Comparison of the modified shuttle walk test in children with cystic fibrosis and healthy controls.

OBJECTIVE: To evaluate and compare performance in the modified shuttle walk test (MSWT) with cardiorespiratory parameters and respiratory muscle strength in children with cystic fibrosis (CF) and healthy controls. METHODS: A cross-sectional study with children divided into the CF group (CFG) and healthy control group (HCG). Two MSWTs were performed and the data from the test with the longest distance walked and its cardiorespiratory parameters (blood pressure, respiratory rate, heart rate (HR), oxygen saturation, and dyspnea sensation) were considered, the last three every 4 levels. Respiratory muscle strength was evaluated using a manometer. The Wilcoxon test was used to compare the parameters before and after the MSWT, and the Mann-Whitney and independent t-tests were applied to compare the data between the groups. The distance walked and its association with the variation in cardiorespiratory parameters and respiratory muscle strength were analyzed by Spearman's correlation. RESULTS: Sixty-two children (31 in each group) participated, with an average age of 10.2 (2.1) years. Children with CF had poorer performance in the average distance walked (CFG 716.7 (274.3)) when compared to the HCG (948.0 (202.8)). Both groups exhibited an increase in all the cardiorespiratory parameters at the end of the test, but the CFG displayed less variation in some parameters. Children with CF presented a positive correlation between distance walked and respiratory muscle strength, HR variation, and systolic blood pressure. CONCLUSIONS: There was no difference in respiratory muscle strength between the groups. In the CFG, the greater the respiratory muscle strength, the better the performance in the MSWT. The CFG exhibited less variation in cardiorespiratory parameters than the HCG.

"Test-retest reliability and minimal detectable change in TGlittre-P test in children and adolescents with cystic fibrosis".

OBJECTIVE: to provide test-retest reliability for the TGlittre-P in children and adolescents with cystic fibrosis (CFG) and healthy controls (HCG), to establish the minimal detectable change for time in TGlittre-P and comparing the performance in the TGlittre-P test between these populations. METHOD: A cross-sectional study evaluated 36 children and adolescents aged 6 to 13. Anthropometric and spirometric evaluation was performed, as well as, on the same day, two TGlittre-P tests with a 30-minute interval between them. RESULTS: TGlittre-P time test-retest reliability was excellent for both groups (CFG: intraclass correlation coefficient [ICC] = 0.849, p < 0.001 and HCG: ICC = 0.913, p < 0.001). As concerning absolute reliability, the time spent presented a small variability with a standard error of measurement of 8.4 s (s) to CFG and 5.3 s to HCG. The minimal detectable change at 95% confidence level (MDC95) was 23.2 s and 14.6 s, respectively. There was no difference between the groups regarding performance in the TGlittre-P test (CFG 179.1 s ± 25.7 s vs. HCG 174.7 s ± 22.3 s) p = 0.589. CONCLUSION: The TGlittre-P is a reliable tool in children and adolescents with CF and healthy controls. The TGlittre-P appears not to be sensitive enough to discriminate a group of children and adolescents with mild cystic fibrosis from healthy counterparts.IMPLICATIONS FOR REHABILITATIONTGlittre-P is a multitasking test that has been used to assess the functional capacity of children and adolescents with chronic diseases.TGlittre-P has excellent reliability in children and adolescents with and without CF.TGlittre-P differences time greater than 12% could indicate changes in the functional capacity of children and adolescents with CF.Other functional capacity tests may be preferred to detect continuous increases in functional capacity through rehabilitation or training, whether children and adolescents obtain performance values close to 100% of predicted.

Immediate effect of inhalation therapy combined with oscillatory positive expiratory pressure on the respiratory system of children with cystic fibrosis.

INTRODUCTION: It is recommended the association of inhalation therapies and physiotherapy on the management of cystic fibrosis (CF); however, it is still necessary to understand the effect on respiratory mechanics of these therapies combined. This study aimed to evaluate the immediate effect of inhalation with Dornase-Alfa (DNase) and hypertonic saline solution (HSS), as well as the impact of these inhalation therapies associated with an oral high-frequency oscillation (OHFO) physiotherapy device, on the respiratory mechanics of children and adolescents with CF. METHOD: Children/adolescents with CF were allocated into two groups (DNaseG and HSSG), where they performed inhalation therapy before using the OHFO device for physiotherapy. In each group, the Impulse Oscillometry System was conducted before and after inhalation therapy, and after OHFO. ANOVA was carried out to analyse the respiratory mechanics at different moments of DNaseG and HSSG. The Mann-Whitney test compared the immediate effect of each inhalation therapy and after OHFO. RESULTS: 30 children (6-14 years old) were studied. In DNaseG, the mean value of most oscillometric parameters decreased in the evaluated moments; in HSSG, only reactance showed an immediate increase. CONCLUSION: Children/adolescents with CF showed an immediate decrease in airway resistance and reactance after the use of DNase and associated with OHFO, indicating improvement. The inhalation with HSS has an immediate effect on peripheral airways.

Força muscular respiratória e qualidade de vida em crianças e adolescentes com fibrose cística / Fuerza muscular respiratoria y calidad de vida en niños y adolescentes con fibrosis quística / Respiratory muscle strength and quality of life in children and adolescent with cystic fibrosis

RESUMO A fibrose cística (FC) é uma doença genética multissistêmica caracterizada por obstrução crônica que, associada a outras alterações pulmonares, pode comprometer a força muscular respiratória (FMR) e, em consequência, interferir no desempenho de atividades típicas da infância, alterando a qualidade de vida (QV) dessa população. O objetivo do estudo foi avaliar a relação entre FMR e QV de crianças e adolesceantes com FC. Trata-se de um estudo transversal, que incluiu pacientes com idades entre 6 e 14 anos, provenientes de um centro de referência no Brasil. Realizou-se avaliação antropométrica e da FMR, esta utilizando as pressões inspiratória (PImáx) e expiratória máximas (PEmáx) por meio da manovacuômetria digital (Globalmed® MVD300). Aplicou-se o Cystic Fibrosis Questionnaire (QFC), questionário específico para a avaliar a QV nessa doença, nas versões para crianças (QFC-C) e para os pais ou responsáveis (QFC-R). A gravidade da doença foi classificada segundo o escore de Schwachman Doeurshuk (ESD). Dados sobre a colonização de bactérias e o genótipo da doença foram consultados por meio da análise dos prontuários. Analisou-se os dados por meio do software SPSS version 20.0 for Windows. Após o teste Shapiro-Wilk, aplicou-se o teste de correlação de Pearson ou Spearman. Em toda a análise foi adotado nível de significância de 5%. Participaram do estudo 28 crianças (15 meninos) com média de idade de 10,10±1,79 anos, as quais apresentaram FMR próxima ao predito e pontuações do QFC indicando boa QV. O ESD relacionou-se negativamente com domínio digestivo (p=0,03; rho=-0,400). A PEmáx apresentou correlação negativa com domínio corpo do QFC-R (p=0,002; rho=-0,426) e com domínio tratamento do QFC-C (p=0,01; rho=-0,453). A PImáx apresentou correlação positiva com os domínios físico (p=0,03; rho=0,410), emocional (p=≤0,001; rho=0,573) e tratamento (p=≤0,01; rho=-0,605) do QFC-C. A PImáx também mostrou correlação positiva com o domínio respiratório (p=0,01; rho=0,572) do QFC-R. Em conclusão, identificou-se associação entre domínios da QV e FMR, bem como com o ESD e aspectos nutricionais. Essa amostra apresentou valores de FMR acima do esperado e boa QV.

RESUMEN La fibrosis quística (FQ) es una enfermedad genética multisistémica caracterizada por una obstrucción crónica que, asociada a otras alteraciones pulmonares, puede comprometer la fuerza de los músculos respiratorios (FMR), lo que en consecuencia interfiere en el desempeño de las actividades típicas de la infancia alterando la calidad de vida (CV) de esta población. El objetivo de este estudio fue evaluar la relación entre la FMR y la CV de niños y adolescentes con FQ. Este es un estudio transversal, en el cual participaron pacientes de entre 6 y 14 años de un centro de referencia en Brasil. Se realizaron evaluaciones antropométricas y de FMR, para esta se utilizó presión inspiratoria máxima (PImáx.) y presión espiratoria máxima (PEmáx.) mediante manovacuometría digital (Globalmed MVD300). Se aplicó el Cuestionario de fibrosis quística (CFC) específico para evaluar la CV en esta enfermedad, en versiones para niños (CFC-N) y para padres o tutores (CFC-T). La clasificación de la gravedad de la enfermedad siguió el puntaje de Schwachman Doeurshuk (ESD). Se consultaron datos sobre la colonización bacteriana y el genotipo de la enfermedad mediante el análisis de historias clínicas. Para el análisis de datos se utilizó el software SPSS versión 20.0 para Windows. Tras la prueba de Shapiro-Wilk, se aplicó la prueba de correlación de Pearson o Spearman. El nivel de significancia que se adoptó fue del 5%. En el estudio participaron 28 niños (15 varones), con promedio de edad de 10,10±1,79 años, que presentaron puntuaciones de FMR cerca al predicho y el puntaje de CFC que indicaba una buena CV. El ESD se relacionó negativamente con el dominio digestivo (p=0,03; rho=-0,400). La PEmáx. presentó una correlación negativa con el dominio cuerpo de CFC-T (p=0,002; rho=-0,426) y con el dominio de tratamiento de CFC-N (p=0,01; rho=-0,453). La PImáx. mostró una correlación positiva con los dominios físico (p=0,03; rho=0,410), emocional (p=≤0,001; rho=0,573) y tratamiento (p=≤0,01; rho=-0,605) del CFC-N. La PImáx. también presentó una correlación positiva con el dominio respiratorio (p=0,01; rho=0,572) del CFC-T. En conclusión, se identificó una asociación entre los dominios CV y FMR, así como el ESD y los aspectos nutricionales. Esta muestra presentó valores de FMR superiores a los esperados y una buena CV.

ABSTRACT Cystic fibrosis (CF) is a multisystemic genetic disease characterized by chronic obstruction that, associated with other pulmonary changes, can compromise respiratory muscle strength (RMS) and, consequently, interfere with the performance of typical childhood activities, changing the quality of life(QOL) of this population. The aim of the study was to evaluate the relationship between RMS and QOL of children and adolescents with CF. This is a cross-sectional study, which included patients without acute pulmonary exacerbation, aged between 6 and 14 years, from a reference center in Brazil. Anthropometric and RMS assessments were performed, using maximum inspiratory (MIP) and expiratory (MEP) pressures using digital manovacuometry (Globalmed® MVD300). The Cystic Fibrosis Questionnaire was applied, a specific questionnaire to assess QOL in this disease, in versions for children (QOL-C) and for parents or guardians (QOL-P). The severity of the disease was classified according to the Schwachman Doeurshuk score (ESD). Data on colonization and genotype were consulted through the analysis of medical records. The data was analyzed using the SPSS version 20.0 for Windows software. After the Shapiro-Wilkt test, Pearson's or Spearman's correlation test was applied. Throughout the analysis, a significance level of 5% was adopted. Twenty-eight children (15 boys) participated in the study, with a mean age of 10.10 ± 1.79 years, who had a near-predicted RMS and QOL scores indicating good QOL. The ESD was negatively related to the digestive domain (p=0.03; rho=-0.400). MEP showed a negative correlation with the QOL-P body domain (p=0.002; rho=-0.426) and with the QOL-C treatment domain (p=0.01; rho=-0.453). MIP showed a positive correlation with the physical (p=0.03; rho=0.410), emotional (p=≤0.001; rho=0.573) and treatment (p=≤0.01; rho=-0.605) domains of the QOL-C. MIP also showed a positive correlation with the respiratory domain (p=0.01; rho=0.572) of the QOL-P. In conclusion, an association was identified between QOL and RMS domains, as well as with ESD and nutritional aspects. This sample showed higher than expected RMS values and good QOL.

Respiratory physical therapy techniques recommended for patients with cystic fibrosis treated in specialized centers.

BACKGROUND: Respiratory therapy is a part of the treatment of patients with cystic fibrosis (CF). However, there is no information about the main factors influencing the recommendation of the use of different techniques or devices by physical therapists from different specialized centers. OBJECTIVE: To determine the respiratory therapy techniques recommended for the treatment of patients with CF seen in specialized treatment centers in Brazil. METHODS: This is a descriptive study including a sample of Brazilian CF specialized treatment centers. Data on demographics, spirometric variables, and recommended respiratory therapy treatment techniques were collected. RESULTS: Twelve specialized treatment centers with a total of 974 patients were included (13.5±11.3 years old and FEV1 (%) 75.7±27.9). The most recommended techniques were huffing (61.1% of patients), high frequency oral oscillation (HFOO) (52.0%), and positive expiratory pressure (PEP) (45.3%). Most often, recommendation was to perform treatment once (54.8% of patients) or twice (34%) a day. There was great variability in the recommendation among the different states. When data were separated by age, there was a predominance of performing conventional and manual techniques in infants and preschool children. There were no significant variations according to pulmonary function. Based on the literature, techniques based on volume, huffing, and PEP were most prevalently performed in international centers. CONCLUSION: The most recommended treatment techniques for patients with CF in Brazil are huffing, HFOO, and PEP, followed by volume-based techniques. There were geographical variations in the preferred treatment techniques, as well as based on patient age, but not based on the level of pulmonary function.