Objective: The aim of this study was to examine the parenchymal and extracellular matrix remodeling process in two histologic patterns-nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP)-in cases of idiopathic and sclerosis/systemic sclerosis (SSc)-associated interstitial pneumonia. Methods: We examined 15 cases of idiopathic NSIP, 10 cases of idiopathic UIP, 5 cases of SSc-UIP and 9 cases of SSc-NSIP. In the lung parenchyma, epithelial cells, endothelial cells and myofibroblasts were evaluated by immunohistochemical staining, whereas histochemical staining was used in order to evaluate collagen/elastic fibers in the extracellular matrix. Results: The percentage of surfactant protein A-positive epithelial cells was significantly greater in idiopathic NSIP than in SSc-NSIP, as well as being greater in idiopathic UIP than in SSc-UIP. Idiopathic NSIP and idiopathic UIP presented significantly higher immunoexpression of alpha smooth muscle actin in myofibroblasts than did SSc-NSIP and SSc-UIP. The percentage of CD34 endothelial cells in the pulmonary microvasculature was significant lower in idiopathic UIP than in SSc-UIP. The density of collagen fibers was significantly greater in idiopathic NSIP and idiopathic UIP than in SSc-NSIP and UIP. In contrast, the elastic fiber density was significantly lower in idiopathic UIP than in SSc-UIP. Conclusions: Increased collagen synthesis, destruction of elastic fibers, high myofibroblast proliferation and poor microvascularization might represent a remodeling process found in idiopathic interstitial pneumonia, whereas the reverse might represent a repair process in SSc-associated interstitial pneumonia.

Keywords: Epithelial cells; Neovascularization, pathologic; Collagen; Elastin; Idiopathic interstitial pneumonias; Scleroderma, systemic.

Objective: Respiratory complications are the main causes of morbidity and mortality in patients with neuromuscular disease (NMD). The objectives of this study were to determine the effects that routine daily home air-stacking maneuvers have on pulmonary function in patients with spinal muscular atrophy (SMA) and in patients with congenital muscular dystrophy (CMD), as well as to identify associations between spinal deformities and the effects of the maneuvers. Methods: Eighteen NMD patients (ten with CMD and eight with SMA) were submitted to routine daily air-stacking maneuvers at home with manual resuscitators for four to six months, undergoing pulmonary function tests before and after that period. The pulmonary function tests included measurements of FVC; PEF; maximum insufflation capacity (MIC); and assisted and unassisted peak cough flow (APCF and UPCF, respectively) with insufflations. Results: After the use of home air-stacking maneuvers, there were improvements in the APCF and UPCF. In the patients without scoliosis, there was also a significant increase in FVC. When comparing patients with and without scoliosis, the increases in APCF and UPCF were more pronounced in those without scoliosis. Conclusions: Routine daily air-stacking maneuvers with a manual resuscitator appear to increase UPCF and APCF in patients with NMD, especially in those without scoliosis.

Keywords: Neuromuscular diseases; Cough; Respiratory function tests; Respiratory therapy.

Background: Gastroplasty has been increasingly used in the treatment of morbidly obese patients, who typically present pronounced alterations in lung volume and capacity. Objective: To evaluate post-gastroplasty lung volume, lung capacity, respiratory muscle strength and respiratory pattern, as well as any postoperative pulmonary complications. Method: 21 patients (3 of them men), with an average age of 39 ± 9.7 years and an average body mass index of 50.4 kg/m2, all candidates for gastroplasty, were evaluated during the preoperative period and again on the first, third and fifth postoperative days. Tidal volume, vital capacity, minute volume, maximal expiratory pressure and maximal inspiratory pressure, as well as chest and waist circumferences, were measured. Postoperative pulmonary complications and mortality were assessed. Results: On the first and third postoperative days, respectively, there were drops of 47% and 30.5% in vital capacity, 18% and 12.5% in minute volume, 28% and 21% in tidal volume, 47% and 32% in the diaphragmatic index, 51% and 26% in maximal inspiratory pressure, and 39.5% and 26% in maximal expiratory pressure (p < 0.05). On the fifth postoperative day, all variables analyzed were higher than on the first postoperative day, indicating a linear increase, with only tidal volume, minute volume diaphragmatic index returning to preoperative values. The incidence of postoperative pulmonary complications was 4.7%, and there were no deaths. Conclusion: Patients submitted to gastroplasty present reduced pulmonary function, following a pattern quite similar to that previously observed following other types of upper abdominal surgery.

Keywords: Key words: Gastroplasty. Obesity. Respiratory function tests. Postoperative care.

Objective: To evaluate inspiratory volume in patients undergoing cardiac surgery and to determine the effects that incentive spirometry (IS) and the breath stacking (BS) technique have on the recovery of FVC in such patients. Methods: A prospective, controlled, randomized clinical trial involving 35 patients undergoing cardiac surgery at the Hospital de Força Aérea do Galeão (HFAG, Galeão Air Force Hospital), in the city of Rio de Janeiro, Brazil. The patients, all of whom performed mobilization and cough procedures, were randomly divided into three groups: exercise control (EC), performing only the abovementioned procedures; IS, performing the abovementioned procedures and instructed to take long breaths using an incentive spirometer; and BS, performing the abovementioned procedures, together with successive inspiratory efforts using a facial mask coupled to a unidirectional valve. Forced spirometry was carried out in the preoperative period and on postoperative days 1 to 5. During the maneuvers, inspiratory volume was measured in the IS and BS groups. Results: On postoperative day 1, FVC significantly decreased in all groups (EC: 87.1 vs. 32.0%; IS: 75.3 vs. 29.5%; and BS: 81.9 vs. 33.2%; p < 0.001 for all), as did inspiratory volume in the IS and BS groups (2.29 vs. 0.82 L; and 2.56 vs. 1.34 L, respectively; p < 0.001 for both). Between postoperative days 1 and 5, FVC partially normalized in all groups (EC: 32.0 vs. 51.3%; IS: 29.5 vs. 46.7%; and BS: 33.3 vs. 54.3%; p < 0.001 for all). During the postoperative period, inspiratory volume was significantly higher in the BS group than in the IS group. Conclusions: The three protocols were equivalent concerning the recovery of FVC on the first five postoperative days. When compared with IS, the BS technique promoted higher inspiratory volumes in this sample of postoperative cardiac patients. (ClinicalTrials.gov Identifier:NCT01074957 [http://www.clinicaltrials.gov/])

Keywords: Postoperative complications; Thoracic surgery; Physical therapy modalities.

Objective: To evaluate cough efficiency using two manually-assisted cough techniques. Methods: We selected 28 patients with Duchenne muscular dystrophy. The patients were receiving noninvasive nocturnal ventilatory support and presented FVC values < 60% of predicted. Peak cough flow (PCF) was measured, with the patient seated, at four time points: at baseline, during a spontaneous maximal expiratory effort (MEE); during an MEE while receiving chest compression; during an MEE after air stacking with a manual resuscitation bag; and during an MEE with air stacking and compression (combined technique). The last three measurements were conducted in random order. The results were compared using Pearson's correlation test and ANOVA with repeated measures, followed by Tukey's post-hoc test (p < 0.05). Results: The mean age of the patients was 20 ± 4 years, and the mean FVC was 29 ± 12%. Mean PCF at baseline, with chest compression, after air stacking and with the use of the combined technique was 171 ± 67, 231 ± 81, 225 ± 80, and 292 ± 86 L/min, respectively. The results obtained with the use of the combined technique were significantly better than were those obtained with the use of either technique alone (F[3.69] = 67.07; p < 0.001). Conclusions: Both chest compression and air stacking techniques were efficient in increasing PCF. However, the combination of these two techniques had a significant additional effect (p < 0.0001).

Keywords: Muscular dystrophy, Duchenne; Intermittent positive-pressure ventilation; Cough; Insufflation;Peak expiratory flow rate.

Objective: To evaluate mouthpiece ventilation (MPV) in patients with Duchenne muscular dystrophy (DMD) who are noncompliant with noninvasive ventilation (NIV). Methods: We evaluated four young patients with DMD who had previously refused to undergo NIV. Each patient was reassessed and encouraged to try MPV. Results: The four patients tolerated MPV well and were compliant with NIV at home. MPV proved to be preferable and more comfortable than NIV with any other type of interface. Two of the patients required overnight NIV and eventually agreed to use a nasal mask during the night. Conclusions: The advantages of MPV over other types of NIV include fewer speech problems, better appearance, and less impact on the patient, eliminating the risk of skin breakdown, gastric distension, conjunctivitis, and claustrophobia. The use of a mouthpiece interface should be always considered in patients with DMD who need to start NIV, in order to promote a positive approach and a rapid acceptance of NIV. Using MPV during the daytime makes patients feel safe and more likely to use NIV at night. In addition, MPV increases treatment compliance for those who refuse to use other types of interfaces.

Keywords: Muscular dystrophy, Duchenne; Noninvasive ventilation; Patient compliance.

Objective: To compare TLC and RV values obtained by the single-breath helium dilution (SBHD) method with those obtained by whole-body plethysmography (WBP) in patients with normal lung function, patients with obstructive lung disease (OLD), and patients with restrictive lung disease (RLD), varying in severity, and to devise equations to estimate the SBHD results. Methods: This was a retrospective cross-sectional study involving 169 individuals, of whom 93 and 49 presented with OLD and RLD, respectively, the remaining 27 having normal lung function. All patients underwent spirometry and lung volume measurement by both methods. Results: TLC and RV were higher by WBP than by SBHD. The discrepancy between the methods was more pronounced in the OLD group, correlating with the severity of airflow obstruction. In the OLD group, the correlation coefficient of the comparison between the two methods was 0.57 and 0.56 for TLC and RV, respectively (p < 0.001 for both). We used regression equations, adjusted for the groups studied, in order to predict the WBP values of TLC and RV, using the corresponding SBHD values. It was possible to create regression equations to predict differences in TLC and RV between the two methods only for the OLD group. The TLC and RV equations were, respectively, ∆TLCWBP-SBHD in L = 5.264 − 0.060 × FEV1/FVC (r2 = 0.33; adjusted r2 = 0.32) and ∆RVWBP-SBHD in L = 4.862 − 0.055 × FEV1/FVC (r2 = 0.31; adjusted r2 = 0.30). Conclusions: The correction of TLC and RV results obtained by SBHD can improve the accuracy of this method for assessing lung volumes in patients with OLD. However, additional studies are needed in order to validate these equations.

Keywords: Plethysmography, whole body; Total lung capacity; Residual volume.

Objective: To derive reference values for healthy white Brazilian adults who have never smoked and to compare the obtained values with reference values derived by Crapo and by Neder. Methods: Reference equations by quantile regressions were derived in 122 men and 122 women, non-obese, living in seven cities in Brazil. Age ranged from 21 to 92 years in women and from 25 to 88 years in men. Lung function tests were performed using SensorMedics automated body plethysmographies according ATS/ERS recommendations. Lower and upper limits were derived by specific equations for 5 and 95 percentiles. The results were compared to those suggested by Crapo in 1982, and Neder in 1999. Results: Median values for total lung capacity (TLC) were influenced only by stature in men, and by stature and age in women. Residual volume was influenced by age and stature in both genders. Weight was directly related to inspiratory capacity and inversely with functional residual capacity and expiratory reserve volume in both genders. A comparison of observed TLC data with values predicted by Neder equations showed significant lower values by the present data. Mean values were similar between data from present study and those derived by Crapo. Conclusion: New predicted values for lung volumes were obtained in a sample of white Brazilians. The values differ from those derived by Neder, but are similar to those derived by Crapo.

Keywords: Pulmonary volumes; Pulmonary function tests; Reference values; Pulmonary plethysmography

Objective: To evaluate the influence of chronic heart failure (CHF) on resting lung volumes in patients with COPD, i.e., inspiratory fraction-inspiratory capacity (IC)/TLC-and relative inspiratory reserve-[1 − (end-inspiratory lung volume/TLC)]. Methods: This was a prospective study involving 56 patients with COPD-24 (23 males/1 female) with COPD+CHF and 32 (28 males/4 females) with COPD only-who, after careful clinical stabilization, underwent spirometry (with forced and slow maneuvers) and whole-body plethysmography. Results: Although FEV1, as well as the FEV1/FVC and FEV1/slow vital capacity ratios, were higher in the COPD+CHF group than in the COPD group, all major "static" volumes-RV, functional residual capacity (FRC), and TLC-were lower in the former group (p < 0.05). There was a greater reduction in FRC than in RV, resulting in the expiratory reserve volume being lower in the COPD+CHF group than in the COPD group. There were relatively proportional reductions in FRC and TLC in the two groups; therefore, IC was also comparable. Consequently, the inspiratory fraction was higher in the COPD+CHF group than in the COPD group (0.42 ± 0.10 vs. 0.36 ± 0.10; p < 0.05). Although the tidal volume/IC ratio was higher in the COPD+CHF group, the relative inspiratory reserve was remarkably similar between the two groups (0.35 ± 0.09 vs. 0.44 ± 0.14; p < 0.05). Conclusions: Despite the restrictive effects of CHF, patients with COPD+CHF have relatively higher inspiratory limits (a greater inspiratory fraction). However, those patients use only a part of those limits, probably in order to avoid critical reductions in inspiratory reserve and increases in elastic recoil.

Keywords: Respiratory function tests; Pulmonary disease, chronic obstructive; Heart failure; Spirometry.

Objective: Many patients with proportional reductions in FVC and FEV1 on spirometry show no reduction in TLC. The aim of this study was to evaluate the role that measuring lung volumes and airway resistance plays in the correct classification of patients with a possible restrictive pattern on spirometry. Methods: This was a prospective study involving adults with reduced FVC and FEV1, as well as an FEV1/FV(C) ratio within the predicted range. Restrictive lung disease (RLD) was characterized by TLC below the 5th percentile, as determined by plethysmography. Obstructive lung disease (OLD) was characterized by high specific airway resistance, significant changes in post-bronchodilator FEV1, or an FEF25-75% < 50% of predicted, together with a high RV/TLC ratio. Nonspecific lung disease (NLD) was characterized by TLC within the predicted range and no obstruction. Combined lung disease (CLD) was characterized by reduced TLC and findings indicative of airflow obstruction. Clinical diagnoses were based on clinical suspicion, a respiratory questionnaire, and the review of tests of interest. Results: We included 300 patients in the study, of whom 108 (36%) were diagnosed with RLD. In addition, 120 (40%) and 72 (24%) were diagnosed with OLD/CLD and NLD, respectively. Among the latter, 24 (33%) were clinically diagnosed with OLD. In this sample, 151 patients (50.3%) were obese, and obesity was associated with all patterns of lung disease. Conclusions: Measuring lung volumes and airway resistance is often necessary in order to provide an appropriate characterization of the pattern of lung disease in patients presenting with a spirometry pattern suggestive of restriction. Airflow obstruction is common in such cases.

Keywords: Spirometry; Airway resistance; Lung volume measurements.

Objective: Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy in children, and children with DMD die prematurely because of respiratory failure. We sought to determine the efficacy and safety of yoga breathing exercises, as well as the effects of those exercises on respiratory function, in such children. Methods: This was a prospective open-label study of patients with a confirmed diagnosis of DMD, recruited from among those followed at the neurology outpatient clinic of a university hospital in the city of São Paulo, Brazil. Participants were taught how to perform hatha yoga breathing exercises and were instructed to perform the exercises three times a day for 10 months. Results: Of the 76 patients who entered the study, 35 dropped out and 15 were unable to perform the breathing exercises, 26 having therefore completed the study (mean age, 9.5  2.3 years; body mass index, 18.2  3.8 kg/m2). The yoga breathing exercises resulted in a significant increase in FVC (% of predicted: 82.3  18.6% at baseline vs. 90.3  22.5% at 10 months later; p = 0.02) and FEV1 (% of predicted: 83.8  16.6% at baseline vs. 90.1  17.4% at 10 months later; p = 0.04). Conclusions: Yoga breathing exercises can improve pulmonary function in patients with DMD.

Keywords: Respiratory therapy; Forced expiratory volume; Vital capacity; Muscular dystrophy, Duchenne; Complementary therapies.