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.

Objective: To identify the indications for physiotherapy and to evaluate physiotherapy practices in patients with COVID-19 admitted to the ICU (on mechanical ventilation) or to the ward (spontaneously breathing). Methods: An online, 50-item survey was completed by physiotherapists who had been treating hospitalized patients with COVID-19 in Brazil. Results: Of the 644 physiotherapists who initiated the survey, 488 (76%) completed it. The main reasons for indications for physiotherapy in both settings reported as "very frequently" and "frequently" both in the ICU and the ward by most respondents were oxygenation improvement (> 95%) and prevention of general complications (> 83%). Physical deconditioning was considered an infrequent indication. When compared with mobilization strategies, the use of respiratory interventions showed great variability in both work settings, and techniques considered effective were underutilized. The most frequently used respiratory techniques in the ICU were positioning (86%), alveolar recruitment (73%), and hard/brief expiratory rib cage compression (46%), whereas those in the ward were active prone positioning (90%), breathing exercises (88%), and directed/assisted cough (75%). The mobilization interventions reported by more than 75% of the respondents were sitting on the edge of the bed, active and resistive range of motion exercises, standing, ambulation, and stepping in place. Conclusions: The least common reason for indications for physiotherapy was avoidance of deconditioning, whereas oxygenation improvement was the most frequent one. Great variability in respiratory interventions was observed when compared with mobilization therapies, and there is a clear need to standardize respiratory physiotherapy treatment for hospitalized patients with COVID-19.

Keywords: COVID-19; Physical therapy modalities; Hospitalization; Critical illness; Surveys and questionnaires.