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.

New scientific articles about tuberculosis (TB) are published daily worldwide. However, it is difficult for health care workers, overloaded with work, to stay abreast of the latest research findings and to discern which information can and should be used in their daily practice on assisting TB patients. The purpose of the III Brazilian Thoracic Association (BTA) Guidelines on TB is to critically review the most recent national and international scientific information on TB, presenting an updated text with the most current and useful tools against TB to health care workers in our country. The III BTA Guidelines on TB have been developed by the BTA Committee on TB and the TB Work Group, based on the text of the II BTA Guidelines on TB (2004). We reviewed the following databases: LILACS (SciELO) and PubMed (Medline). The level of evidence of the cited articles was determined, and 24 recommendations on TB have been evaluated, discussed by all of the members of the BTA Committee on TB and of the TB Work Group, and highlighted. The first version of the present Guidelines was posted on the BTA website and was available for public consultation for three weeks. Comments and critiques were evaluated. The level of scientific evidence of each reference was evaluated before its acceptance for use in the final text.

Keywords: Tuberculosis; Mycobacterium infections; Diagnosis; Tuberculosis, multidrug-resistant.

Objective: To evaluate the accuracy of determining specific IgA to HspX recombinant antigen in pleural fluid and serum samples for the diagnosis of pleural tuberculosis in patients with pleural effusion. Methods: This was a cross-sectional study. Serum and pleural fluid samples of patients with pleural effusion and suspected of having pleural tuberculosis were tested with indirect ELISA in order to determine the optical density of specific IgA to HspX. Results: We evaluated serum and pleural fluid samples from 132 patients: 97 diagnosed with pleural tuberculosis (study group) and 35 diagnosed with pleural effusion due to other causes (control group). The determination of IgA in pleural fluid satisfactorily discriminated between pleural tuberculosis patients and control patients. The sensitivity of the test in pleural fluid and in serum was 69% and 30%, respectively, whereas the specificity was 83% and 84%, respectively. Conclusions: Our data suggest that this test can be used in the diagnosis of pleural tuberculosis. Further studies, involving larger patient samples and different epidemiological scenarios, are warranted.

Keywords: Pleural effusion; Tuberculosis/diagnosis; Enzyme-linked immunosorbent assay.

Objective: To investigate dyspnea in individuals with Class II or III obesity and look for correlations among the respiratory data related to such individuals. Methods: This study involved 49 subjects with a body mass index >35 kg/m2, divided into two groups (those with dyspnea and those without). The baseline dyspnea index was evaluated, as were spirometry findings, maximal respiratory pressures and arterial blood gas analysis. Results: Of the 49 subjects evaluated, 37 reported dyspnea and 12 reported no dyspnea. The baseline dyspnea index differed between the two groups. The mean values were within the range of normality for all subjects and all parameters, except for the following: ratio of residual volume to total lung capacity; expiratory reserve volume; and the alveolar-arterial oxygen gradient. The subjects with dyspnea presented significantly lower values for expiratory reserve volume, maximal expiratory pressure and arterial pH. In all subjects, body mass index correlated significantly with the following: baseline dyspnea index; the residual volume/total lung capacity ratio; the forced expiratory volume in one second/forced vital capacity ratio; forced expiratory flow between 25% and 75% of forced vital capacity; arterial oxygen tension; the alveolar-arterial oxygen gradient; and arterial carbon dioxide tension. The baseline dyspnea index was found to correlate significantly with the following parameters: residual volume/total lung capacity ratio; expiratory reserve volume; arterial oxygen tension; the alveolar-arterial oxygen gradient; and arterial carbon dioxide tension. Conclusion:  Dyspnea is a common complaint in individuals with class II or III obesity. Such individuals present a pronounced reduction in expiratory reserve volume and an increase in the alveolar-arterial oxygen gradient. The correlations found suggest that obese individuals present dysfunction of the lower airways, and that obesity itself plays a role in the genesis of dyspnea.

Keywords: Obesity/complications; Dyspnea/epidemiology; Dyspnea/physiopathology; Respiratory function tests

Objective: To determine whether the guidelines put forth in the III Brazilian Consensus on Asthma Management are being applied in a population of asthma patients treated at a public hospital that is a referral center for asthma in the city of Porto Alegre, Brazil. Methods: All adult asthma patients who began their treatment between 1999 and 2002 were evaluated. The treatment given was classified as consistent or inconsistent with the Consensus guidelines. The clinical features of asthma and the frequency of treatment provided by a specialist were compared between the two groups (those receiving guideline-consistent treatment and those receiving guideline-inconsistent treatment). Results: The charts of 357 patients were evaluated. The study sample consisted of 106 males (29.9%) and 251 females (70.3%). The mean age was 41 years, and 33 (9.2%) of the patients were smokers. The treatment was considered inconsistent with the Consensus guidelines in 246 cases (70%). Of those 246, 174 (71%) had presented persistent asthma and were not treated with an inhaled corticosteroid. Normal forced expiratory volume in one second, being from 12 to 18 years of age, and having intermittent asthma were more frequently observed among the patients receiving guideline-consistent treatment (p < 0.01). No correlations were found between guideline-inconsistent treatment and being treated by a pulmonologist, severity of persistent asthma or number of emergency room visits. Conclusion: Most of the asthma patients treated at the public referral center in Porto Alegre did not receive treatment that was consistent with the Consensus guidelines. Undertreatment with inhaled corticosteroids was the principal source of that inconsistency.

Keywords: Asthma/therapy; Guidelines; Comparative study

Job's syndrome, or hyperimmunoglobulinemia E, is a rare immunodeficiency condition with no established etiology, characterized by recurrent lower and upper respiratory tract and skin infections associated with high levels of immunoglobulin E, eosinophilia, and a characteristic facial alteration. The authors report on a 22-year-old man who carried this disease complicated by pleural empyema and lung cysts, showing a good evolution.

Keywords: Job's syndrome. Respiratory tract infections. Recurrence.

Objective: To evaluate the management of asthma attacks in children up to five years of age treated in the pediatric emergency room of a hospital in the metropolitan area of the city of Recife, Brazil, as well as to determine whether the way in which asthma attacks are managed can influence the duration of emergency room visits for such children. Methods: A descriptive, exploratory study employing a quantitative, cross-sectional approach. The study sample comprised 246 children treated for asthma attacks in an emergency room. The approach used was evaluated in comparison with the approach recommended in the III Brazilian Consensus on Asthma Management, as was the length of time each child spent in the emergency room. Results: In 69 (28.1%) of the cases, the medications were used in accordance with the Consensus guidelines. In 34 (13.8%) of the cases, the doses used were those recommended in the Consensus guidelines, and the guidelines regarding nebulization procedures were followed in 33 cases (13.4%). No correlation was found between the approach taken and the length of the emergency room visit. In the logistic regression analysis, we observed that the adjusted risk of being released from the emergency room sooner than recommended in the Consensus guidelines (a length of time sufficient to allow the response to the treatment to be analyzed) was four times and fifteen times greater, respectively, for children with mild persistent asthma and for those with intermittent asthma. Conclusion: Although there are obstacles to using the asthma management approach recommended in the Consensus guidelines (such obstacles including the lack of familiarity with the guidelines on the part of the multidisciplinary team, as well as the lack of recommended material and therapeutic resources), the duration of emergency room visits was found to be related to the degree of asthma severity.

Keywords: Emergency medicine; Pediatrics; Asthma; Status asthmaticus