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.

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 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.

Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.

Keywords: Cystic fibrosis; GRADE approach; Cystic fibrosis/drug treatment; Clinical practice guide.