 95% CI [0.94, 0.79], p < 0.001) and RPEArms with VO2 (rrm (33) = 0.82, 95% CI [0.91, 0.66], p < 0.001) and HR (rrm (33) = 0.82, 95% CI [0.91, 0.66], p < 0.001). CONCLUSIONS: The OMNI scale was cross-culturally adapted to Brazilian-Portuguese. The Brazilian OMNI-Wheel was validated based on strong associations of RPE with HR and VO2. Implications For RehabilitationThe original English OMNI was cross-culturally adapted to Brazilian-Portuguese.A Brazilian wheelchair OMNI was concurrently validated for Arm-Crank Activity in adolescents with spina bifida.This OMNI scale version may aid health providers in monitoring perceived exertion in Brazil.

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

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.

Validity of the VO2peak prediction model to Brazilian youth with spina bifida / Validação de um modelo preditivo do VO2pico em jovens brasileiros com espinha bífida

Objective:To validate a peak oxygen uptake (VO2peak) prediction model in Brazilian youth with spina bifida. Methods:Twenty participants with spina bifida performed a graded arm crank test to measure VO2peak. The VO2peakvalues predicted by the equation "VO2peak(mL/min) = 194 + 18 × peak workload ­110 × sex" were compared to the VO2peakvalues measured. Results:The predicted VO2peakwas not different from the measured VO2peak. A high correlation was found between both VO2peak values, and the Bland-Altman analysis did not show a significant difference, demonstrating agreement between the values. Conclusions:The VO2peak prediction model in Brazilian youth with spina bifida was validated, being an advantageous alternative to assess and follow physical fitness and prescribe exercise training intensity.

Objetivo: Validar uma equação preditiva do consumo pico de oxigênio (VO2pico) em jovens brasileiros com espinha bífida. Métodos: Vinte participantes com espinha bífida realizaram um teste ergoespirométrico de membros superiores para medir o VO2pico. Os valores de VO2pico preditos pela equação "VO2pico (mL/min) = 194 + 18 × carga pico ­ 110 × sexo" foram comparados com o VO2pico medido. Resultados: O VO2pico predito pela equação não foi diferente do VO2pico medido. Foi encontrada alta correlação entre os valores de VO2pico e, a análise Bland Altman não mostrou diferença significativa, demonstrando concordância entre os valores. Conclusão: A equação preditiva do VO2pico é válida para jovens brasileiros com espinha bífida e é uma alternativa vantajosa para obter e acompanhar o condicionamento físico e prescrever a intensidade de treinamento nesses indivíduos.

The cross-cultural adaptation, construct validity, and intra-rater reliability of the functional mobility scale in Brazilian Portuguese for children and adolescents with spina bifida.

PURPOSE: The purpose of this study was to perform a cross-cultural adaptation of the Functional Mobility Scale (FMS) to Brazilian Portuguese and analyse its construct validity and intra-rater reliability in a sample of caregivers of children and adolescents with spina bifida (SB). MATERIAL AND METHODS: The cross-cultural adaptation followed five stages: two forward translations, synthesis, back-translation, committee review and pre-testing (n = 20). Construct validity was assessed by comparing the FMS with the classifications of Hoffer and Schoenmakers (n = 40). Intra-rater reliability was assessed by comparing the ratings of 14 caregivers, on two occasions. Kendall's tau correlation coefficient was used to test the construct validity of the FMS, while the kappa coefficient was used to test intra-rater reliability. RESULTS: Caregivers reported no difficulties with completing the FMS in Brazilian Portuguese. Construct validity tests showed positive correlations between the distances of 5 m (house), 50 m (school) and 500 m (community) in the FMS and the classifications of Hoffer (τ = 0.84; τ = 0.90; τ = 0.68; p < 0.01) and Schoenmakers (τ = 0.83; τ = 0.89; τ = 0.76; p < 0.01), respectively. Excellent intra-rater reliability (kappa = 0.9-1.0) was found for all three distances in the FMS. CONCLUSIONS: The FMS in Brazilian Portuguese showed acceptable intra-rater reliability and construct validity when used to measure the mobility level of patients with SB. We recommend its use in clinical practice and research.Implications for RehabilitationA valid and reliable instrument for assessing the mobility of patients with SB;A cross-cultural and adapted FMS in Brazilian Portuguese;An instrument for therapeutic and functional approaches outside the clinicalsetting;The FMS in the Brazilian Portuguese online version shows mistranslations.

Instruments to assess upper-limb function in children and adolescents with neuromuscular diseases: a systematic review.

AIM: To synthesize clinical and scientific evidence regarding the instruments available to assess upper-limb function in paediatric patients with neuromuscular disease (NMD). METHOD: This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) guidelines (Prospective Registry of Systematic Reviews no. CRD42020140343). Two independent reviewers searched the PubMed/MEDLINE, LILACS, Embase, and Scopus databases. Inclusion criteria were cross-sectional or longitudinal studies or randomized controlled trials that used scales or questionnaires to assess upper-limb function in paediatric patients with NMDs. The COSMIN Risk of Bias checklist and criteria for good measurement properties were applied to assess the methodological quality of the instruments. RESULTS: In total, 34 articles and 12 instruments were included. The Brooke Upper Extremity (n=16) and Performance of Upper Limb (PUL) (n=12) instruments were the most used tools. The PUL and Duchenne muscular dystrophy (DMD) Upper Limb patient-reported outcome measures (PROMs) tested more measurement properties and provided higher methodological quality scores for patients with DMD. Likewise, the Revised Upper Limb Module (RULM) was the most suitable instrument for patients with spinal muscular atrophy. No instrument has been devised to assess upper-limb function in patients with Charcot-Marie-Tooth disease and no other disease-specific instruments were found. INTERPRETATION: The PUL, DMD Upper Limb PROM, and RULM are the most suitable instruments to assess upper-limb function in the two most prevalent paediatric NMDs. The identified gaps and methodological flaws of the available instruments indicate a need to develop high-quality instruments to assess other types of paediatric NMDs. What this paper adds The most suitable observer-rater instrument to assess upper-limb function in Duchenne muscular dystrophy (DMD) is the Performance of Upper Limb. The most suitable observer-rater instrument to assess upper-limb function in spinal muscular atrophy is the Revised Upper Limb Module. The DMD Upper Limb patient-reported outcome measure is recommended to assess the upper-limb performance of patients with DMD. Literature gaps and methodological flaws indicate the need to develop high-quality instruments to assess other types of paediatric neuromuscular disease.

Abdominal muscular endurance in Brazilian children and adolescents: systematic review of cross-sectional studies / Resistência muscular abdominal em crianças e adolescentes do Brasil: revisão sistemática dos estudos transversais

Abstract The literature has demonstrated the importance of musculoskeletal fitness in the prevention of chronic noncommunicable diseases. Although current recommendations of physical activities include strengthening and muscular endurance aspects, little is known about the muscular endurance levels of children and adolescents in a national scope. The aim of this study was to systematically review the literature to identify the prevalence of Brazilian children and adolescents who meet health criteria for muscular endurance. A hierarchical search was conducted in four databases (MEDLINE; Scopus; SciELO; LILACS) using the following terms: "muscular endurance", "muscle endurance", "physical fitness", "child", "adolescent", "adults" "school" and correspondents in the Portuguese language. Overall, 2,652 articles (2,269 had their title and abstract read) were found and 70 were eligible for reading in full. Seventeen studies were reviewed and evaluated for risk of bias. Among 32,661 children and adolescents, only 40.2% of boys and 31.9% of girls presented abdominal muscular endurance adequate for health, and most studies were conducted in the southern region of Brazil. The variability in procedures for evaluating abdominal muscular endurance and cut-points used for interpretation occurred due to the use of different standardizations (PROESP/BR®, FITNESSGRAM® and AAHPERD®). Less than half of Brazilian adolescents of both sexes have adequate abdominal muscular endurance for health. Studies investigating the causes and consequences of inadequate abdominal muscular endurance may contribute to strategies for disease prevention and health promotion of children and adolescents.

Resumo A literatura tem demonstrado a importância da aptidão musculoesquelética na prevenção de doenças crônicas não transmissíveis. Embora as recomendações atuais de atividades físicas incluam aspectos de fortalecimento e a resistência muscular, pouco se conhece sobre os níveis de resistência muscular de crianças e adolescentes numa abrangência nacional. O objetivo deste estudo foi revisar sistematicamente a literatura para identificar a prevalência de crianças e adolescentes brasileiros que atendem aos critérios de saúde para a resistência muscular. Foi conduzida uma busca sistemática em quatro bases de dados (MEDLINE; Scopus; SciELO; LILACS), utilizando os termos "muscular endurance", "muscle endurance", "physical fitness", "child", "adolescent", "adults", "school" e correspondentes no idioma português. Foram encontrados 2.652 artigos (2.269 tiveram seu título e resumo lidos) e 70 foram elegíveis para leitura na íntegra. Dezessete estudos foram revisados e avaliados quanto ao risco de viés. Entre 32.661 crianças e adolescentes, apenas 40,2% dos meninos e 31,9% das meninas apresentaram resistência muscular abdominal adequada para a saúde, sendo que a maior parte dos estudos foi conduzida na região Sul do Brasil. A variabilidade nos procedimentos para avaliação da resistência muscular abdominal e nos pontos de corte utilizados para interpretação ocorreu devido à utilização de diferentes padronizações (PROESP/BR®, FITNESSGRAM® e AAHPERD®). Menos da metade dos adolescentes brasileiros de ambos os sexos tem resistência muscular abdominal adequada para a saúde. Estudos que investiguem as causas e as consequências da resistência muscular abdominal inadequada podem contribuir para estratégias de prevenção de doenças e promoção da saúde de crianças e adolescentes.