Assessment of the Clinical and Functional Health Status of Patients with Amyotrophic Lateral Sclerosis during the COVID-19 Pandemic in Brazil Using Telemedicine.

This study aimed to monitor the clinical and functional progression of patients with amyotrophic lateral sclerosis (ALS) and adjust ventilatory support during the COVID-19 pandemic in Brazil using telemedicine. This longitudinal case series included five evaluations from January 2019 to June 2021. The first and second assessments were performed in person and consisted of pulmonary function, respiratory muscle strength, functionality (ALS Functional Rating Scale-Revised [ALSFRS-R]) and disease staging (King's College criteria). The use of non-invasive ventilation (NIV), ALSFRS-R, and disease staging were assessed in the third, fourth, and fifth assessments during the COVID-19 pandemic, using telemedicine. The rate of functional decline was calculated by the difference in the total score of ALSFRS-R between evaluations. A cutoff of 0.77 in the ALSFRS-R was used to characterize the speed of functional decline. Eleven patients (mean age of 51 years, eight males) were assessed. The total score of the ALSFRS-R (p < 0.01) and its motor domain (p < 0.01) reduced significantly during the pandemic. NIV prescription increased from 54.4% to 83.3%. Telemedicine helped with the clinical and functional follow-up of patients with ALS.

Non-invasive assessment of fatigue and recovery of inspiratory rib cage muscles during endurance test in healthy individuals.

INTRODUCTION: Fatigue is defined as loss of capacity to develop muscle force and/or velocity that is reversible at rest. We assessed non-invasively the fatigue and recovery of inspiratory rib cage muscles during two respiratory endurance tests in healthy individuals. METHODS: The sniff nasal inspiratory pressure (SNIP) was assessed before and after two respiratory endurance tests: normocapnic hyperpnea (NH) and inspiratory pressure threshold loading (IPTL). Contractile (maximum rate of pressure development and time to peak pressure) and relaxation parameters (maximum relaxation rate [MRR], time constant of pressure decay [τ], and half relaxation time) obtained from sniff curves and shortening velocity and mechanical power estimated using optoelectronic plethysmography were analyzed during SNIP maneuvers. Respiratory muscle activity (electromyography) and tissue oxygenation (near-infrared spectroscopy-NIRS) were obtained during endurance tests and SNIP maneuvers. Fatigue development of inspiratory rib cage muscles was assessed according to the slope of decay of median frequency. RESULTS: Peak pressure during SNIP decreased after both protocols (p <0.05). MRR, shortening velocity, and mechanical power decreased (p <0.05), whereas τ increased after IPTL (p <0.05). The median frequency of inspiratory rib cage muscles (i.e., sum of sternocleidomastoid, scalene, and parasternal) decreased linearly during IPTL and exponentially during NH, mainly due to the sternocleidomastoid. CONCLUSION: Fatigue development behaved differently between protocols and relaxation properties (MRR and τ), shortening velocity, and mechanical power changed only in the IPTL.

Noninvasive assessment of respiratory muscle strength and activity in Myotonic dystrophy.

OBJECTIVE: To evaluate sensitivity/specificity of the maximum relaxation rate (MRR) of inspiratory muscles, amplitude of electromyographic activity of the sternocleidomastoid (SCM), scalene (SCA), parasternal (2ndIS) and rectus abdominis (RA) muscles; lung function and respiratory muscle strength in subjects with Myotonic dystrophy type 1 (DM1) compared with healthy subjects. DESIGN AND METHODS: Quasi-experimental observational study with control group. MRR of inspiratory muscles, lung function and amplitude of the electromyographic activity of SCM, SCA, 2ndIS and RA muscles during maximum inspiratory pressure (PImax), maximum expiratory pressure (PEmax) and sniff nasal inspiratory pressure (SNIP) tests were assessed in eighteen DM1 subjects and eleven healthy. RESULTS: MRR was lower in DM1 group compared to healthy (P = 0.001) and was considered sensitive and specific to identify disease in DM1 and discard it in controls, as well as SNIP% (P = 0.0026), PImax% (P = 0.0077) and PEmax% (P = 0.0002). Contraction time of SCM and SCA was higher in DM1 compared to controls, respectively, during PImax (P = 0.023 and P = 0.017) and SNIP (P = 0.015 and P = .0004). The DM1 group showed lower PImax (P = .0006), PEmax (P = 0.0002), SNIP (P = 0.0014), and higher electromyographic activity of the SCM (P = 0.002) and SCA (P = 0.004) at rest; of 2ndIS (P = 0.003) during PEmax and of SCM (P = 0.02) and SCA (P = 0.03) during SNIP test. CONCLUSIONS: MD1 subjects presented restrictive pattern, reduced respiratory muscle strength, muscular electrical activity and MRR when compared to higher compared to controls. In addition, the lower MRR found in MD1 subjects showed to be reliable to sensitivity and specificity in identifying the delayed relaxation of respiratory muscles.

Effects of diaphragmatic control on the assessment of sniff nasal inspiratory pressure and maximum relaxation rate

OBJECTIVE: To assess the influence of diaphragmatic activation control (diaphC) on Sniff Nasal-Inspiratory Pressure (SNIP) and Maximum Relaxation Rate of inspiratory muscles (MRR) in healthy subjects. METHOD: Twenty subjects (9 male; age: 23 (SD=2.9) years; BMI: 23.8 (SD=3) kg/m2; FEV1/FVC: 0.9 (SD=0.1)] performed 5 sniff maneuvers in two different moments: with or without instruction on diaphC. Before the first maneuver, a brief explanation was given to the subjects on how to perform the sniff test. For sniff test with diaphC, subjects were instructed to perform intense diaphragm activation. The best SNIP and MRR values were used for analysis. MRR was calculated as the ratio of first derivative of pressure over time (dP/dtmax) and were normalized by dividing it by peak pressure (SNIP) from the same maneuver. RESULTS: SNIP values were significantly different in maneuvers with and without diaphC [without diaphC: -100 (SD=27.1) cmH2O/ with diaphC: -72.8 (SD=22.3) cmH2O; p<0.0001], normalized MRR values were not statistically different [without diaphC: -9.7 (SD=2.6); with diaphC: -8.9 (SD=1.5); p=0.19]. Without diaphC, 40% of the sample did not reach the appropriate sniff criteria found in the literature. CONCLUSION: Diaphragmatic control performed during SNIP test influences obtained inspiratory pressure, being lower when diaphC is performed. However, there was no influence on normalized MRR.

Effects of diaphragmatic control on the assessment of sniff nasal inspiratory pressure and maximum relaxation rate.

OBJECTIVE: To assess the influence of diaphragmatic activation control (diaphC) on Sniff Nasal-Inspiratory Pressure (SNIP) and Maximum Relaxation Rate of inspiratory muscles (MRR) in healthy subjects. METHOD: Twenty subjects (9 male; age: 23 (SD=2.9) years; BMI: 23.8 (SD=3) kg/m²; FEV1/FVC: 0.9 (SD=0.1)] performed 5 sniff maneuvers in two different moments: with or without instruction on diaphC. Before the first maneuver, a brief explanation was given to the subjects on how to perform the sniff test. For sniff test with diaphC, subjects were instructed to perform intense diaphragm activation. The best SNIP and MRR values were used for analysis. MRR was calculated as the ratio of first derivative of pressure over time (dP/dtmax) and were normalized by dividing it by peak pressure (SNIP) from the same maneuver. RESULTS: SNIP values were significantly different in maneuvers with and without diaphC [without diaphC: -100 (SD=27.1) cmH2O/ with diaphC: -72.8 (SD=22.3) cmH2O; p<0.0001], normalized MRR values were not statistically different [without diaphC: -9.7 (SD=2.6); with diaphC: -8.9 (SD=1.5); p=0.19]. Without diaphC, 40% of the sample did not reach the appropriate sniff criteria found in the literature. CONCLUSION: Diaphragmatic control performed during SNIP test influences obtained inspiratory pressure, being lower when diaphC is performed. However, there was no influence on normalized MRR.

Costs and benefits of pulmonary rehabilitation in chronic obstructive pulmonary disease: a randomized controlled trial.

OBJECTIVE: The current study evaluated the costs and benefits of a simple aerobic walking program for patients with chronic obstructive pulmonary disease (COPD). METHOD: This was a blinded randomized controlled clinical trial that recruited 72 patients diagnosed with COPD, 40 of whom were included in the study and divided into two groups [control group (CG) and pulmonary rehabilitation group (GPR)]. We assessed pulmonary function, distance covered during the 6-minute walk test (6MWT), respiratory and peripheral muscle strength, health-related quality of life (HRQOL), body composition, and level of activities of daily living (ADLs) before and after an 8-week walking program. The financial costs were calculated according to the pricing table of the Brazilian Unified Health System (SUS). RESULTS: Only 34 of the 40 patients remained in the final sample; 16 in the CG and 18 in the GPR (FEV1: 50.9 ± 14% predicted and FEV1: 56 ± 0.5% predicted, respectively). The intervention group exhibited improvements in the 6MWT, sensation of dyspnea and fatigue, work performed, BODE index (p<0.01), HRQOL, ADL level (p<0.001), and lower limb strength (p<0.05). The final mean cost per patient for the GPR was R$ 148.75 (~US$ 75.00) and no patient significantly exceeded this value. However, 2 patients in the CG did exceed this value, incurring a cost of R$ 689.15 (~US$ 345.00). CONCLUSION: Aerobic walking demonstrated significant clinical benefits in a cost-efficient manner in patients with COPD.

Costs and benefits of Pulmonary Rehabilitation in Chronic Obstructive Pulmonary Disease: a randomized controlled trial

Objective: The current study evaluated the costs and benefits of a simple aerobic walking program for patients with chronic obstructive pulmonary disease (COPD). Method: This was a blinded randomized controlled clinical trial that recruited 72 patients diagnosed with COPD, 40 of whom were included in the study and divided into two groups [control group (CG) and pulmonary rehabilitation group (GPR)]. We assessed pulmonary function, distance covered during the 6-minute walk test (6MWT), respiratory and peripheral muscle strength, health-related quality of life (HRQOL), body composition, and level of activities of daily living (ADLs) before and after an 8-week walking program. The financial costs were calculated according to the pricing table of the Brazilian Unified Health System (SUS). Results: Only 34 of the 40 patients remained in the final sample; 16 in the CG and 18 in the GPR (FEV1: 50.9±14% predicted and FEV1: 56±0.5% predicted, respectively). The intervention group exhibited improvements in the 6MWT, sensation of dyspnea and fatigue, work performed, BODE index (p<0.01), HRQOL, ADL level (p<0.001), and lower limb strength (p<0.05). The final mean cost per patient for the GPR was R$ 148.75 (~US$ 75.00) and no patient significantly exceeded this value. However, 2 patients in the CG did exceed this value, incurring a cost of R$ 689.15 (~US$ 345.00). Conclusion: Aerobic walking demonstrated significant clinical benefits in a cost-efficient manner in patients with COPD. .