Efeitos do treinamento muscular inspiratório na função pulmonar de pacientes com Ataxia Telangiectasia

• Ano de defesa: 2011
• Autor(a) principal: Félix, Erika [UNIFESP]
• Tipo de documento: Dissertação
• Tipo de acesso: Acesso aberto
• Idioma: por
• Instituição de defesa: Universidade Federal de São Paulo (UNIFESP)
• Palavras-chave em Português: Immunologic deficiency syndromes; Inspiratory muscle training; Physical therapy modalities; Respiratory function tests; Ataxia telangiectasia; Exercise therapy; Breathing exercises; Adolescent; Child; Modalidades de fisioterapia; Síndromes de imunodeficiência; Testes de função respiratória; Treinamento muscular inspiratório; Terapia por exercício; Exercícios respiratórios; Adolescente; Criança
• Link de acesso: https://repositorio.unifesp.br/handle/11600/9426

Background: Ataxia Telangiectasia (A-T) is an autosomal recessive syndrome, characterized by defects in DNA repair or replication which is caused by a mutation in a gene on the chromosome 11. The typical manifestations are cerebellar ataxia, telangiectasia and progressive muscular weakness. Besides, they also have a restrictive ventilatory pattern due to gradual loss of muscle strength, resulting in increased susceptibility to lung infections and severe respiratory failure. Although there is a lot of data about the effects of inspiratory muscle training in patients with chronic pulmonary diseases, there are no studies that correlate its effects specifically in A-T patients. Objective: To evaluate the effects of IMT on inspiratory muscle strength and its impact on pulmonary function and on quality of life of patients with A-T. Methods: This is a controlled, longitudinal intervention. We have selected 11 patients with A-T and 9 healthy controls matched for age and sex. A-T patients and controls underwent an inicial evaluation protocol that included: (i) measuring of ventilometry to obtain the minute volume (MV), tidal volume (VT), vital capacity (VC), respiratory rate (f) and ( ii) manovacuometry assessing the maximum inspiratory pressure (MIP) and maximal expiratory pressure (MEP). In this same phase, patients with A-T underwent further evaluation of (iii) quality of life using the SF-36 and (iv) application of the Borg scale for assessment of perceived shortness of breath. These same parameters were evaluated in the A-T group in the post-IMT period. The IMT protocol was initiated with a load of 40% of MIP, with weekly increases of 5%, up to 60% of MIP, which is the target load. The IMT was performed for 20 minutes daily during 24 weeks. Results: A-T patients, when compared with the control group, showed a significant difference in weight and height (36.91 ± 91vs 54.22 ± 8.80, p = 0.001), MIP and MEP in absolute terms (p <0.001) as percentage of predicted (p <0.001 and p = 0.009), and VC (p <0.001), respectively. In the post-IMT period, A-T patients showed a significant increase in VT (476.5 ± 135 ml vs 583.3 ± 66 ml, p = 0.015), VC (1664 ± 463 ml/kg vs 2145 ± 750 ml/kg , p = 0.002), MIP (22.2 ± 2 cmH2O vs 38 ± 9 cmH2O, p <0.001), MEP (29 ± 7 cmH2O vs 40 ± 8 cmH2O, p = 0.001), and significant decrease in f (23 , 3 ± 6 rpm vs 20.4 ± 4 rpm, p = 0.018) compared to baseline IMT. There was a decrease in the Borg/dyspnoea ratio (p = 0.022) and improvement in the domains of the SF-36 related to general health (p = 0.009) and vitality (p = 0.014). Conclusion: IMT was significantly effective in increasing muscle strength, with subsequent improvement in lung function in patients with A-T and should be considered an adjunctive therapy to drug treatment to improve quality of life of these patients.