Navegando por Palavras-chave "Lung Function"
Agora exibindo 1 - 4 de 4
Resultados por página
Opções de Ordenação
- ItemAcesso aberto (Open Access)Ataxia telangiectasia: evolução da escala SARA (Scale for the Assessment and Rating of Ataxia) com força de preensão palmar e força muscular inspiratória(Universidade Federal de São Paulo (UNIFESP), 2019-11-04) Trajano, Karina Kakiuchi [UNIFESP]; Sole, Dirceu [UNIFESP]; Lanza, Fernanda de Cordoba [UNIFESP]; http://lattes.cnpq.br/5288152697269527; http://lattes.cnpq.br/8188258243306974; http://lattes.cnpq.br/7478208044937030; Universidade Federal de São Paulo (UNIFESP)Ataxia Telangiectasia (A-T) is a rare, hereditary, non-raceable, neurodegenerative disease that affects both sexes. It results from the ATM mutation (ataxiatelangiectasia, mutated) on chromosome 11q22-23. The clinical presentation is progressive and debilitating with evidenced motor impairment. Objective: To evaluate the evolution of motor function, palmar grip strength, and respiratory muscle strength in patients with ataxia telangiectasia. Methods: This was a longitudinal study in which 24 patients (children, adolescents and adults) with A-T diagnosis were evaluated. The Scale for the Assessment and Rating of Ataxia (SARA) scale which assess posture, gait, balance, coordination and speech was used for motor assessment. The score ranges from 0 to 40, the higher the severity of ataxia. Palmar grip strength was assessed as a measure of peripheral muscle strength with the analog dynamometer. The inspiratory pressure (PiMáx) and expiratory pressure (PeMáx) maxims were used to measure respiratory muscle strength by using the manovacuometer. Pulmonary function was assessed by spirometry according to recommendations, with the following outcomes: forced vital capacity (FVC), forced expiratory volume on the first second (FEV1), relationship between (FEV1/FVC) and forced expiratory flow (FEF). All evaluations mentioned were repeated every three months for a year, totaling four evaluations. Patients were questioned about the history of disease and performance and / or follow-up of physical therapy during the study months. Results: of the total number of volunteers, 17 (71%) were males, mean age 13 years 7.6 years, symptoms onset at 1.3 years (1 month - 4 years of life) and telangiectasia at 3.3 years (1 month up to 10 years of life); who were diagnosed at 4.7 years (1-11 years). Of the total number of patients, 14 (58%) underwent supervised physiotherapy and the others, unsupervised physical therapy. There was no change at SARA scale at baseline (19.5 +/- 9.7 – 21.7) at the end of the protocol (18.5 +/- 9.7 – 22.0), p = 0.72. No change in palmar grip values (12.1 ± 5.5 kg) was observed for the final (12.4 ± 6.1 kg), p = 0.79. PiMax increased over the course of 12 months, initial 69.1 +/- 26.6 cmH2O vs final 83.1 +/- 29,6 cmH2O, p = 0.03, in the same manner as PeMax of 50.5 +/- 14.8 cmH2O to 58.9 +/- 29.6 cmH 2 O final, p = 0.04. Conclusions: Although A-T is a degenerative and progressive disease, no motor worsening and muscular strength peripheral were observed over 12 months, and respiratory muscle strength showed significant improvement.
- ItemSomente MetadadadosAvaliação da função pulmonar e da capacidade funcional em pacientes com mucopolissacaridose(Universidade Federal de São Paulo (UNIFESP), 2021) Dias, Bianca Moreira Cardoso [UNIFESP]; Wandalsen, Gustavo Falbo [UNIFESP]; Universidade Federal de São PauloIntroduction: Mucopolysaccharidoses (MPS) are a group of rare diseases caused by the intralisosomal accumulation of glycosaminoglycans, secondary to enzyme deficiencies. Respiratory impairment and impaired functionality may be present in MPS, however, there is a lack of studies on these aspects. Objective: To evaluate lung function and functional capacity in patients with MPS and compare it with a control group. Methods: This is an unicentric and cross-sectional study of patients aged 6 to 39 years, followed up at a Brazilian referral center. Lung function was assessed by spirometry and impulse oscillometry (IOS) and functional capacity was assessed by the Five times Sit-To-Stand test (FTSTS) and Shuttle Walk Incremental Test (ISWT). All tests were performed according to recommended standards and in the absence of respiratory infections. The results were compared with a control group by equivalent age and sex. Results: 21 patients with MPS were evaluated, most of them male (90%), with an average age of 17 ± 11 years. The most frequent type of MPS was II (62%). All patients underwent enzyme replacement therapy (ERT). Alteration in lung function was found in 13/16 (81%) patients, with the presence of obstructive and restrictive disorder. In comparison with control group, patients with MPS had lower values of FEV1 (%) (75,6 ± 13,5 vs 91,3 ± 11,4, p <0.01) and FVC (%) (80,4 ± 12,9 vs 96,0 ± 12,4, p <0, 01), fifteen percent showed a bronchodilator response. At IOS, 18/21 (86%) patients had changes, with a predominance of distal obstruction (47%). MPS patients had higher airway resistance values [R5 (%): 157,1 ± 63,0 vs 111,5 ± 35,3, p <0.01 and R20 (%): 1509, ± 60,2 vs 115,7 ± 30,8, p <0.01] when compared to control group, thirty-three percent showed a bronchodilator response. In the FTSTS, the MPS group needed almost 40% more time to perform the test (10.6 ± 2.5s vs 6.7 ± 1.2s, p <0.01). In the ISWT, patients with MPS reached significantly lower distance values than control group (407,6 ± 329,8m vs 1131,9 ± 183,3m, p <0.01) Conclusions: Eighty-six percent of patients with MPS showed changes in lung function, with the presence of obstructive and restrictive ventilatory disorder, indicating that the impairment of the respiratory system in MPS is multicausal. MPS patients also had worse functional performance when compared to their healthy peers, suggesting worse physical conditioning.
- ItemSomente MetadadadosPrevalência de asma e avaliação da função pulmonar em pacientes com doença de Fabry(Universidade Federal de São Paulo (UNIFESP), 2021) Monteiro, Fernanda Pereira [UNIFESP]; Wandalsen, Gustavo Falbo [UNIFESP]; Universidade Federal de São PauloFabry disease (FD) is characterized by excessive accumulation of globotriaosylceramide inside the lysosome, affecting mainly the vascular endothelium with repercussion in multiple systems. Pulmonary complications in FD are still not well established. The objectives of our study were to assess lung function in patients with a confirmed diagnosis of DF who were followed up at a Brazilian referral center and to repeat the evaluations after 12 months to monitor the evolutionary patterns. Methods: The study included patients with a confirmed diagnosis of FD. Lung function was evaluated by spirometry and impulse oscillometry (IOS). Symptoms and medical history were obtained by questionnaire and review of medical records. The reassessment followed the interval of 12 months ± 2 months. Results: In the first moment, forty-seven patients were evaluated. Spirometry was abnormal in 32% of the patients and IOS in 47%. Restrictive pattern of pulmonary dysfunction was found in 60% (spirometry) and in 50% (IOS) of patients with abnormal results. Positive bronchodilator response was observed in 17% of the patients. Worse values of pulmonary function were observed in patients with proteinuria (FEV1: 80% vs 93%, p=0.02; R5: 152% vs 116%, p<0.01), fatigue (FEV1: 83% vs 97%, p=0.02), wheezing in the last year (R5: 164% vs 125%, p=0.02), renal insufficiency (FEV1: 65% vs 89%, p<0.01), and with classical phenotype (X5: 1.7cmH2O/L/s vs 2.6 cmH2O/L/s, p=0.04). Forty-one of these patients were reassessed. Spirometry was abnormal in 48% after one year with a mixed pattern (restrictive and obstructive) predominating. Altered IOS were found in 39% after reassessment. Worse evolution of pulmonary function values was observed in patients with classic phenotype (p = 0.04) and there was a trend towards worse evolution of pulmonary function in male patients. Conclusions: Pulmonary involvement is a relevant manifestation of FD, especially observed with disease progression and in the classical phenotype. IOS seems to be a sensitive test that may play a complementary role to spirometry in the evaluation of the pulmonary function of these patients, but a year is a relatively short period to monitor the evolution of the disease and progression in the deterioration of lung function.
- ItemAcesso aberto (Open Access)Relação entre alteração torácica musculoesquelética e prova de função pulmonar em lactentes nascidos prematuros e com muito baixo peso(Universidade Federal de São Paulo (UNIFESP), 2017-02-21) Marinonio, Ana Silvia Scavacini [UNIFESP]; Santos, Amelia Miyashiro Nunes dos [UNIFESP]; Davidson, Josy; Wandalsen, Gustavo Falbo [UNIFESP]; http://lattes.cnpq.br/6101587222452300; http://lattes.cnpq.br/4807350957191775; http://lattes.cnpq.br/9925075057283150; http://lattes.cnpq.br/8611935304738792; Universidade Federal de São Paulo (UNIFESP)Preterm infants may present lung function alterations. Furthermore, studies showed thoracic musculoskeletal alterations due to respiratory pattern alterations in with very low birth weight preterm infants, especially those with bronchopulmonary dysplasia. Objective: To verify the prevalence of thoracic musculoskeletal alterations and lung function alterations and the existence of an association between both variables in toddlers from 6 months to 1 year of age born before 37 weeks of gestational age and birth weight less than or equal to 1500g. Methods: Cross-sectional study with a convenience sample of children 6-12 months of corrected age born prematurely with very low birth weight. Malformations, neurological diseases or respiratory diseases whiting the last 15 days were excluded. Pulmonary function was evaluated using the Infant Pulmonary Lab-Collins, USA, according to the American Thoracic Society techniques and measurements of thoracic angles: Acromium/manubrium/acromium (A1), Acromium/xiphoid appendix/acromium (A2), Manubrium/left acromium/trapezium (A3), Right inframamilar point/xiphoid apendix/left inframamilar point (A4), Left trapezium/acromium/left deltoid (A5) and Measure of costal retraction (Ma) by photogrammetry using the Software for postural analysis SAPO®. Univariate linear regression was used to study the association between measurements of thoracic angles and parameters of pulmonary function. Results: 38 toddlers were studied, 50% male (gestational age 29.9 ± 2.4 weeks, birth weight 1.145 ± 252 g, corrected age 8.9 ± 2.2 m, weight 7.760 ± 1.138 kg and height 68.8 ± 4.8cm). Of the 38 infants, 31.5% had pulmonary function alterations and 24% and 32% presented alterations in A3 and Ma angles. The linear regression showed significant associations between A2 and FEV0.5 <-2 z-score, between A3 and FVC <-2 z-score, between A4 and VR <80% of predicted value and FEV0.5/FVC <- 2 z-score and between A5 and VR <80% of predicted. Conclusion: Premature infants show relatively high frequency of pulmonar function and thoracic musculoskeletal alterations. The musculoskeletal alterations due to hypertrophy of the trapezium and deltoid muscles were associated to alterations of lung function, showing that the shape of the thorax might be influenced by pulmonary function.