Brazilian Journal of Pulmonology

ISSN (on-line): 1806-3756 | ISSN (printed): 1806-3713

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Brazilian guidelines for the diagnosis and treatment of cystic fibrosis

Diretrizes brasileiras de diagnóstico e tratamento da fibrose cística

Rodrigo Abensur Athanazio1*, Luiz Vicente Ribeiro Ferreira da Silva Filho2,3*, Alberto Andrade Vergara4, Antônio Fernando Ribeiro5, Carlos Antônio Riedi6, Elenara da Fonseca Andrade Procianoy7, Fabíola Villac Adde2, Francisco José Caldeira Reis4, José Dirceu Ribeiro5, Lídia Alice Torres8, Marcelo Bicalho de Fuccio9, Matias Epifanio10, Mônica de Cássia Firmida11, Neiva Damaceno12, Norberto Ludwig-Neto13,14, Paulo José Cauduro Maróstica7,15, Samia Zahi Rached1, Suzana Fonseca de Oliveira Melo4; Grupo de Trabalho das Diretrizes Brasileiras de Diagnóstico e Tratamento da Fibrose Cística.

J Bras Pneumol.2017;43(3):219-245

Abstract PDF PT PDF EN Portuguese Text Appendix

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions.

 


Keywords: Cystic fibrosis/diagnosis; Cystic fibrosis/therapy; Cystic fibrosis/complications; Practice guideline.

 


The pulmonary microbiome: challenges of a new paradigm

Microbioma pulmonar: desafios de um novo paradigma

André Nathan Costa1,a, Felipe Marques da Costa1,b, Silvia Vidal Campos1,c, Roberta Karla Salles1,d, Rodrigo Abensur Athanazio1,e

J Bras Pneumol.2018;44(5):424-432

Abstract PDF PT PDF EN Portuguese Text

The study of the human microbiome-and, more recently, that of the respiratory system-by means of sophisticated molecular biology techniques, has revealed the immense diversity of microbial colonization in humans, in human health, and in various diseases. Apparently, contrary to what has been believed, there can be nonpathogenic colonization of the lungs by microorganisms such as bacteria, fungi, and viruses. Although this physiological lung microbiome presents low colony density, it presents high diversity. However, some pathological conditions lead to a loss of that diversity, with increasing concentrations of some bacterial genera, to the detriment of others. Although we possess qualitative knowledge of the bacteria present in the lungs in different states of health or disease, that knowledge has advanced to an understanding of the interaction of this microbiota with the local and systemic immune systems, through which it modulates the immune response. Given this intrinsic relationship between the microbiota and the lungs, studies have put forth new concepts about the pathophysiological mechanisms of homeostasis in the respiratory system and the potential dysbiosis in some diseases, such as cystic fibrosis, COPD, asthma, and interstitial lung disease. This departure from the paradigm regarding knowledge of the lung microbiota has made it imperative to improve understanding of the role of the microbiome, in order to identify possible therapeutic targets and to develop innovative clinical approaches. Through this new leap of knowledge, the results of preliminary studies could translate to benefits for our patients.

 


Keywords: Microbiota; Microbiology; Immune system.

 


Recommendations for the pharmacological treatment of COPD: questions and answers

Recomendações para o tratamento farmacológico da DPOC: perguntas e respostas

Frederico Leon Arrabal Fernandes1, Alberto Cukier1, Aquiles Assunção Camelier2,3, Carlos Cezar Fritscher4, Cláudia Henrique da Costa5, Eanes Delgado Barros Pereira6, Irma Godoy7, José Eduardo Delfini Cançado8, José Gustavo Romaldini8, Jose Miguel Chatkin4, José Roberto Jardim9, Marcelo Fouad Rabahi10, Maria Cecília Nieves Maiorano de Nucci11, Maria da Penha Uchoa Sales12, Maria Vera Cruz de Oliveira Castellano13, Miguel Abidon Aidé14, Paulo José Zimermann Teixeira15,16, Renato Maciel17, Ricardo de Amorim Corrêa18, Roberto Stirbulov8, Rodrigo Abensur Athanazio1, Rodrigo Russo19, Suzana Tanni Minamoto7, Fernando Luiz Cavalcanti Lundgren20

J Bras Pneumol.2017;43(4):290-301

Abstract PDF PT PDF EN Portuguese Text

The treatment of COPD has become increasingly effective. Measures that range from behavioral changes, reduction in exposure to risk factors, education about the disease and its course, rehabilitation, oxygen therapy, management of comorbidities, and surgical and pharmacological treatments to end-of-life care allow health professionals to provide a personalized and effective therapy. The pharmacological treatment of COPD is one of the cornerstones of COPD management, and there have been many advances in this area in recent years. Given the greater availability of drugs and therapeutic combinations, it has become increasingly challenging to know the indications for, limitations of, and potential risks and benefits of each treatment modality. In order to critically evaluate recent evidence and systematize the major questions regarding the pharmacological treatment of COPD, 24 specialists from all over Brazil gathered to develop the present recommendations. A visual guide was developed for the classification and treatment of COPD, both of which were adapted to fit the situation in Brazil. Ten questions were selected on the basis of their relevance in clinical practice. They address the classification, definitions, treatment, and evidence available for each drug or drug combination. Each question was answered by two specialists, and then the answers were consolidated in two phases: review and consensus by all participants. The questions answered are practical questions and help select from among the many options the best treatment for each patient and his/her peculiarities.

 


Keywords: Pulmonary disease, chronic obstructive/drug therapy; pulmonary disease, chronic obstructive/prevention & control; pulmonary disease, chronic obstructive/therapy.

 


Symptom variability over the course of the day in patients with stable COPD in Brazil: a real-world observational study

Variabilidade dos sintomas diários de pacientes com DPOC estável no Brasil: um estudo observacional de vida real

Alberto Cukier1, Irma de Godoy2, Claudia Henrique da Costa3, Adalberto Sperb Rubin4, Marcelo Gervilla Gregorio5, Aldo Agra de Albuquerque Neto6, Marina Andrade Lima7, Monica Corso Pereira8, Suzana Erico Tanni2, Rodrigo Abensur Athanazio1, Elizabeth Jauhar Cardoso Bessa3, Fernando Cesar Wehrmeister9, Cristina Bassi Lourenco10, Ana Maria Baptista Menezes9

J Bras Pneumol.2020;46(3):e20190223-e20190223

Abstract PDF PT PDF EN Portuguese Text Appendix

Objective: To analyze symptoms at different times of day in patients with COPD. Methods: This was a multicenter, cross-sectional observational study conducted at eight centers in Brazil. We evaluated morning, daytime, and nighttime symptoms in patients with stable COPD. Results: We included 593 patients under regular treatment, of whom 309 (52.1%) were male and 92 (15.5%) were active smokers. The mean age was 67.7 years, and the mean FEV1 was 49.4% of the predicted value. In comparison with the patients who had mild or moderate symptoms, the 183 (30.8%) with severe symptoms were less physically active (p = 0.002), had greater airflow limitation (p < 0.001), had more outpatient exacerbations (p = 0.002) and more inpatient exacerbations (p = 0.043), as well as scoring worse on specific instruments. The most common morning and nighttime symptoms were dyspnea (in 45.2% and 33.1%, respectively), cough (in 37.5% and 33.3%, respectively), and wheezing (in 24.4% and 27.0%, respectively). The intensity of daytime symptoms correlated strongly with that of morning symptoms (r = 0.65, p < 0.001) and that of nighttime symptoms (r = 0.60, p < 0.001), as well as with the COPD Assessment Test score (r = 0.62; p < 0.001), although it showed only a weak correlation with FEV1 (r = −0.205; p < 0.001). Conclusions: Dyspnea was more common in the morning than at night. Having morning or nighttime symptoms was associated with greater daytime symptom severity. Symptom intensity was strongly associated with poor quality of life and with the frequency of exacerbations, although it was weakly associated with airflow limitation.

 


Keywords: Pulmonary disease, chronic obstructive; Signs and symptoms, respiratory; Quality of life; Disease progression; Brazil.

 


 

 


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E-mails: jbp@jbp.org.br
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