Lung function in severe pediatric asthma: a longitudinal study in children and adolescents in Brazil

Abstract Background In severe asthma, high doses of inhaled corticosteroids (ICS) are used in order to achieve clinical and functional control. This study aimed to evaluate lung function in outpatients (children and adolescents) with severe asthma in Brazil, all of whom were treated with high doses of ICS. We evaluated all spirometry tests together and by ICS dose: 800 and > 800 µg/day. Methods This was a 3-year longitudinal study in which we analyzed 384 spirometry tests in 65 severe asthma patients (6–18 years of age), divided into two groups by the dose of ICS (budesonide or equivalent): 800 and > 800 µg/day. Results At baseline, the forced expiratory volume in one second (FEV1) and the FEV1/forced vital capacity (FVC) ratio were both  800 µg/day group, there were no statistical increases or decreases in FEV1, the FEV1/FVC ratio, or forced expiratory flow between 25 and 75% of the FVC (FEF25–75%), when calculated as percentages of the predicted values. However, the z-score for FEF25–75% showed a statistically significant reduction, in the sample as a whole and in the > 800 µg/day group. Also in the > 800 µg/day group, there was a significant reduction in the post-bronchodilator FEV1% (p = 0.004). Conclusions The fact that the spirometric parameters (as percentages of the predicted values) remained constant in the > 800 µg/day group, whereas there was a gain in lung function in the sample as a whole, suggests an early plateau phase in the > 800 µg/day group. However, there was some loss of lung function in the > 800 µg/day group, as evidenced by a decrease in the z-score for FEF25–75%, suggesting irreversible small airway impairment, and by a reduction in the post-bronchodilator FEV1%, suggesting reduced reversibility of airway obstruction. Among children and adolescents with severe asthma, the use of ICS doses higher than those recommended for age does not appear to improve lung function

The Newcastle Pediatric Mitochondrial Disease Scale: translation and cultural adaptation for use in Brazil

ABSTRACT Objective The aim of this study was to translate and adapt the Newcastle Paediatric Mitochondrial Disease Scale (NPMDS) to Portuguese for use in Brazil. Methods The scale was applied in 20 pediatric patients with mitochondrial disease, in three groups: myopathy (n = 4); Leigh syndrome (n = 8); and encephalomyopathy (n = 8). Scores were obtained for the various dimensions of the NPMDS, and comparisons were drawn between the groups. Results There was a statistically significant difference between the myopathy group and the Leigh syndrome group (p = 0.0085), as well as between the myopathy and encephalomyopathy groups (p = 0.01). Conclusions The translation of the NPMDS, and its adaptation to the socioeconomic and cultural conditions in Brazil, make the NPMDS score useful as an additional parameter in the evaluation and monitoring of pediatric patients with MD in Brazil

Atualização sobre a abordagem da fisioterapia respiratória nas doenças vneuromusculares - doi:10.5020/18061230.2010.p92

Objective: To assess the role of physiotherapy in approaching neuromuscular disease (NMD), with emphasis on preventive and therapeutic aspects of respiratory therapy. Methods: A nonsystematic literature review covering the past twenty years, using the databases MEDLINE and LILACS through the following descriptors: neuromuscular diseases, physical therapy, vital capacity and respiratory failure. Results: The studies reviewed show the need to establish a routine periodic evaluation of respiratory function in order to introduce physical therapy measures relevant to each stage of the disease. The monitoring should include pulmonary function tests and specific techniques of chest physiotherapy, in order to avoid complications such as respiratory failure. Conclusion: The introduction of regular monitoring and preventive physiotherapy measures have helped to increase survival and improve quality of life of patients with neuromuscular diseasesObjetivo: Avaliar o papel da fisioterapia na abordagem das doenças neuromusculares (DNM), com ênfase nos aspectos preventivos e terapêuticos da fisioterapia respiratória. Método: Revisão bibliográfica não sistemática, abrangendo os últimos vinte anos, utilizando as bases de dados MEDLINE e LILACS, através dos seguintes descritores: Doenças neuromusculares, fisioterapia, capacidade vital e insuficiência respiratória. Resultados: Os artigos pesquisados sugerem a necessidade de se estabelecer uma rotina de avaliação periódica da função respiratória a fim de se introduzir medidas fisioterapêuticas pertinentes a cada estágio da doença. O acompanhamento deve incluir provas de função pulmonar, bem como técnicas específicas de fisioterapia respiratória, a fim de se evitar complicações como insuficiência respiratória. Conclusão: O acompanhamento periódico e a introdução de medidas preventivas de fisioterapia têm contribuído para aumentar a sobrevida e melhoraria da qualidade de vida dos pacientes com doenças neuromusculares

Update on the approach of respiratory therapy in patients with neuromuscular diseases

Objective: To assess the role of physiotherapy in approaching neuromuscular disease (NMD), with emphasis on preventive and therapeutic aspects of respiratory therapy. Methods: A nonsystematic literature review covering the past twenty years, using the databases MEDLINE and LILACS through the following descriptors: neuromuscular diseases, physical therapy, vital capacity and respiratory failure. Results: The studies reviewed show the need to establish a routine periodic evaluation of respiratory function in order to introduce physical therapy measures relevant to each stage of the disease. The monitoring should include pulmonary function tests and specific techniques of chest physiotherapy, in order to avoid complications such as respiratory failure. Conclusion: The introduction of regular monitoring and preventive physiotherapy measures have helped to increase survival and improve quality of life of patients with neuromuscular diseases

O uso da visita domiciliar na asma grave pediátrica: estudo experimental randomizado controlado

Objetivo: Avaliar a eficácia da visita domiciliar, em um grupo de crianças e adolescentes com asma grave, utilizando o escore da técnica inalatória e as taxas de adesão ao tratamento medicamentoso. Método: Estudo experimental randomizado controlado, com duração de 12 meses, envolvendo pacientes, com idades entre três e 17 anos, acompanhados regularmente em um ambulatório de pneumologia pediátrica de um hospital universitário da região Sudeste do Brasil. Um grupo de pacientes recebeu apenas consultas ambulatoriais e o outro grupo, além das consultas ambulatoriais, recebeu visitas da enfermeira no domicílio. Foram analisadas as diferenças entre os grupos nos desfechos: escores da técnica inalatória e taxa de adesão ao tratamento. Resultados: Participaram 29 pacientes. No grupo-intervenção, houve aumento estatisticamente significante dos escores da técnica inalatória (p<0,05) e eliminação de erros críticos da primeira para a segunda avaliação, resultados que foram mantidos na terceira. Não houve alterações significativas na técnica inalatória do grupo-controle. As taxas de adesão ao tratamento medicamentoso em ambos os grupos não se elevaram. Conclusão: A visita domiciliar foi eficaz em melhorar os escores de técnica inalatória em pacientes com asma grave. Registro Brasileiro de Ensaios Clínicos: RBR-8GZWZP.Objective: To evaluate the effectiveness of home visits in a group of children and adolescents with severe asthma by using the inhalation technique score and adherence rates to drug treatment. Method: A 12-month randomized controlled trial involving patients aged between three and 17 years under regular follow-up treatment at a pediatric pulmonology outpatient clinic of a university hospital in southeastern Brazil. A group of patients received only outpatient consultations and the other group received home visits from nurses in addition to outpatient consultations. The differences between groups were analyzed through the outcomes of inhalation technique scores and treatment adherence rate. Results: Participation of 29 patients. In the intervention group, there was a statistically significant increase in inhalation technique scores (p<0.05) and elimination of critical errors between the first and the second evaluation, and results were maintained in the third evaluation. In the control group, there were no significant changes in inhalation technique scores. Rates of adherence to drug treatment in both groups did not rise. Conclusion: Home visits were effective for improving inhalation technique scores in patients with severe asthma. Brazilian Registry of Clinical Trials: RBR-8GZWZP.Objetivo: Evaluar la efectividad de la visita domiciliaria en un grupo de niños y adolescentes con asma severa, utilizando el score de la técnica inhalatoria y las tasas de adhesión al tratamiento medicamentoso. Método: Estudio experimental randomizado controlado, con duración de 12 meses, que incluyó a pacientes con edades entre tres y 17 años, acompañados regularmente en un ambulatorio de neumología pediátrica de un hospital universitario de la región Sureste de Brasil. Un grupo de pacientes recibió solo consultas ambulatorias y el otro grupo, además de las consultas ambulatorias, recibió visitas de la enfermera en el hogar. Fueron analizadas las diferencias entre los grupos en los resultados: scores de la técnica inhalatoria y tasa de adhesión al tratamiento. Resultados: Participaron 29 pacientes. En el grupo intervención, hubo aumento estadísticamente significativo de los scores de la técnica inhalatoria (p<0,05) y eliminación de errores críticos de la primera a la segunda evaluación, resultados que se mantuvieron en la tercera. No hubo modificaciones significativas en la técnica inhalatoria del grupo de control. Las tasas de adhesión al tratamiento medicamentoso en ambos grupos no se elevaron. Conclusión: La visita domiciliaria fue eficaz en mejorar los scores de técnica inhalatoria en pacientes con asma severa. Registro Brasileño de Ensayos Clínicos: RBR-8GZWZ