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Abstract: The sit-to-stand test provides insight into age-related functional capacity; however, there are various variants of sit-to-stand, and we do not know which of these better discriminates against age-related functional capacity. Our study aimed to compare the age-related functional capacity in older people by evaluating sit-to-stand power variants, using young individuals as a reference. A cross-sectional study was conducted in 102 adults (57 women) aged 60-80 and 105 adults (54 women) aged 20-30. Participants performed five times sit-to-stand (5 sit-to-stand), 30-second sit-to-stand, and 1-min sit-to-stand. Z scores were obtained for each sit-to-stand variant using power (W), relative (W/kg), and allometric (W/m 2 ) normalization methods. A mixed repeated-measures analysis of variance assessed the interactions among the sit-to-stand variants, normalization methods, sex, physical activity, and tobacco history. A significant interaction between sit-to-stand variants, normalization methods, and sex ( P = 0.002) was found. The mean effect of sit-to-stand variants revealed that the 1-min sit-to-stand had the lowest Z score ( P < 0.05). Significant variations were observed between sit-to-stand variants in all normalization methods for women ( P < 0.001). However, in men, only the difference between 5 sit-to-stand and 1-min sit-to-stand remained consistent across normalization methods ( P < 0.05). Our findings highlight the efficacy of 1-min sit-to-stand in distinguishing age-related functional capacity over the other sit-to-stand tests, especially in women., Competing Interests: Conflict of Interest: None. Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Individuals with Parkinson's disease (PD) present respiratory dysfunctions, mainly due to decreased chest wall expansion, which worsens with the course of the disease. These findings contribute to the restrictive respiratory pattern and the reduction in chest wall volume. According to literature, inspiratory muscle thixotropic conditioning maneuvers may improve lung volumes in these patients. The study aimed to determine the after-effects of respiratory muscle thixotropic maneuvers on breathing patterns and chest wall volumes of PD. A crossover study was performed with twelve patients with PD (8 males; mean age 63.9±8.8 years, FVC%pred 89.7±13.9, FEV1%pred 91.2±15, FEV1/FVC%pred 83.7±5.7). Chest wall volumes were assessed using OEP during thixotropic maneuvers. Increases in EIVCW (mean of 126mL, p = 0.01) and EEVCW (mean of 150mL, p = 0.005) were observed after DITLC (deep inspiration from total lung capacity) due to increases in pulmonary (RCp) and abdominal (RCa) ribcage compartments. Changes in ICoTLC (inspiratory contraction from TLC) led to significant EIVCW (mean of 224mL, p = 0.001) and EEVCW (mean of 229mL, p = 0.02) increases that were mainly observed in the RCp. No significant changes were found when performing DERV (deep expiration from residual volume) and ICoRV (Inspiratory contraction from RV). Positive correlations were also observed between the degree of inspiratory contraction during ICoTLC and EEVRCp (rho = 0.613, p = 0.03) and EIVRCp (rho = 0.697, p = 0.01) changes. Thixotropy conditioning of inspiratory muscles at an inflated chest wall volume increases EIVCW and EEVCW in the ten subsequent breaths in PD patients. These maneuvers are easy to perform, free of equipment, low-cost, and may help patients improve chest wall volumes during rehabilitation., Competing Interests: The authors have declared that no competing interests exist.

Background: Congenital Generalized Lipodystrophy (CGL) is an ultra-rare disease characterized by metabolic disorders. However, the evaluation of functional exercise capacity, cardiovascular (CV) response to exercise, and peripheral arterial disease (PAD) in CGL is scarce. Here we evaluated the performance and CV response to exercise and their association with PAD in CGL compared to healthy individuals., Methods: Twelve CGL and 12 healthy subjects matched for age and gender were included. Functional exercise capacity, CV response, and PAD were measured using the six-minute walk test (6MWT) and ankle-brachial index (ABI), respectively., Results: At baseline, CGL subjects showed reduced predicted walked distance (6MWD) (p = 0.009) and increased heart rate (HR), systolic (SBP), and diastolic (DBP) pressures compared to healthy subjects (p < 0.05). Most CGL subjects presented normal ABI values (1.0 ≤ ABI ≤ 1.4). Only 25% (n = 3) had ABI ≤ 0.9. CGL subjects did not present changes in ABI and blood pressure 12 months after metreleptin (MLP) replacement, but they walked a greater 6MWD than baseline (p = 0.04). Further, 6MWD and right ABI measurements were positively correlated in CGL subjects (p = 0.03). Right ABI negatively correlated with glucose, triglycerides, and VLDL-c (p < 0.05)., Conclusions: We observed that CGL subjects had lower functional exercise capacity and higher cardiovascular effort for similar performance of 6MWT, suggesting that strategies for decreasing exercise effort in this population should be essential. Furthermore, better physical performance was associated with high ABI in CGL. Additional studies are needed to clarify leptin's role in preserving functional exercise capacity in CGL., (© 2022. The Author(s).)

Muscle fatigue is a complex phenomenon enclosing various mechanisms. Despite technological advances, these mechanisms are still not fully understood in vivo. Here, simultaneous measurements of pressure, volume, and ribcage inspiratory muscle activity were performed non-invasively during fatigue (inspiratory threshold valve set at 70% of maximal inspiratory pressure) and recovery to verify if inspiratory ribcage muscle fatigue (1) leads to slowing of contraction and relaxation properties of ribcage muscles and (2) alters median frequency and high-to-low frequency ratio (H/L). During the fatigue protocol, sternocleidomastoid showed the fastest decrease in median frequency and slowest decrease in H/L. Fatigue was also characterized by a reduction in the relative power of the high-frequency and increase of the low-frequency. During recovery, changes in mechanical power were due to changes in shortening velocity with long-lasting reduction in pressure generation, and slowing of relaxation [i.e., tau (τ), half-relaxation time (½RT), and maximum relaxation rate (MRR)] was observed with no significant changes in contractile properties. Recovery of median frequency was faster than H/L, and relaxation rates correlated with shortening velocity and mechanical power of inspiratory ribcage muscles; however, with different time courses. Time constant of the inspiratory ribcage muscles during fatigue and recovery is not uniform (i.e., different inspiratory muscles may have different underlying mechanisms of fatigue), and MRR, ½RT, and τ are not only useful predictors of inspiratory ribcage muscle recovery but may also share common underlying mechanisms with shortening velocity.

Background and Purpose: Sniff nasal inspiratory pressure (SNIP) is a voluntary inspiratory maneuver measured through a plug occluding one nostril. The investigation of the number of maneuvers necessary to reach the highest peak of SNIP in pediatric populations has been inconsistent. Thus, this study aimed to assess the reliability of SNIP in healthy children aged 6 to 11 years according to sex and age group, and to determine the optimal number of SNIP maneuvers for this age group., Methods: This cross-sectional study included healthy children with normal pulmonary function. We performed 12 to 20 SNIP maneuvers, with a 30 s rest between each maneuver. The reliability was tested using the intraclass correlation coefficient (ICC), standard error of measurement (SEM), minimal detectable change (MDC), and Bland-Altman analysis for agreement., Results: A total of 121 healthy children (62 girls [51%]) were included in this study. The ICC and corresponding confidence interval (CI) between the highest measure and the first reproducible maneuver were 0.752 (0.656-0.824), SEM = 10.37 cmH2O, and MDC = 28.74 cmH2O. For children aged 6 to 7 years, the ICC was 0.669 (0.427-0.822), SEM = 10.76 cmH2O and MDC = 29.82 cmH2O; for children aged 8 to 11 years, the ICC was 0.774 (0.662-0.852), SEM = 9.74 cmH2O, and MDC = 26.05 cmH2O. For girls, the ICC was 0.817 (0.706-0.889), SEM = 9.40 cmH2O and MDC = 26.05 cmH2O; for boys, the ICC was 0.671 (0.487-0.798), SEM = 11.51 cmH2O, and MDC = 31.90 cmH2O. Approximately 80% of the total sample reached the highest SNIP before the 10th maneuver., Conclusions: SNIP demonstrated moderate reliability between the maneuvers in children aged 6 to 11 years; older children and girls reached the SNIP peak faster. Finally, results indicated that 12 maneuvers were sufficient for healthy children aged 6 to 11 years to achieve the highest SNIP peak., Competing Interests: The authors have declared that no competing interests exist.

Objective: To determine reference values of sniff nasal inspiratory pressure (SNIP) in healthy children., Methods: This cross-sectional observational study included healthy children aged 6 to 11 years of both sexes. The volunteers underwent a pulmonary function test to rule out respiratory disorders. Respiratory muscle strength was measured using maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP), respectively, with MIP close to functional residual capacity (FRC) and residual volume, while MEP to total lung capacity. SNIP was performed through the nostril contralateral to the occlusion, close to FRC. Two 6-minute walk tests were performed to assess functional exercise capacity., Results: The sample comprised 121 healthy children (62 girls); 54% presented body mass index (BMI) percentile less than 85th and 46% more than equal to 85th percentile, higher than expected. SNIP values were similar between sexes (91.1 ± 21.0 cmH 2 O in girls and 87.7 ± 19.4 cmH 2 O in boys; P = .36) as well as the means of lower limits of normal (56.88 cmH 2 O for girls and 56.66 cmH 2 O for boys). Girls presented SNIP measurements larger than those of the MIP of FRC (P < .0001), while boys presented similar mean values for both. We found a positive correlation between SNIP and BMI percentile (r = .25, P = .04) in girls, unlike boys, in whom no correlation was observed., Conclusions: SNIP reference values and mean lower limits of normal were defined for healthy children aged 6 to 11. BMI percentile seems to positively influence the inspiratory muscle strength measured by SNIP in girls only., (© 2019 Wiley Periodicals, Inc.)

Aims: To assess cardiac autonomic control and its association with submaximal exercise measured using the 6-minute walk test (6MWT) in subjects with type-2 diabetes mellitus (DM2)., Methods: Cardiac autonomic control was assessed using Ewing's tests and heart rate variability (HRV) in DM2 volunteers (DG, n = 22) and sex-, age- and body mass index-matched non-diabetic controls (CG, n = 22) before, during and after 6MWT., Results: Before the 6MWT, DG presented lower HRV represented by reduced SDNN [median 28.9 ms 2 (IQR:18.6-35.4) vs. 45.1 (IQR:39.2-62.67), p < 0.001] and Total Power [median 785 ms 2 (IQR:256-1264) vs. 1757 ms 2 (IQR:1006-2912), p = 0.004]. Exercise capacity was reduced in DG [maximal predicted distance (%) = 88.4 ± 6.4 vs. 95.2 ± 11.0%, p = 0.018]. DG demonstrated lower global HRV during recovery and lower parasympathetic drive, represented by reduced RMSSD, during all phases of the 6MWT. Moreover, supine HR (r = -0.32), HR orthostatism (ρ = -0.40), SDNN pre-6MWT (ρ = 0.39), TP pre-6MWT (ρ = 0.38), Valsalva ratio (ρ = 0.39) and 30:15 ratio (ρ = 0.38) were all correlated with maximal walked distance., Conclusions: DM2 subjects presented abnormal HRV during and after submaximal exercise. Furthermore, autonomic control impairment in orthostatism, represented by lower global HRV (SDNN, Total power) and lower Ewing's indexes (Valsalva and 30:15 ratios), was associated with lower exercise capacity., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Aim: To assess thoracoabdominal asynchrony (TAA) and the presence of paradoxical motion in middle stage amyotrophic lateral sclerosis (ALS) and its relationships with chest wall tidal volume (V T,CW ), breathing pattern and cough peak flow (CPF)., Methods: Phase angle (θ) between upper (RCp) and lower ribcage (RCa) and abdomen (AB), as well as percentage of inspiratory time for the lower ribcage (IP RCa ) and abdomen (IP AB ) moving in opposite directions were quantified using optoelectronic plethysmography in 12 ALS patients during quiet breathing and coughing. Paradoxical motion of the compartments was based on threshold values of θ and IP, obtained in twelve age and sex matched healthy persons., Results: During quiet breathing, significantly higher RCa and AB θ (p < .05), IP RCa (p = 0.001) and IP AB (p < 0.05) were observed in ALS patients as compared to controls. In ALS patients, correlations between RCa and AB θ with forced vital capacity (FVC) (r=-0.773, p < 0.01), vital capacity (r=-0.663, p < 0.05) and inspiratory capacity (IC) (r=-0.754, p < 0.01), as well as between RCp and RCa θ with FVC (r=-0.608, p < 0.05) and CPF (r=-0.601, p < 0.05) were found. During coughing, correlations between RCp and AB θ with CPF (r=-0.590, p < 0.05), IC (r=-0.748, p < 0.01) and V T,CW (r=-0.608, p < 0.05), as well as between RCa and AB θ with CPF (r=-0.670, p < 0.05), IC (r=-0.713, p < 0.05) and peak expiratory flow (r=-0.727, p < 0.05) were also observed in ALS patients. ALS patients with paradoxical motion presented lower vital capacity and FVC %pred (p < 0.05) compared to those without paradoxical motion., Conclusions: Middle stage ALS patients exhibit TAA and paradoxical motion during quiet spontaneous breathing and coughing. In addition, diaphragmatic weakness (i.e. decrease in excursion of the RCa and AB compartments) was observed earlier in the lower ribcage rather than the abdominal compartment in this population., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Patients with Parkinson's disease often exhibit respiratory disorders and there are no Respiratory Therapy protocols which are suggested as interventions in Parkinson's patients. The aim of this study is to evaluate the effects of Breathing-Stacking (BS) and incentive spirometer (IS) techniques in volume variations of the chest wall in patients with Parkinson's Disease (PD). 14 patients with mild-moderate PD were included in this randomized cross-over study. Volume variations of the chest wall were assessed before, immediately after, then 15 and 30 min after BS and IS performance by optoelectronic plethysmography. Tidal volume (VT) and minute ventilation (MV) significantly increased after BS and IS techniques (p < 0.05). There was greater involvement of pulmonary and abdominal compartments after IS. The results suggest that these re-expansion techniques can be performed to immediately improve volume., (Copyright © 2018 Elsevier B.V. All rights reserved.)

This study aimed to better understand how subjects with stable asthma and without exercise-induced bronchoconstriction respond to mild exercise. Breathing pattern, chest wall compartmental and operational volumes, and thoracoabdominal asynchrony were assessed in 11 stable asthmatic subjects and 10 healthy subjects at rest and during exercise in a cycle-ergometer through optoelectronic plethysmography. Dyspnea and sensation of leg effort were assessed through Borg scale. During exercise, with similar minute ventilation, a significant lower chest wall tidal volume ( p = 0.003) as well as a higher respiratory rate ( p < 0.05) and rapid shallow breathing ( p < 0.05) were observed in asthmatic when compared to healthy subjects. Asthmatic subjects exhibited a significantly lower inspiratory ( p < 0.05) and expiratory times ( p < 0.05). Intergroup analysis found a significant higher end-expiratory chest wall volume in asthmatic subjects, mainly due to a significant increase in volume of the pulmonary ribcage (RCp; 170 ml, p = 0.002), indicating dynamic hyperinflation (DH). Dyspnea and sensation of leg effort were both significantly greater ( p < 0.0001) in asthmatic when compared to healthy subjects. In addition to a higher thoracoabdominal asynchrony found between RCp and abdominal (AB) ( p < 0.005) compartments in asthmatic subjects, post-inspiratory action of the inspiratory ribcage and diaphragm muscles were observed through the higher expiratory paradox time of both RCp ( p < 0.0001) and AB ( p = 0.0002), respectively. Our data suggest that a different breathing pattern is adopted by asthmatic subjects without exercise-induced bronchoconstriction during mild exercise and that this feature, associated with DH and thoracoabdominal asynchrony, contributes significantly to exercise limitation.

The relaxation rates and contractile properties of inspiratory muscles are altered with inspiratory muscle weakness and fatigue. This fact plays an important role in neuromuscular disorders patients and had never been extensively studied in amyotrophic lateral sclerosis (ALS). In this cross-sectional study, these parameters were investigated non-invasively through nasal inspiratory sniff pressure test (SNIP) in 39 middle stage spinal onset ALS subjects and compared with 39 healthy controls. ALS patients were also divided into three subgroups according to a decline in their percentage of predicted forced vital capacity (FVC %pred ) as well as a decline in the ALS functional rating scale score and its respiratory subscore (R-subscore) in order to determine the best parameter linked to early respiratory muscle weakness. When compared with healthy subjects, middle stage ALS subjects exhibited a significantly lower ( p  < 0.0001) maximum relaxation rate and maximum rate of pressure development (MRPD), as well as a significantly higher ( p  < 0.0001) tau (τ), contraction time, and half-relaxation time. The results from receiver operating characteristic curves showed that MRPD (AUC 0.735, p  < 0.001) and FVC %pred (AUC 0.749, p  = 0.009) were the best discriminator parameters between ALS patients with ≤30 and >30 points in the ALS functional rating scale. In addition, 1/2RT (AUC 0.720, p  = 0.01), FVC %pred (AUC 0.700, p  = 0.03), τ (AUC 0.824, p  < 0.0001), and MRPD (AUC 0.721, p  = 0.01) were the parameters more sensitive in detecting a fall of three points in the R-subscore. On the other hand, MRPD (AUC 0.781, p  < 0.001), τ (AUC 0.794, p  = 0.0001), and percentage of predicted of SNIP (AUC 0.769, p  = 0.002) were the parameters able to detect a fall in 30% of the FVC %pred in middle stage ALS patients. The contractile properties and relaxation rates of the diaphragm are altered in middle stage spinal onset ALS when compared with healthy subjects. These parameters are able to discriminate between those middle stage ALS subjects with early decline in inspiratory muscle function and those who not.

Objectives: To assess the acute effects of air stacking on cough peak flow (CPF) and chest wall compartmental volumes of persons with amyotrophic lateral sclerosis (ALS) versus healthy subjects positioned at 45° body inclination., Design: Cross-sectional study with a matched-pair design., Setting: University hospital., Participants: Persons (N=24) with ALS (n=12) and age-matched healthy subjects (n=12)., Main Outcomes Measures: CPF, chest wall compartmental inspiratory capacity, chest wall vital capacity, chest wall tidal volume and operational volumes, breathing pattern, and percentage of contribution of the compartments to the inspired volume were measured by optoelectronic plethysmography., Results: Compared with healthy subjects, significantly lower CPF (P=.007), chest wall compartmental inspiratory capacity (P<.001), chest wall vital capacity (P<.001), and chest wall tidal volume (P<.001) were found in subjects with ALS. Immediately after air stacking, CPF (P<.001) and chest wall compartmental inspiratory capacity (P<.001) significantly increased in both groups, with values returning to basal only in healthy subjects. After air stacking, the abdominal compartment (P=.004) was determined to be responsible for the inspired volume in subjects with ALS. Significantly higher chest wall vital capacity (P=.05) was observed in subjects with ALS 5 minutes after air stacking, with the rib cage compartment (P=.049) being responsible for volume change. No differences were found in chest wall vital capacity and compartmental volumes of healthy subjects. Chest wall tidal volume (P<.001) significantly increased during the protocol in the healthy subjects, mainly because of end-inspiratory (P<.001) and abdominal volumes (P=.008). No significant differences were observed in percentage of contribution of the compartments to the inspired volume and end-expiratory volume of both groups. No significant differences were found in chest wall tidal volume, operational volume, and breathing pattern in persons with ALS., Conclusions: Air stacking is effective in increasing CPF, chest wall compartmental inspiratory capacity, and chest wall vital capacity of persons with ALS with no hyperinflation. Differences in compartmental volume contributions are probably because of lung and chest wall physiological changes., (Copyright © 2016 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

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.

Background: Exercise is an important part of chronic obstructive pulmonary disease (COPD) treatment. However, it is not know about the minimum effective time of physical training that could beneficially modify the cardiac autonomic modulation (CAM) and exercise capacity in these patients., Aim: To contrast the potential effects of a physical training program (PTP), for 6 versus 12 weeks, on CAM by linear and nonlinear heart rate variability (HRV) indices and exercise capacity in COPD patients., Design: Prospective randomized controlled trial., Setting: Outpatient pulmonary rehabilitation., Population: Twenty moderate-to-severe COPD patients were randomly assigned to either a training group (N.=10) or a control group (N.=10)., Methods: HRV at rest and during submaximal test was determined by linear (rMSSD and SDNN) and non-linear indices (SD1, SD2 and sample entropy [SE]). In addition, key responses were obtained during cardiopulmonary exercise testing (CPET), the walking distance (WD) during the six minute walking test and submaximal constant speed testing (CST). PTP consisted of 30 minutes of aerobic exercise training on a treadmill, 3 times per week at 70% of CPET peak speed rate. Patients were evaluated on baseline, 6 and 12 weeks., Results: Significant improvements in HRV indices, WD, as well as, other physiological responses were observed after 6 weeks of the PTP and maintained until 12 weeks (P<0.05). However, after 12 weeks, the SD1 index demonstrated an additional improvement compared with 6 weeks (P<0.05). Peak oxygen uptake and dioxide carbon production improved only after 12 weeks (P<0.05). Interestingly, the 6th week-baseline delta (6th week-baseline) of WD, SDNN and SE were significantly higher than 12th week-6th week delta (P<0.05)., Conclusion: These results indicate that beneficial changes on cardiac autonomic modulation in conjunction with improvement in submaximal functional capacity occur in the first 6 weeks of PTP in moderate to severe COPD., Clinical Rehabilitation Impact: Short-term rehabilitation (6 weeks) is an effective sufficient time to beneficially modify important outcomes as cardiac modulation and exercise capacity in COPD patients.

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.

Background: Cystic fibrosis (CF) is a multisystem disease that affects the cardiorespiratory system and the cardiac autonomic control may be altered at rest and after a submaximal exercise., Objective: To assess the cardiac autonomic control by heart rate variability (HRV) analysis before and after a 6-minute walk test (6MWT)., Method: Thirteen children diagnosed with Cystic Fibrosis (CFG) aged 12±2.7 years (FEV1/FVC: 0.83±0.11, FEV1: 71.4±21 %pred) and 12 healthy children (control group-CG) aged 11.4±2.4 years (FEV1/FVC: 0.91±0.12, FEV1: 91.6±17.4 %pred) were included in the study. HRV was evaluated prior to and immediately after the 6MWT and the heart rate recovery assessed on the first and second minute after test., Results: Prior to exercise, CF patients presented higher values for the variables LF(nu) (53.2±15.0 vs. 32.8±7.9, p=0.0003) and LF/HF (1.25±0.72 vs. 0.49±0.18, p<0.006) as well as lower values of HF(%) (25.4±18.4 vs. 53±9.6, p=0.002) and HF(nu) (47.3±14.7 vs. 68.3±8.7, p0.001) when compared to CG. After the 6MWT, both groups demonstrated HRV recovery to baseline levels; however, the differences between CFG and CG were maintained for the variables LF(ms 2) (846.7±754.8 vs. 345.6±197.2, p=0.02); LF(%) (35.4±8.1 vs. 25.9±6.2, p=0.002); LF(nu) (60.0±16.3 vs. 34.9±8.7, p<0.0001); HF(%) (27.4±13.7 vs. 48.1±6.3, p=0.0003); HF(nu) (40.4±15.8 vs. 65.6±8.2, p=0.0003) and LF/HF (1.9±1.7 vs. 0.5±0.2, p=0.0001)., Conclusions: Children with cystic fibrosis exhibited predominance of sympathetic activity at rest that persisted after the 6-minute walking test when compared to the control group.

Background: Cardiac involvement is common in myotonic dystrophy (MD) patients. Heart rate variability (HRV) is a simple and reliable technique that can be useful for studying the influence of the autonomic nervous system on the heart., Objective: Study heart rate variability in patients with type 1 MD., Methods: We studied HRV during 5-minute recordings in MD patients and in a healthy control group. We analyzed frequency domains (LF and HF) in normalized units (nu) and sympathovagal balance, in the sitting and supine position., Results: Seventeen patients (10 men and 7 women) and seventeen matched healthy individuals (10 men and 7 women) were studied. Sympathetic and parasympathetic modulations of the heart increased in male MD patients from supine to sitting position in 19% of LFnu and the LF/HF ratio rose by 42.3%. In the sitting position, male MD patients exhibited significantly higher sympathovagal balances in 50.9% compared to healthy control individuals. HRV was influenced by both gender and disease. Gender influenced LFnu in the supine position while the LF/HF ratio and HFnu were affected in both positions. Post hoc analyses showed that gender significantly impacts MD patients and healthy individuals in different ways (p < 0.01). The low frequency domain in the sitting position (LFnu) was significantly influenced by the disease., Conclusion: The results of this study suggest that the sympathetic drive in middle-aged male MD patients who are not severely impaired and present moderate disease duration seems to be greater than in healthy matched individuals.

Introduction: Studies on quality of life in myotonic dystrophy (MD) are scarce and the relationship between respiratory muscle strength and health-related quality of life (HRQoL) has yet to be determined. The present study aims to investigate respiratory muscle strength and HRQoL and their relationship in MD patients., Methods: Twenty-three patients (13 men, aged 40 ± 16 years) with MD were evaluated for pulmonary function, maximal inspiratory and expiratory pressure (MIP and MEP, respectively), sniff nasal inspiratory pressure (SNIP) and HRQoL using the Short Form (SF-36) quality of life questionnaire., Results: Respiratory muscle strength values were 71 ± 20 cmH₂O (64 % predicted), 76 ± 32 cmH₂O (70 % predicted), and 79 ± 28 cmH₂O (80 % predicted) for MEP, MIP, and SNIP respectively. Significant differences were found in the SF-36 domains of physical functioning 58.7 ± 31.4 vs. 84.5 ± 23 (p < 0.01, 95 % CI = 1.6-39.9) and physical problems 43.4 ± 35.2 vs. 81.2 ± 34 (p < 0.001, 95 % CI = 19.4-6.1) when compared with the reference values. According to single linear regression analysis, MIP explains 29 % of the variance in physical functioning, 18 % of physical problems and 20 % of vitality., Conclusions: Individuals with MD have reduced expiratory muscle strength. HRQoL may be more impaired in some physical domains, which might be influenced by variations in inspiratory muscle strength.

The inelastic bandage is an adaptation of the manual lymphatic drainage, which substitutes the circular movements of the fingers. A patient with lymphoedema underwent 20 sessions using the modified lymphatic drainage technique. Perimetric measurements were taken before and after each session, and volumetric measurements at the first, 10th, and 20th sessions. Limb circumference was significantly reduced at three points on the perimeter (10, 15 and 25 cm): 2.5 cm (7.3%), 2.5 cm (6.5%) and 1.5 cm (5%), respectively. Volumetry decrease of 26.4% from initial limb volume was observed. The use of the inelastic bandage proved to be an effective adaptation when compared with the results using other techniques described.

Objective: The benefits of a domiciliary program of pulmonary rehabilitation for patients with severe to very severe chronic obstructive pulmonary disease (COPD) are uncertain. We aimed to assess the short- and medium-term efficacy of such a program in this clinical setting., Patients and Methods: Patients with severe COPD (stages III-IV, classification of the Global Initiative for Chronic Obstructive Lung Disease) and incapacitating dyspnea (scores 3-5, Medical Research Council [MRC] scale) were randomized to a control or domiciliary rehabilitation group. The 9-week supervised pulmonary rehabilitation program included educational sessions, respiratory physiotherapy, and muscle training in weekly sessions in the patient's home. We assessed the following variables at baseline, 9 weeks, and 6 months: lung function, exercise tolerance (3-minute walk test), dyspnea (MRC score), and health-related quality of life with the Chronic Respiratory Questionnaire (CRQ)., Results: Thirty-eight patients with a mean (SD) age of 68 (6) years were enrolled. The mean MRC score was 4 (0.8) and mean forced expiratory volume in 1 second was 29% of reference. Twenty-nine patients completed the study (6 months). Distance covered on the walk test increased significantly in the rehabilitation group (P=.001) and the difference was maintained at 6 months. Dyspnea also improved significantly with rehabilitation (P<.05), but the reduction was not evident at 6 months. Statistically significant improvements in symptoms related to 2 CRQ domains were detected between baseline and 9 weeks: dyspnea (3.1 [0.8] vs 3.6 [0.7]; P=.02) and fatigue (3.7 [0.8] vs 4.2 [0.9]; P=.002). A clinically relevant but not statistically significant change in mastery over disease was detected (from 4.3 to 4.9). All improvements were maintained at 6 months., Conclusions: Home-based pulmonary rehabilitation for patients with severe to very severe COPD and severe functional incapacity leads to improvements in exercise tolerance and health-related quality of life that are maintained at 6 months.

Objective: To study lung function abnormalities and health-related quality of life (HRQL) in a group of patients diagnosed with generalized myasthenia gravis, and to analyze the relationship between these 2 sets of variables., Patients and Methods: In a prospective study of 20 patients diagnosed with generalized myasthenia gravis, the following parameters were measured: spirometry, static lung volumes, breathing pattern, maximal respiratory pressures, and maximum voluntary ventilation. HRQL was assessed using the 36-item short form general health questionnaire (SF-36)., Results: The mean (SD) age of the patients was 64 (11) years. Patients were classified into 2 groups depending on whether they had IIa (12 patients) or IIb (8 patients) type disease. A small decrease in total lung capacity (86%) and slight reductions in maximal inspiratory pressure (88%) and maximum voluntary ventilation (63% in group IIb) were observed. The HRQL domains most affected were those related to physical activity and self-perceived health status in all groups, although women were more affected. The scores relating to vitality and physical activity were found to be significantly associated with forced vital capacity and lung volumes. Tidal volume was associated with maximal inspiratory and expiratory pressures, inspiratory capacity, and maximum voluntary ventilation. The respiratory rate to tidal volume ratio was inversely associated with the first three of these variables., Conclusions: A very slight restrictive pattern and a reduction in inspiratory muscle strength were observed. The HRQL domains most affected were those related to physical activity and the patients' self-perceived health status. The weakness of the respiratory muscles contributes to the abnormalities observed in lung function and to the deterioration of health-related quality of life.