scholarly journals Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil

2018 ◽  
Vol 18 (1) ◽  
pp. 83-92
Author(s):  
Bárbara Bernardo Rinaldo da Silva Figueirêdo ◽  
Paulo André Freire Magalhães ◽  
Liívia Barbosa de Andrade ◽  
Patrícia Bezerra ◽  
Maria do Carmo Menezes Bezerra Duarte

Abstract Objectives: to assess the functional independence, functional capacity and respiratory muscle strength (RMS) in individuals with mucopolysaccharidosis (MPS) type VI. Methods: in this cross-sectional study, the Pediatric Evaluation of Disability Inventory (PEDI) and the Functional Independence Measure (FIM) scales were used to assess the functional independence. The functional capacity and the RMS were assessed by a 6-minute walk test (6MWT) and Manovacuometry, respectively. The associations between RMS and 6MWD were analyzed by using the adjusted simple linear regression models. And besides, the T-test was used to compare the differences among the groups. Results: twenty-four patients with MPS VI were included, the median age was 8 (ranged from 2-38 years old). The PEDI presented a functional performance below the expectations at the self-care and mobility domains of 33.3% when compared to groups of Brazilian children at the same age assessed with typical development. The decreased social function was found in only one single case. According to the FIM, of the 12 patients evaluated (age> 7.5 years), 58.3% were classified as modified dependence and 41.7% as modified independence. The mean distance in the 6MWT was significantly lower than predicted, 263m in G1 (children, n= 7) and 336m in G2 (adolescents and adults, n=6), p<0.001. The RMS was also lower than predicted in both groups, except for the RMS in G1. Conclusions: the functional capacity and the RMS were significantly reduced in individuals with MPS VI, with a decreased functional independence in one-third of the patients. However, a multidisciplinary follow-up in this population is essential to prevent, diagnose and treat early complications.

2019 ◽  
Vol 77 (6) ◽  
pp. 387-392 ◽  
Author(s):  
Rafaela Sant’anna dos SANTOS ◽  
Sara Carolina Fontoura DALL’ALBA ◽  
Soraia Genebra Ibrahim FORGIARINI ◽  
Daniele ROSSATO ◽  
Alexandre Simões DIAS ◽  
...  

ABSTRACT Stroke often leads to abnormalities in muscle tone, posture, and motor control that may compromise voluntary motor function, thus affecting the motor control required for maintaining the synergy of both peripheral and respiratory muscles. Objective To evaluate respiratory muscle strength, pulmonary function, trunk control, and functional independence in patients with stroke and to correlate trunk control with the other variables. Methods This was a cross-sectional study of patients diagnosed with stroke. We assessed respiratory muscle strength, trunk control as assessed by the Trunk Impairment Scale, spirometric variables, and the Functional Independence Measure. Results Forty-four patients were included. Pulmonary function and respiratory muscle strength were significantly lower than predicted for the study population, and the mean Trunk Impairment Scale score was 14.3 points. The following significant correlations were found between the variables: trunk control vs. maximal inspiratory pressure (r = 0.26, p < 0.05); trunk control vs. forced vital capacity (r = 0.28, p < 0.05); trunk control vs. forced expiratory volume in one second (r = 0.29, p < 0.05), and trunk control vs. the Functional Independence Measure (r = 0.77, p < 0.05). Conclusion The present study showed that respiratory muscle strength, pulmonary function, functional independence, and trunk control are reduced in patients diagnosed with stroke.


2016 ◽  
Vol 29 (2) ◽  
pp. 343-350 ◽  
Author(s):  
Simone Regina Posser ◽  
Sheila Cristina Cecagno-Zanini ◽  
Fabiana Piovesan ◽  
Camila Pereira Leguisamo

Abstract Introduction: Individuals with chronic kidney disease (CKD) undergoing hemodialysis (HD) present low cardiorespiratory fitness and functional capacity. Metabolic changes, due to the disease, can result in a variety of pathophysiological conditions that favor the development of respiratory muscle weakness. However, very little is known about the performance of the respiratory muscles and the influence of HD on them. Aim: To evaluate and correlate pulmonary function, functional capacity and respiratory muscle strength in patients with CKD undergoing HD. Methods: Cross-sectional study comprising 23 patients with CKD, that met the following inclusion criteria: patients of both genders, who perform HD three times a week for a minimum period of three months. Respiratory muscle strength was evaluated using a respiratory pressure meter, lung function through spirometry and functional capacity through the 6-minute walk test (6MWT) before the HD session. Results: All patients were male and mean age was 50.2 ± 15.8 years. The median duration of HD was 3 (1.5 to 6.0) years. The mean values obtained in comparison to those predicted were MIP% 36.0 ± 13.6, MEP% 49.5 ± 15.8, FVC% 93.8 ± 21.1, FEV1% 93.7 ± 21.1, FVC/VEF1% 104.1 ± 10.3, and 6MWT% 66.33 ± 20.53. A statistically significant positive correlation was observed between the 6MWT and MIP (r = .63, p =.001) and MEP (r = .67, p < .001), between the MIP and MEP (r =.79, p < .001) and between the FEV1 and FVC (r = .91, p < .001). Conclusion: Patients with CKD undergoing HD present changes in respiratory muscle strength, with the predicted values decreasing for age and gender, as well as the distance covered in the 6MWT, although, with normal spirometric values. Functional capacity was dependent on respiratory muscle strength, as well as the values of MIP and MEP, and the values of FVC and FEV1.


2016 ◽  
Vol 48 ◽  
pp. 835
Author(s):  
RENATA TRIMER ◽  
Katyane Lopes Zangrando ◽  
Paula Angelica Ricci ◽  
Humberto Lanzotti ◽  
Daniela K. Andaku ◽  
...  

2017 ◽  
Vol 63 (2) ◽  
pp. 76-83 ◽  
Author(s):  
Ana Irene Carlos de Medeiros ◽  
Helen Kerlen Bastos Fuzari ◽  
Catarina Rattesa ◽  
Daniella Cunha Brandão ◽  
Patrícia Érika de Melo Marinho

2009 ◽  
Vol 127 (2) ◽  
pp. 78-83 ◽  
Author(s):  
Rodrigo Polaquini Simões ◽  
Viviane Castello ◽  
Marco Antonio Auad ◽  
Jadiane Dionísio ◽  
Marisa Mazzonetto

CONTEXT AND OBJECTIVES: Respiratory muscle strength is relevant to the clinical situation of elderly patients, particularly those presenting with respiratory or cardiac diseases. The objectives of this study were to evaluate the respiratory muscle strength of institutionalized elderly women, compare this with predicted values for the Brazilian population and calculate the correlation with age and anthropometric characteristics. DESIGN AND SETTING: Cross-sectional study at the Department of Physiotherapy of Universidade Camilo Castelo Branco. METHODS: The participants were 56 institutionalized elderly women (74.87 ± 10.55 years of age), evaluated in eight institutions in three cities in the central region of the State of São Paulo, between January 2005 and March 2006. They were separated into three subgroups according to age: 60-69 years (n = 20), 70-79 (n = 18) and 80-89 years (n = 18). Maximal respiratory pressures were obtained using a manovacuometer. The values obtained were compared between subgroups and with predicted values. Correlation analysis was used to evaluate age, weight, height and body mass index in relation to maximal respiratory pressures. The significance level was P < 0.05. RESULTS: No significant differences in maximal respiratory pressures were seen between the three subgroups. The maximal respiratory pressures were significantly lower in the three subgroups, compared with predicted values. Negative correlations between maximal respiratory pressures and age and positive correlations in relation to weight, height and body mass index were found. CONCLUSIONS: Respiratory muscle strength was markedly reduced in institutionalized 60 to 89-year-old women and the values demonstrated correlations with age and anthropometric characteristics.


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