Background: Progressive respiratory muscle weakness and ineffective cough contributes to pulmonary morbidity and mortality in children with neuromuscular disease. Inspiratory muscle training aims to preserve or improve respiratory muscle strength, reduce respiratory complications and improve health-related quality of life. Objectives: To describe South African physiotherapists' knowledge and respiratory management strategies and determine the safety, viability, acceptability and efficacy of inspiratory muscle training for children 5-18 years with neuromuscular disease. Methods: Four studies were conducted: i) a quantitative descriptive survey; ii) a systematic review using Cochrane methodology; iii) a prospective, pre-experimental observational study and iv) a prospective, cross-over randomised controlled trial using a standardised 12-week inspiratory muscle training intervention. Results: i) South African physiotherapists (n=64) reported being aware of international clinical practice recommendations, however they favoured manual airway clearance techniques. The use of inspiratory muscle training in chronic management was well supported by South African physiotherapists. ii) Results of the systematic review (seven included studies; n=168) suggested that inspiratory muscle training may be effective in improving inspiratory muscle strength. There was insufficient evidence for an effect on patient morbidity or health-related quality of life. iii) The pre-experimental, pilot study (n=8) suggested that a six-week inspiratory muscle training programme was safe, viable, acceptable and associated with a significant increase in inspiratory muscle strength. iv) The cross-over randomised controlled trial (n=23) did not show evidence of a difference in the primary outcome measures (number of hospitalisations and respiratory tract infections) between intervention and control periods. There were no adverse events related to inspiratory muscle training. Inspiratory muscle strength (Pimax) and peak expiratory cough flow increased by 14.57 (±15.67)cmH2O and 32.27 (±36.60)L/min respectively during the intervention period compared to a change of 3.04 (±11.93)cmH2O (p=0.01) and -16.59 (±48.29)L/min (p=0.0005) during the control period. There was no evidence of change in spirometry, functional ability or total health-related quality of life scores following the intervention. Overall participant satisfaction with inspiratory muscle training was high and adherence was good. Conclusions: Inspiratory muscle training in children with neuromuscular disease is well tolerated, appears to be safe and is associated with significant improvements in inspiratory muscle strength and cough efficacy.

Introdução: Pacientes com hipertensão pulmonar (HP) além de apresentar redução da capacidade funcional, da qualidade de vida e da sobrevida, apresentam fraqueza dos músculos respiratórios, o que pode contribuir para o aumento dos sintomas de fadiga e dispneia. Objetivos: Verificar o efeito do treinamento muscular inspiratório (TMI) sobre a capacidade funcional, a qualidade de vida, a força muscular respiratória, pressão sistólica da artéria pulmonar, a função pulmonar e o nível de atividade física de pacientes com HP. Métodos: Doze pacientes com HP foram randomizados em um grupo controle (n=7) e um grupo TMI (n=5). O protocolo do TMI foi realizado durante 8 semanas. Foram feitas avaliações da pressão arterial pulmonar média, através do ecocardiograma; da qualidade de vida, através do questionário SF-36; da capacidade funcional, através do teste de caminhada de 6 minutos e da força muscular respiratória, através de transdutor de pressão. Todos os pacientes foram avaliados na fase pré e pós protocolo. Resultados: A força muscular inspiratória aumentou significativamente no grupo TMI quando comparado ao grupo controle (105,2±6,6 vs 82,9±6,1; p=0,01), e o escore de saúde mental avaliado pelo questionário SF-36 aumentou no grupo TMI (de 70,4±21,6 para 80±14,4; p=0,05). Entretanto a capacidade funcional não apresentou alteração após o protocolo de TMI de 8 semanas. Conclusão: O TMI é capaz de aumentar a força muscular inspiratória e melhorar a qualidade de vida, em relação a saúde mental, de pacientes com HP. / Introduction: Besides showing pulmonary hypertension (PH), decreased functional capacity, quality of life and survival, patients also present respiratory muscle weakness, which may contribute to increased fatigue and dyspnea symptoms. Objectives: Evaluate the effects of inspiratory muscle training (IMT) on functional capacity, quality of life, respiratory muscle strength, pulmonary artery pressure, pulmonary function and level of physical activity. Methods: Twelve chronic PH patients randomized to a control group (n = 7) and an IMT group (n = 5) were studied. The IMT program was performed for 8 weeks. The following measures were obtained before and after the program: respiratory muscle function; function capacity (6-min walk test); pulmonary artery pressure; quality of life (SF-36); and level de physical activity (IPAQ). Results: Maximal inspiratory pressure (PI,max) was higher in the IMT group than in the control group (105.2±6.6 vs 82.9±6.1; p=0.01), and the mental health score by SF-36 increased in de IMT group (from 70.4±21.6 to 80±14.4; p=0.05). However, the six-min walk test did not change after the IMT program. Conclusions: This study indicates that IMT results in improvement in inspiratory muscle strength and in the quality of life regarding PH.

Introdução: Pacientes com hipertensão pulmonar (HP) além de apresentar redução da capacidade funcional, da qualidade de vida e da sobrevida, apresentam fraqueza dos músculos respiratórios, o que pode contribuir para o aumento dos sintomas de fadiga e dispneia. Objetivos: Verificar o efeito do treinamento muscular inspiratório (TMI) sobre a capacidade funcional, a qualidade de vida, a força muscular respiratória, pressão sistólica da artéria pulmonar, a função pulmonar e o nível de atividade física de pacientes com HP. Métodos: Doze pacientes com HP foram randomizados em um grupo controle (n=7) e um grupo TMI (n=5). O protocolo do TMI foi realizado durante 8 semanas. Foram feitas avaliações da pressão arterial pulmonar média, através do ecocardiograma; da qualidade de vida, através do questionário SF-36; da capacidade funcional, através do teste de caminhada de 6 minutos e da força muscular respiratória, através de transdutor de pressão. Todos os pacientes foram avaliados na fase pré e pós protocolo. Resultados: A força muscular inspiratória aumentou significativamente no grupo TMI quando comparado ao grupo controle (105,2±6,6 vs 82,9±6,1; p=0,01), e o escore de saúde mental avaliado pelo questionário SF-36 aumentou no grupo TMI (de 70,4±21,6 para 80±14,4; p=0,05). Entretanto a capacidade funcional não apresentou alteração após o protocolo de TMI de 8 semanas. Conclusão: O TMI é capaz de aumentar a força muscular inspiratória e melhorar a qualidade de vida, em relação a saúde mental, de pacientes com HP. / Introduction: Besides showing pulmonary hypertension (PH), decreased functional capacity, quality of life and survival, patients also present respiratory muscle weakness, which may contribute to increased fatigue and dyspnea symptoms. Objectives: Evaluate the effects of inspiratory muscle training (IMT) on functional capacity, quality of life, respiratory muscle strength, pulmonary artery pressure, pulmonary function and level of physical activity. Methods: Twelve chronic PH patients randomized to a control group (n = 7) and an IMT group (n = 5) were studied. The IMT program was performed for 8 weeks. The following measures were obtained before and after the program: respiratory muscle function; function capacity (6-min walk test); pulmonary artery pressure; quality of life (SF-36); and level de physical activity (IPAQ). Results: Maximal inspiratory pressure (PI,max) was higher in the IMT group than in the control group (105.2±6.6 vs 82.9±6.1; p=0.01), and the mental health score by SF-36 increased in de IMT group (from 70.4±21.6 to 80±14.4; p=0.05). However, the six-min walk test did not change after the IMT program. Conclusions: This study indicates that IMT results in improvement in inspiratory muscle strength and in the quality of life regarding PH.

Introdução: Pacientes com hipertensão pulmonar (HP) além de apresentar redução da capacidade funcional, da qualidade de vida e da sobrevida, apresentam fraqueza dos músculos respiratórios, o que pode contribuir para o aumento dos sintomas de fadiga e dispneia. Objetivos: Verificar o efeito do treinamento muscular inspiratório (TMI) sobre a capacidade funcional, a qualidade de vida, a força muscular respiratória, pressão sistólica da artéria pulmonar, a função pulmonar e o nível de atividade física de pacientes com HP. Métodos: Doze pacientes com HP foram randomizados em um grupo controle (n=7) e um grupo TMI (n=5). O protocolo do TMI foi realizado durante 8 semanas. Foram feitas avaliações da pressão arterial pulmonar média, através do ecocardiograma; da qualidade de vida, através do questionário SF-36; da capacidade funcional, através do teste de caminhada de 6 minutos e da força muscular respiratória, através de transdutor de pressão. Todos os pacientes foram avaliados na fase pré e pós protocolo. Resultados: A força muscular inspiratória aumentou significativamente no grupo TMI quando comparado ao grupo controle (105,2±6,6 vs 82,9±6,1; p=0,01), e o escore de saúde mental avaliado pelo questionário SF-36 aumentou no grupo TMI (de 70,4±21,6 para 80±14,4; p=0,05). Entretanto a capacidade funcional não apresentou alteração após o protocolo de TMI de 8 semanas. Conclusão: O TMI é capaz de aumentar a força muscular inspiratória e melhorar a qualidade de vida, em relação a saúde mental, de pacientes com HP. / Introduction: Besides showing pulmonary hypertension (PH), decreased functional capacity, quality of life and survival, patients also present respiratory muscle weakness, which may contribute to increased fatigue and dyspnea symptoms. Objectives: Evaluate the effects of inspiratory muscle training (IMT) on functional capacity, quality of life, respiratory muscle strength, pulmonary artery pressure, pulmonary function and level of physical activity. Methods: Twelve chronic PH patients randomized to a control group (n = 7) and an IMT group (n = 5) were studied. The IMT program was performed for 8 weeks. The following measures were obtained before and after the program: respiratory muscle function; function capacity (6-min walk test); pulmonary artery pressure; quality of life (SF-36); and level de physical activity (IPAQ). Results: Maximal inspiratory pressure (PI,max) was higher in the IMT group than in the control group (105.2±6.6 vs 82.9±6.1; p=0.01), and the mental health score by SF-36 increased in de IMT group (from 70.4±21.6 to 80±14.4; p=0.05). However, the six-min walk test did not change after the IMT program. Conclusions: This study indicates that IMT results in improvement in inspiratory muscle strength and in the quality of life regarding PH.

- examination and treatment took place in ambulant facility REHAMIL s.r.o. in Lysá nad f PhDr. Jitka Malá, PhD and pneumologist MUDr. Šárka Klimešová, PhD. g's dyspnea scale, Otto inclination and reclination distance, respiratory the reduction in exertional dyspnea assessed by the Borg's dyspnea scale (p