Ottoni IE1, dos Santos DO1, Mattiello-Sverzut AC1, Moroli RG1, Nascimento JP1, Perossi L1, Holtz M1, Gastaldi AC1
1University of Sao Paulo, Ribeirao Preto, Brazil
Background: Duchenne Muscular Dystrophy (DMD) is a hereditary, degenerative and progressive disease. Individuals initially present functional disability and during evolution may have pulmonary implications. In patients with neuromuscular diseases, the measurement of respiratory muscle function and strength are important measures for evaluation, early therapeutic intervention and prognosis.
Purpose: To correlate measures of motor function using the motor function measure scale (MFM) and the strength of respiratory muscles in patients with DMD.
Methods: The subjects were evaluated by MFM, which includes static and dynamic evaluations, divided into three dimensions, that provides a Total Score, and dimension 1 (D1) - standing position and transfers, 13 items; dimension 2 (D2) - axial and proximal motor function, 12 items, and dimension 3 (D3): distal motor function, 7 items (6 referring to the upper limbs). Each item was graded on a 4-point scale (scores from 0 to 3). Maximal inspiratory and expiratory pressures (MIP and MEP, respectively) were evaluated, as well as the sniff test (Psniff) with a digital manovacuometer. Data were analysed using the statistical programs R (RCore Team, Vienna, Austria). Correlations between the MFM (total score, D1, D2 and D3) and percentage of predicted for MIP, MEP and Psniff tests were calculated by the Pearson correlation coefficient, and p 0.05 was considered significant.
Results: A total of 28 male children were evaluated, with a mean age of 13.13 ± 3.78 years. The statistical analysis showed significant correlations between MEP and Total Score (r = 0.67, p = 0.00006), D1 (r = 0.60, p = 0.0005), D2 (r = 0.63, p = 0.0001), D3 0.002). Also significant correlations were obtained between Psniff and Total Score (r = 0.56, p = 0.00005), D1 (r = 0.42, = 0.003), D2 (r = 0.64, p = 0.000002) and D3 0.002). There was no significant correlation between MFM and MIP.
Conclusion(s): The decrease in the functional capacity is positively correlated with a decrease in the respiratory muscle strength, assessed by MEP and Psniff.
Implications: To plan actions to improve or maintain the strength of the respiratory muscles, especially the expiratory, in an early intervention; to conduct a better and more objective assessment, avoiding unnecessary and fatiguing measures for these individuals.
Keywords: Duchenne Muscular Dystrophy, Respiratory Assessment, Respiratory Muscles
Funding acknowledgements: Laboratory of Assessment Respiratory and Clinical Hospital of the Ribeirão Preto Medical School.
Purpose: To correlate measures of motor function using the motor function measure scale (MFM) and the strength of respiratory muscles in patients with DMD.
Methods: The subjects were evaluated by MFM, which includes static and dynamic evaluations, divided into three dimensions, that provides a Total Score, and dimension 1 (D1) - standing position and transfers, 13 items; dimension 2 (D2) - axial and proximal motor function, 12 items, and dimension 3 (D3): distal motor function, 7 items (6 referring to the upper limbs). Each item was graded on a 4-point scale (scores from 0 to 3). Maximal inspiratory and expiratory pressures (MIP and MEP, respectively) were evaluated, as well as the sniff test (Psniff) with a digital manovacuometer. Data were analysed using the statistical programs R (RCore Team, Vienna, Austria). Correlations between the MFM (total score, D1, D2 and D3) and percentage of predicted for MIP, MEP and Psniff tests were calculated by the Pearson correlation coefficient, and p 0.05 was considered significant.
Results: A total of 28 male children were evaluated, with a mean age of 13.13 ± 3.78 years. The statistical analysis showed significant correlations between MEP and Total Score (r = 0.67, p = 0.00006), D1 (r = 0.60, p = 0.0005), D2 (r = 0.63, p = 0.0001), D3 0.002). Also significant correlations were obtained between Psniff and Total Score (r = 0.56, p = 0.00005), D1 (r = 0.42, = 0.003), D2 (r = 0.64, p = 0.000002) and D3 0.002). There was no significant correlation between MFM and MIP.
Conclusion(s): The decrease in the functional capacity is positively correlated with a decrease in the respiratory muscle strength, assessed by MEP and Psniff.
Implications: To plan actions to improve or maintain the strength of the respiratory muscles, especially the expiratory, in an early intervention; to conduct a better and more objective assessment, avoiding unnecessary and fatiguing measures for these individuals.
Keywords: Duchenne Muscular Dystrophy, Respiratory Assessment, Respiratory Muscles
Funding acknowledgements: Laboratory of Assessment Respiratory and Clinical Hospital of the Ribeirão Preto Medical School.
Topic: Cardiorespiratory
Ethics approval required: Yes
Institution: Ribeirão Preto Medical School
Ethics committee: Ethics Committee of the Hospital and Ribeirão Preto Medical School
Ethics number: 57001216.0.000.5440
All authors, affiliations and abstracts have been published as submitted.
