Navegando por Palavras-chave "Immunologic deficiency syndromes"

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    Efeitos do treinamento muscular inspiratório na função pulmonar de pacientes com Ataxia Telangiectasia
    (Universidade Federal de São Paulo (UNIFESP), 2011-03-30) Félix, Erika [UNIFESP]; Costa-Carvalho, Beatriz Tavares [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)
    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.
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    Pneumonias de repetição em paciente com deficiência de anticorpos e imunoglobulinas normais
    (Sociedade Brasileira de Pneumologia e Tisiologia, 2002-06-01) Costa-Carvalho, Beatriz Tavares [UNIFESP]; Cocco, Renata Rodrigues [UNIFESP]; Rodrigues, Waldinei M. [UNIFESP]; Colla, Viviane A. [UNIFESP]; Solé, Dirceu [UNIFESP]; Carneiro-Sampaio, Magda Maria Sales; Universidade Federal de São Paulo (UNIFESP); Universidade de São Paulo (USP)
    The authors report the case of a 7-year-old girl, with a history of recurrent respiratory infections since five months of age. Immunological evaluation at four years of age revealed a normal level of total serum immunoglobulins, but a deficient humoral response to Streptococcus pneumoniae. Although having all normal IgG subclasses, the patient had inadequate antibody levels to all serotypes tested (1, 3, 5, 6, 9, and 14). Concurrent radiological evaluation showed diffuse atelectasis and bronchiectasis. Patient was started on IVIG, and subsequent chest CT scan revealed resolution of the above-mentioned lesions. To prevent irreversible damage to lung parenchyma, due to recurrent lower respiratory infections, it is important to identify immunocompromised individuals and treat them accordingly.