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.

A pneumonia por Pneumocystis carinii (PPC), que é a infecção oportunista que mais comumente põe em risco a vida de pacientes com síndrome da imunodeficiência adquirida (SIDA), tem sido há bastante tempo associada a outros estados de imunodeficiência. Os relatos iniciais sugeriram que a apresentação clínica da PPC em SIDA difere de sua apresentação em outras situações, embora ambas tenham sofrido modificações nos últimos anos. O objetivo do presente estudo é comparar os achados clínicos e laboratoriais da PPC em SIDA com os de pacientes portadores de PPC sem SIDA. Foram revisados os prontuários médicos de todos os pacientes em nossa instituição que tiveram o diagnóstico confirmado de PPC e SIDA durante o ano de 1994 (17 pacientes) e sem SIDA no período de 1992-1994 (dez pacientes). Quando da apresentação, os pacientes sem SIDA tinham uma duração de sintomas significativamente menor (8,4 ± 7,7 vs 19,5 ± 10,2 dias, p < 0,05), menor PaO2 média a ar ambiente (47 ± 8,2 vs 63 ± 10mmHg, p < 0,05), e mais freqüente infiltrado unilateral no radiograma do tórax (60 vs 6%, p < 0,05). O índice de mortalidade da PPC foi marcadamente maior nos pacientes sem SIDA (40 vs 6%, p < 0,05), e este fato estava associado com retarde no início do tratamento específico nestes pacientes. Devido a seu curso mais agudo, apresentação radiológica atípica e alto índice de mortalidade, concluímos que o diagnóstico de PPC deve ser afastado em pacientes que recebem terapia imunossupressora e que profilaxia anti-PPC deve ser fortemente considerada em pacientes que serão imunossuprimidos.

Keywords: PPC-AIDS. PPC não-AIDS.

Mycobacterium tuberculosis is one of the most successful human pathogens. Highly virulent strains, which are more easily transmitted than are less virulent strains, elicit variable immune responses. We evaluated the Th1 responses (IFN-γ production) in healthy volunteers after stimulation with various strains. Our results show that the individuals with negative tuberculin skin test (TST) results were not necessarily naive to all of the strains tested, whereas individuals with positive TST results did not respond to all of the strains tested. Drug-resistant strains induced a lower mean level of IFN-γ production than did drug-sensitive strains. One possible practical application of this finding would be for the prediction of responses to treatment, in which it might be advantageous to have knowledge of the estimated IFN-γ production elicited by a specific isolated strain.

Keywords: Tuberculosis; Polymorphism, restriction fragment length; Mycobacterium tuberculosis; Immunity, cellular; Interferon-gamma.

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: To determine the prevalence and the characteristics of Mycoplasma pneumoniae-related community-acquired pneumonia (CAP) and parapneumonic pleural effusion (PPE) in children and adolescents. Methods: This was a retrospective observational study involving 121 patients with CAP/PPE hospitalized in a tertiary referral hospital between 2000 and 2008, divided into six groups according to the etiologic agent (G1 to G6, respectively): M. pneumoniae with or without co-infection, in 44 patients (group 1); etiologic agents other than M. pneumoniae, in 77 (group 2); M. pneumoniae without co-infection, in 34 (group 3); Streptococcus pneumoniae, in 36 (group 4); Staphylococcus aureus, in 31 (group 5); and M. pneumoniae/S. pneumoniae co-infection, in 9 (group 6). Results: In comparison with group 2, group 1 showed higher frequencies of females, dry cough, and previous use of beta-lactam antibiotics; longer duration of symptoms prior to admission; and lower frequencies of use of mechanical ventilation and chest tube drainage. In comparison with groups 4 and 5, group 3 showed higher frequencies of previous use of beta-lactam antibiotics and dry cough; longer duration of symptoms prior to admission; a lower frequency of use of chest tube drainage; a higher mean age and a lower frequency of nausea/vomiting (versus group 4 only); and a lower frequency of use of mechanical ventilation (versus group 5 only). M. pneumoniae/S. pneumoniae co-infection increased the duration of symptoms prior to admission. Conclusions: In this sample, the prevalence of M. pneumoniae-related CAP/PPE was 12.75%. Although the disease was milder than that caused by other microorganisms, its course was longer. Our data suggest that M. pneumoniae-related CAP/PPE in children and adolescents should be more thoroughly investigated in Brazil.

Keywords: Pleural effusion; Empyema, pleural; Pneumonia; Mycoplasma pneumoniae.

Objective: Pleurodesis is an effective alternative for the control of malignant pleural effusions. However, there is as yet no consensus regarding the indications for the procedure and the techniques employed therein. The objective of this study was to evaluate how pleurodesis is performed in South and Central America. Methods: Professionals who perform pleurodesis completed a questionnaire regarding the indications for the procedure, the techniques used therein, and the outcomes obtained. Results: Our sample comprised 147 respondents in Brazil, 49 in other South American countries, and 36 in Central America. More than 50% of the respondents reported performing pleurodesis only if pleural malignancy had been confirmed. However, scores on dyspnea and performance status scales were rarely used as indications for the procedure. Nearly 75% of the respondents in Brazil and in Central America preferred to perform pleurodesis only for recurrent effusions and stated that lung expansion should be 90-100%. Talc slurry, instilled via medium-sized chest tubes, was the agent most often employed. Thoracoscopy was performed in less than 25% of cases. Fever and chest pain were the most common side effects, and empyema occurred in ≤ 14% of cases. The mean survival time after the procedure was most often reported to be 6-12 months. Conclusions: There was considerable variation among the countries evaluated in terms of the indications for pleurodesis, techniques used, and outcomes. Talc slurry is the agent most commonly used, and thoracoscopy is the technique of choice in Brazil. Pleurodesis is an effective procedure that has few side effects, as evidenced by the low complication rates and high survival times.

Keywords: Pleural effusion, malignant; Pleura; Pleurodesis.

Objetivo: Realizar a tradução para a língua portuguesa e a adaptação cultural para uso no Brasil do Quality of Communication Questionnaire (QOC) para pacientes com DPOC em cuidados paliativos. Métodos: Após a aprovação do autor do questionário e do comitê de ética da instituição, a versão original do QOC com 13 itens foi traduzida, de forma independente, por dois tradutores brasileiros com fluência na língua inglesa. Uma síntese das duas traduções foi realizada por uma médica bilíngue e os dois tradutores, que chegaram a um consenso, gerando uma versão na língua portuguesa. Essa versão foi retraduzida por dois tradutores nativos de países de língua inglesa com fluência na língua portuguesa. Para resolver quaisquer discrepâncias, um comitê de especialistas comparou a versão original com todas as versões produzidas e, assim, obteve-se a versão pré-final do QOC. O pré-teste foi aplicado em 32 pacientes internados em UTIs de três hospitais públicos da grande Florianópolis (SC) para avaliar a clareza e a aceitabilidade cultural da versão pré-final do QOC. Resultados: A média de idade dos pacientes foi de 48,5 ± 18,8 anos. A maioria dos itens foi bem compreendida e aceita, recebendo pontuações ≥ 8. Um item, sobre morte, suscitou dificuldades na compreensão dos participantes do pré-teste. Ao submeter a retradução ao autor do QOC, ele solicitou alterações em dois itens, que foram acatadas. Após essas alterações, a versão final foi aprovada. Conclusões: A tradução e a adaptação cultural do QOC para uso no Brasil foi concluída com sucesso.

Keywords: Doença pulmonar obstrutiva crônica; Inquéritos e questionários; Comunicação; Unidades de terapia intensiva.