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.

The evaluation of patients with lung cancer involves not only the detection of enlarged mediastinal lymph nodes and the delineation of the relationship between the primary lesion with the mediastinal structures but also the determination of extra thoracic metastases. This article reviews the major imaging techniques that are currently used to stage bronchogenic carcinoma. Radiologic studies have made an important impact on the evaluation of the solitary pulmonary nodule. Mediastinal staging coupled with imaging methods is not accurate. CT and MR imaging of the chest and abdomen are often used to stage a known or suspected lung carcinoma. Despite their limitations, CT and MR prevent unnecessary thoracotomies and invasive procedures. MR can be helpful in the evaluation of parts of the chest not well shown on CT. PET-FDG imaging complements chest CT in the noninvasive evaluation of lung cancer and strategies for its use merit further investigation.

Keywords: Radiology. Bronchogenic carcinoma. Lung neoplasms. Diagnostic imaging. Emission-computed tomography. Nuclear magnetic resonance.

Once a rare disease, lung cancer has become the most common type of lethal cancer throughout the world. This change has occurred since the 1920s , when it was observed both in Europe and in North America that the number of the cases began to increase. Although mentioned sporadically in the medical literature in the first half of the century, it was in 1950 that several well-performed case-controlled studies first suggested tobacco smoking as the overwhelming cause of lung cancer. It took even more years before results of large prospective studies, like the pioneering work of Doll and Hill, convinced both the public and the governments that smoking is the most important cause of the disease. Histologically, lung cancer is divided into four major subgroups: squamous-, adeno-, small cell, and large cell carcinomas. In a substantial number of cases, however, accurate histological diagnosis may be difficult, due to both intrinsic tumor heterogeneity and to inter-observer variability. Kreyberg suggested that only squamous and small cell carcinomas were caused by tobacco smoking, but most of the more recent studies indicate that the majority of adenocarcinomas is also caused by tobacco smoking. In all studies the proportion of adenocarcinoma is much higher in females than in males and secular trends during 1980s and 1990s have shown an increase in the occurrence of adenocarcinoma. Most occupational carcinogens, with the exception of chloromethyl ethers and radiation (radon), which have a higher tendency to promote small cell lung cancer, give rise to tumors with a histological distribution that is similar to tumors caused by tobacco smoke.

Keywords: Lung cancer. Epidemiology.

Lung cancer is a multifactorial disease. Hereditary, genetic, and environmental factors interact in its genesis. The principal risk factor for lung cancer is smoking. However, the workplace provides an environment in which there is a risk of exposure to carcinogens. The International Agency for Research on Cancer currently lists 19 substances/work situations/occupations that have been proven to be associated with lung cancer (group 1). Thorough occupational history taking is not widely practiced in patients with lung cancer, which has a negative impact on the investigation of causality and, consequently, on the identification of cases of occupational cancer. The objectives of this review were to list the agents that are recognized as causes of lung cancer, to discuss the contribution of occupation to the development of the disease, to cite national studies on the subject, and to propose a list of procedures that are essential to the appropriate investigation of causality between lung cancer and occupation.

Keywords: Lung neoplasms; Occupational diseases; Occupations; Carcinogens.

A review of the role of radiotherapy in lung cancer is presented, with the most recent innovations. Indications for postoperative irradiation and chemo-radiotherapy in non-small cell lung cancer are discussed. Recommendations are made for prophylactic cranium irradiation and perspectives of thoracic radiotherapy for small cell lung cancer. Recent techniques and results of the "Brazilian Consensus on Lung Cancer - Radiotherapy" are also presented.

The relationship between cancer and coagulopathy was suggested by Trousseau nearly 150 years ago. Later, it became more evident that oncologic patients are at a higher risk of experiencing thromboembolic events. This can be due to activation of the coagulation system either by neoplastic cells or by prescribed therapies (chemotherapy or surgical procedures). In fact, these events can constitute the first manifestation of cancer, and their recurrence, despite efficient anticoagulation, has been described. The coagulation system is normally activated in order to provide healing. In the presence of neoplasms, this complex system is activated as a response to multiple stimuli and seems to contribute to cancer progression. Activation of the coagulation system has a greater effect on metastatic foci than on the primary tumor. However, most cancer victims die from complications caused by metastasis, which underscores the importance of this theme. In this area, various mechanisms have been described, creating promising perspectives for future treatments. The current success in using low-molecular-weight heparins against small cell lung cancer is encouraging. Although the knowledge of those mechanisms is relatively incipient, many basic research and clinical studies are underway.

Keywords: Lung neoplasms; Thrombophilia.

Lung cancer is one of the most incident types of cancer and a leading cause of cancer mortality in Brazil. We reviewed the current status of lung cancer by searching relevant data on prevention, diagnosis, and treatment in the country. This review highlights several issues that need to be addressed, including smoking control, patient lack of awareness, late diagnosis, and disparities in the access to cancer health care facilities in Brazil. We propose strategies to help overcome these limitations and challenge health care providers, as well as the society and governmental representatives, to work together and to take a step forward in fighting lung cancer.

Keywords: Lung neoplasms/epidemiology; Lung neoplasms/therapy; Lung neoplasms/diagnosis; Biomarkers; Brazil.