Objective: To determine the impact of adherence to long-term oxygen therapy (LTOT) on quality of life, dyspnea, and exercise capacity in patients with COPD and exertional hypoxemia followed for one year. Methods: Patients experiencing severe hypoxemia during a six-minute walk test (6MWT) performed while breathing room air but not at rest were included in the study. At baseline and after one year of follow-up, all patients were assessed for comorbidities, body composition, SpO2, and dyspnea, as well as for anxiety and depression, having also undergone spirometry, arterial blood gas analysis, and the 6MWT with supplemental oxygen. The Saint George's Respiratory Questionnaire (SGRQ) was used in order to assess quality of life, and the Body mass index, airflow Obstruction, Dyspnea, and Exercise capacity (BODE) index was calculated. The frequency of exacerbations and the mortality rate were noted. Treatment nonadherence was defined as LTOT use for < 12 h per day or no LTOT use during exercise. Results: A total of 60 patients with COPD and exertional hypoxemia were included in the study. Of those, 10 died and 11 experienced severe hypoxemia during follow-up, 39 patients therefore being included in the final analysis. Of those, only 18 (46.1%) were adherent to LTOT, showing better SGRQ scores, higher SpO2 values, and lower PaCO2 values than did nonadherent patients. In all patients, SaO2, the six-minute walk distance, and the BODE index worsened after one year. There were no differences between the proportions of adherence to LTOT at 3 and 12 months of follow-up. Conclusions: Quality of life appears to be lower in patients with COPD and exertional hypoxemia who do not adhere to LTOT than in those who do. In addition, LTOT appears to have a beneficial effect on COPD symptoms (as assessed by SGRQ scores). (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR‑9b4v63 [http://www.ensaiosclinicos.gov.br])

Keywords: Respiratory insufficiency; Pulmonary disease, chronic obstructive; Patient compliance; Hypoxia; Oxygen inhalation therapy.

Objective: Assess the relationship between adherence to long-term oxygen therapy (LTOT) with mortality in patients with chronic obstructive pulmonary disease (COPD) and chronic respiratory failure and their clinical features. Methods: Longitudinal retrospective analysis of 254 patients with COPD and chronic respiratory failure from 2008 to 2016. At baseline, we evaluated the diagnosis, spirometry values, arterial blood gas analysis, blood count, pulse oximetry, body composition and health questionnaires (dyspnea, quality of life, anxiety and depression). For referred adherence analysis to LTOT we included 199 patients, divided according to prescription of oxygen: 12h/day (G1), 15h/day (G2) and 24h/day (G3). The cause of death and dates were studied over the five-year period. Results: In five years we identified 124 deaths (62.3%). No significant difference was found in mortality between the adherence groups (p=0.75) nor did we find differences in the clinical parameters evaluated. LTOT prescription was not associated with mortality (p=0.07). In Cox regression analysis, there was no association between mortality and non-adherence to LTOT (HR: 0.75; IC95%: 0.21-2.70). The risk of mortality was increased in G3 compared with G1 (HR: 7.16; IC 95%: 1.44-35.38) and in those with a higher depression score (HR: 1.35; IC: 1.14-1.59). Conclusion: No association was found between LTOT adherence and mortality in patients with COPD and respiratory failure. There were no clinical differences between the adherence groups.

Keywords: Chronic obstructive pulmonary disease; Oxygen therapy; Cooperation and treatment adherence; Morbidity and mortality indicators.

Objective: To evaluate the health-related quality of life of patients with chronic obstructive pulmonary disease receiving long-term oxygen therapy (LTOT) at home through oxygen cylinders and compare these results with those obtained six months after the transition from oxygen cylinders to oxygen concentrators. Methods: A total of 45 patients were evaluated. Of those, 24 had chronic hypoxemia and 21 presented no evidence of hypoxemia. The patients with chronic hypoxemia had been regularly receiving LTOT for at least the last six months and were evaluated at baseline, while using cylinders, and six months after the transition from cylinders to concentrators. The non-hypoxemic patients were evaluated at the same time points as were the hypoxemic patients. In order to evaluate quality of life, a version of the Saint George's Respiratory Questionnaire (SGRQ), translated and validated for use in Brazil, was administered. Results: At baseline, quality of life, as evaluated using the total score and the symptom and impact domain scores of the SGRQ, was more impaired in the hypoxemic patients than in the non-hypoxemic patients. After six months of using the concentrators, the hypoxemic patients presented a significant improvement in the quality of life, and, at that time, no difference was found between the patients with and without hypoxemia. Conclusion: Our findings show that quality of life is impaired in patients with chronic obstructive pulmonary disease and chronic hypoxemia, that their quality of life can be improved through regular use of LTOT, and that the oxygen delivery system has an influence on this improvement.

Keywords: Pulmonary disease, Chronic obstructive; Anoxemia; Oxygen inhalation therapy; Quality of life.

Objective: To evaluate the agreement between the criteria used for hospitalization of patients with community-acquired pneumonia (CAP) and those of the Brazilian Thoracic Association guidelines, and to evaluate the association of that agreement with 30-day mortality. Secondarily, to evaluate the agreement between the treatment given and that recommended in the guidelines with length of hospital stay, microbiological profile, 12-month mortality, complications, ICU admission, mechanical ventilation, and 30-day mortality. Methods: This was a retrospective study involving adult patients hospitalized between 2005 and 2007 at the Federal University of Minas Gerais Hospital das Clínicas, located in Belo Horizonte, Brazil. Medical charts and chest X-rays were reviewed. Results: Among the 112 patients included in the study, admission and treatment criteria were in accordance with the guidelines in 82 (73.2%) and 66 (58.9%), respectively. The 30-day and 12-month mortality rates were 12.3% and 19.4%, respectively. The 30-day mortality rate was lower for patients in whom the CRB-65 (mental Confusion, Respiratory rate, Blood pressure, and age ≥ 65 years) score was 1-2 and the antibiotic therapy was in accordance with the guidelines (p = 0.01). Cerebrovascular disease and appropriate antibiotic therapy showed independent associations with 30-day mortality. There was a trend toward an association between guideline-concordant antibiotic therapy and shorter hospital stay. Conclusions: In the population studied, admission and treatment criteria that were in accordance with the guidelines were associated with favorable outcomes in hospitalized patients with CAP. Cerebrovascular disease, as a risk factor, and guideline-concordant antibiotic therapy, as a protective factor, were associated with 30-day mortality.

Keywords: Pneumonia/therapy; Pneumonia/mortality; Hospitalization; Guideline adherence.

Objective: To evaluate and correlate the quality of life (QoL) of COPD patients on long-term home oxygen therapy (LTOT) with their sociodemographic/clinical characteristics and level of dependence. Methods: This was a cross-sectional analytical study involving COPD patients on LTOT followed at the Oxygen Therapy Outpatient Clinic of the Federal University of São Paulo Hospital São Paulo, in the city of São Paulo, Brazil. Sociodemographic, clinical, and biochemical data were collected. We assessed QoL and level of dependence using the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36) and the Katz index, respectively. Multiple linear regression models were constructed in order to determine the influence of these variables on QoL. Results: We included 80 patients in the study. The mean age was 69.6 ± 9.1 years, and 51.3% were female. The lowest SF-36 scores were for the physical functioning and role-physical domains. All sociodemographic characteristics (except gender) were found to correlate significantly with the SF-36 domains mental health, vitality, role-physical, and social functioning. We also found that body mass index, PaO2, post-bronchodilator FEV1, hemoglobin, and Katz index correlated significantly with the physical functioning, mental health, role-physical, and bodily pain domains. In addition, oxygen flows were found to correlate negatively with the physical functioning, mental health, vitality, and role-emotional domains. Conclusions: Low scores for SF-36 domains, as well as the variables that negatively influence them, should be considered and analyzed during the development and implementation of strategies for improving the QoL of COPD patients on LTOT.

Keywords: Pulmonary disease, chronic obstructive; Quality of life; Oxygen inhalation therapy.

Objective: To evaluate the effect of a short-term individualized education program on adherence to asthma treatment, inhalation techniques, and asthma control. Methods: A prospective study involving patients aged 14 years or older, with a confirmed diagnosis of asthma and recruited from the asthma outpatient clinic of a university hospital in the city of Porto Alegre, Brazil. The study was conducted in two phases (before and after the educational intervention). At a routine medical visit, the participants completed a general questionnaire in order to assess the level of asthma control and inhalation techniques. The participants also underwent pulmonary function testing. Subsequently, they participated in an asthma education program, which consisted of one individualized session. The participants were reevaluated after three months. Results: Of the 174 patients recruited, 115 completed the study. Between the first and second evaluations, there was a significant improvement in the effective use of inhaled corticosteroids (90.4% vs. 93.3%; p = 0.003), the effective use of long-acting β2 agonists (57.4% vs. 63.5%; p < 0.0001), the effective use of a combined regimen with these two medications (57.4% vs. 62.6%; p < 0.0001), and the self-reported adherence to corticosteroid therapy (p = 0.001). There was a significant decrease in the proportion of patients visiting ERs (30.4% vs. 23.5%; p = 0.012). However, the level of asthma control and the inhalation technique did not improve significantly (p = 0.095 and p = 0.512, respectively). Conclusions: This short-term asthma education program resulted in an improvement in the use of medications for asthma control and a decrease in the number of ER visits, although it had no significant effect on the inhalation technique.

Keywords: Ambulatory care; Patient education as topic; Respiratory therapy; Metered dose inhalers.

Objective: To verify association between left ventricular (LV) mass and thickness and the presence of significant nocturnal hypoxemia in patients with COPD with mild diurnal hypoxemia. Methods: A cross-sectional study carried out in clinically stable outpatients with COPD and mild hypoxemia (oxygen saturation ≥90 to ≤94%, identified by noninvasive oximetry) in a clinic specialized in the treatment of respiratory diseases in Goiânia-GO. All patients were submitted to clinical evaluation, spirometry, polysomnography, echocardiography, arterial blood gas analysis, 6-minute walk test and chest X-ray. Results: Patients with significant nocturnal hypoxemia had echocardiographic parameters associated with increase of LV musculature when compared to patients with mild nocturnal hypoxemia. The LV volume/mass ratio was significantly lower in the group with significant nocturnal hypoxemia (ratio 0.64 ± 0.13 versus 0.72 ± 0.12, p = 0.04), the thickness diastolic diameter of the interventricular septum and the diastolic thickness of the LV posterior wall were significantly higher in this group (9.7 ± 0.92 versus 9.1 ± 0.90 p = 0.03), (9.7 ± 1.0 versus 8.9 ± 1.0, p = 0.01. The time in REM sleep with saturation below 85% significantly predicted septum thickness (adjustment for BMI, age and mean blood pressure, r2 = 0.20; p = 0.046). Conclusion: We observed association between severe REM sleep hypoxemia and echocardiographic parameters indicating increased LV mass in individuals with COPD and significant nocturnal hypoxemia. This suggests that this subgroup of individuals may benefit from an echocardiographic evaluation of the left ventricle.

Keywords: Chronic Obstructive Pulmonary Disease; Left ventricular hypertrophy; Echocardiography.

Objective: To evaluate the association between adherence to treatment and mortality among Chronic Obstructive Pulmonary Disease (COPD) patients treated in the Brazilian public health system. Methods: This is cohort study of moderate-to-severe COPD patients monitored in a public pharmaceutical care-based Disease Management Program (DMP). All subjects who died one year after the beginning of the cohort were age-matched with those who remained alive at the end of the cohort period. Treatment adherence was measured through pharmacy records. Patients who received at least 90% of the prescribed doses were considered adherent to treatment. Results: Of the 333 patients (52.8% age = 65 years, 67.9% male), 67.3% were adherent to treatment (adherence rate, 87.2%). Mortality was associated with lack of adherence (p = 0.04), presence of symptoms (mMRC = 2) and COPD treatment use. The death was associated with non-adherence, presence of symptoms and previous hospitalization. After adjustment, non-adherent patients to treatment were almost twice times likely to die compared to those adherents (Hazard Ratio (HR) 1.86; CI 1.16-2.98, p = 0.01). Conclusion: Non-adherence to treatment was associated with higher mortality among moderate-to-severe COPD patients treated in the Brazilian public health system. Strategies to monitor and optimize adherence should be strengthened to reduce COPD-related mortality.

Keywords: COPD; Treatment adherence; Mortality; Pharmaceutical care.

Nonadherence to inhaled corticosteroid therapy is common and has a negative effect on clinical control, as well as increasing morbidity rates, mortality rates and health care costs. This review was conducted using direct searches, together with the following sources: Medline; HighWire; and the Latin American and Caribbean Health Sciences Literature database. Searches included articles published between 1992 and 2008. The following methods of assessing adherence, listed in ascending order by degree of objectivity, were identified: patient or family reports; clinical judgment; weighing/dispensing of medication, electronic medication monitoring; and (rarely) biochemical analysis. Adherence rates ranged from 30 to 70%. It is recognized that the degree of adherence determined by patient/family reports or by clinical judgment is exaggerated in comparison with that obtained using electronic medication monitors. Physicians should bear in mind that true adherence rates are lower than those reported by patients, and this should be considered in cases of poor clinical control. Weighing the spray quantifies the medication and infers adherence. However, there can be deliberate emptying of inhalers and medication sharing. Pharmacies provide the dates on which the medication was dispensed and refilled. This strategy is valid and should be used in Brazil. The use of electronic medication monitors, which provide the date and time of each triggering of the medication device, although costly, is the most accurate method of assessing adherence. The results obtained with such monitors demonstrate that adherence was lower than expected. Physicians should improve their knowledge on patient adherence and use accurate methods of assessing such adherence.

Keywords: Patient compliance; Asthma/therapy; Asthma/prevention & control.

Continuous positive airway pressure (CPAP) is the gold standard for the treatment of obstructive sleep apnea (OSA). Although CPAP was originally applied with a nasal mask, various interfaces are currently available. This study reviews theoretical concepts and questions the premise that all types of interfaces produce similar results. We revised the evidence in the literature about the impact that the type of CPAP interface has on the effectiveness of and adherence to OSA treatment. We searched the PubMed database using the search terms "CPAP", "mask", and "obstructive sleep apnea". Although we identified 91 studies, only 12 described the impact of the type of CPAP interface on treatment effectiveness (n = 6) or adherence (n = 6). Despite conflicting results, we found no consistent evidence that nasal pillows and oral masks alter OSA treatment effectiveness or adherence. In contrast, most studies showed that oronasal masks are less effective and are more often associated with lower adherence and higher CPAP abandonment than are nasal masks. We concluded that oronasal masks can compromise CPAP OSA treatment adherence and effectiveness. Further studies are needed in order to understand the exact mechanisms involved in this effect.

Keywords: Sleep apnea, obstructive; Continuous positive airway pressure; Masks.

Objective: To infer the prevalence and variables predictive of isolated nocturnal hypoxemia and obstructive sleep apnea (OSA) in patients with COPD and mild hypoxemia. Methods: This was a cross-sectional study involving clinically stable COPD outpatients with mild hypoxemia (oxygen saturation = 90-94%) at a clinical center specializing in respiratory diseases, located in the city of Goiânia, Brazil. The patients underwent clinical evaluation, spirometry, polysomnography, echocardiography, arterial blood gas analysis, six-minute walk test assessment, and chest X-ray. Results: The sample included 64 patients with COPD and mild hypoxemia; 39 (61%) were diagnosed with sleep-disordered breathing (OSA, in 14; and isolated nocturnal hypoxemia, in 25). Correlation analysis showed that PaO2 correlated moderately with mean sleep oxygen saturation (r = 0.45; p = 0.0002), mean rapid eye movement (REM) sleep oxygen saturation (r = 0.43; p = 0.001), and mean non-REM sleep oxygen saturation (r = 0.42; p = 0.001). A cut-off point of PaO2 ≤ 70 mmHg in the arterial blood gas analysis was significantly associated with sleep-disordered breathing (OR = 4.59; 95% CI: 1.54-13.67; p = 0.01). The model showed that, for identifying sleep-disordered breathing, the cut-off point had a specificity of 73.9% (95% CI: 51.6-89.8%), a sensitivity of 63.4% (95% CI: 46.9-77.9%), a positive predictive value of 81.3% (95% CI: 67.7-90.0%), and a negative predictive value of 53.1% (95% CI: 41.4-64.4%), with an area under the ROC curve of 0.69 (95% CI: 0.57-0.80), correctly classifying the observations in 67.2% of the cases. Conclusions: In our sample of patients with COPD and mild hypoxemia, the prevalence of sleep-disordered breathing was high (61%), suggesting that such patients would benefit from sleep studies.

Keywords: Pulmonary disease, chronic obstructive/complications; Sleep wake disorders/epidemiology; Anoxia/etiology.

Objective: Currently, little is known about the long-term outcomes of COVID-19 in the pediatric population. The aim of this study was to investigate the long-term clinical outcomes of pediatric patients hospitalized with COVID-19. Methods: This was a prospective cohort study involving unvaccinated children and adolescents admitted to a tertiary hospital in southern Brazil with a COVID-19 diagnosis. Data were collected from electronic medical records for one year after the diagnosis. Results: A total of 66 children were included: the median age was 2.9 years; 63.6% were male; and 48.5% were under 2 years of age. Over 70% had at least one comorbidity prior to the COVID-19 diagnosis. During the one-year follow-up period, 59.1% of the children revisited the emergency department, 50% required readmission, and 15.2% died. Younger children with longer hospital stays were found to be at greater risk of readmission. Having cancer and impaired functionality were found to increase the risk of death within one year. Conclusions: Our findings indicate that most children hospitalized with COVID-19 have comorbidities. Younger age at admission and a longer hospital stay seem to be risk factors for readmission. In addition, the presence of cancer and impaired functionality are apparently associated with the poor outcome of death within the first year after the diagnosis of COVID-19.

Keywords: COVID-19; Pediatrics; Physical functional performance.

Objective: To evaluate symptoms, lung function, and quality of life of a cohort of patients hospitalized for severe COVID-19 12 months after hospital admission. Methods: This was a cross-sectional study. We included severe COVID-19 survivors hospitalized in one of three tertiary referral hospitals for COVID-19 in the city of Belo Horizonte, Brazil. Participants were submitted to lung function and six-minute walk tests and completed the EQ-5D-3L questionnaire. Results: The whole sample comprised 189 COVID-19 survivors (mean age = 59.6 ± 13.4 years) who had been admitted to a ward only (n = 96; 50.8%) or to an ICU (n = 93; 49.2%). At 12 months of follow-up, 43% of patients presented with dyspnea, 27% of whom had a restrictive ventilatory disorder and 18% of whom presented with impaired DLCO. There were no significant differences in FVC, FEV1, and TLC between the survivors with or without dyspnea. However, those who still had dyspnea had significantly more impaired DLCO (14.9% vs. 22.4%; p < 0.020) and poorer quality of life. Conclusions: After one year, survivors of severe COVID-19 in a middle-income country still present with high symptom burden, restrictive ventilatory changes, and loss of quality of life. Ongoing follow-up is needed to characterize long COVID-19 and identify strategies to mitigate its consequences.

Objective: To evaluate the impact of thoracic radiotherapy on respiratory function and exercise capacity in patients with breast cancer. Methods: Breast cancer patients in whom thoracic radiotherapy was indicated after surgical treatment and chemotherapy were submitted to HRCT, respiratory evaluation, and exercise capacity evaluation before radiotherapy and at three months after treatment completion. Respiratory muscle strength testing, measurement of chest wall mobility, and complete pulmonary function testing were performed for respiratory evaluation; cardiopulmonary exercise testing was performed to evaluate exercise capacity. The total radiotherapy dose was 50.4 Gy (1.8 Gy/fraction) to the breast or chest wall, including supraclavicular lymph nodes (SCLN) or not. Dose-volume histograms were calculated for each patient with special attention to the ipsilateral lung volume receiving 25 Gy (V25), in absolute and relative values, and mean lung dose. Results: The study comprised 37 patients. After radiotherapy, significant decreases were observed in respiratory muscle strength, chest wall mobility, exercise capacity, and pulmonary function test results (p < 0.05). DLCO was unchanged. HRCT showed changes related to radiotherapy in 87% of the patients, which was more evident in the patients submitted to SCLN irradiation. V25% significantly correlated with radiation pneumonitis. Conclusions: In our sample of patients with breast cancer, thoracic radiotherapy seemed to have caused significant losses in respiratory and exercise capacity, probably due to chest wall restriction; SCLN irradiation represented an additional risk factor for the development of radiation pneumonitis.

Keywords: Breast neoplasms; Radiotherapy; Radiation pneumonitis; Respiratory function tests; Exercise test.

Objective: To identify factors that lead to a positive oxygenation response and predictive factors of mortality after prone positioning. Methods: This was a retrospective, multicenter, cohort study involving seven hospitals in Brazil. Inclusion criteria were being > 18 years of age with a suspected or confirmed diagnosis of COVID-19, being on invasive mechanical ventilation, having a PaO2/FIO2 ratio < 150 mmHg, and being submitted to prone positioning. After the first prone positioning session, a 20 mmHg improvement in the PaO2/FIO2 ratio was defined as a positive response. Results: The study involved 574 patients, 412 (72%) of whom responded positively to the first prone positioning session. Multiple logistic regression showed that responders had lower Simplified Acute Physiology Score III (SAPS III)/SOFA scores and lower D-dimer levels (p = 0.01; p = 0.04; and p = 0.04, respectively). It was suggested that initial SAPS III and initial PaO2/FIO2 were predictors of oxygenation response. The mortality rate was 69.3%. Increased risk of mortality was associated with age (OR = 1.04 [95 CI: 1.01-1.06]), time to first prone positioning session (OR = 1.18 [95 CI: 1.06-1.31]), number of sessions (OR = 1.31 [95% CI: 1.00-1.72]), proportion of pulmonary impairment (OR = 1.55 [95% CI: 1.02-2.35]), and immunosuppression (OR = 3.83 [95% CI: 1.35-10.86]). Conclusions: Our results show that most patients in our sample had a positive oxygenation response after the first prone positioning session. However, the mortality rate was high, probably due to the health status and the number of comorbidities of the patients, as well as the severity of their disease. Our results also suggest that SAPS III and the initial PaO2/FIO2 predict the oxygenation response; in addition, age, time to first prone positioning, number of sessions, pulmonary impairment, and immunosuppression can predict mortality.

Keywords: Respiratory distress syndrome; Coronavirus infections; Pulmonary medicine; COVID-19; Prone position; SARS-CoV-2.

Objective: To compare the behavior of the oxygen uptake efficiency slope (OUES) with that of oxygen uptake at peak exertion (VO2peak). Methods: This was a prospective cross-sectional study involving 21 patients (15 men) with mild-to-moderate COPD undergoing spirometry, handgrip strength (HGS) testing, cardiopulmonary exercise testing, and determination of lactate at peak exertion (LACpeak). Results: Mean weight was 66.7 ± 13.6 kg, and mean age was 60.7 ± 7.8 years. With the exception of FEV1 and FEV1/FVC ratio (75.8 ± 18.6% of predicted and 56.6 ± 8.8%, respectively), all spirometric variables were normal, as was HGS. The patients exhibited significant metabolic and hemodynamic stress, as evidenced by the means (% of predicted) for VO2peak (93.1 ± 15.4), maximum HR (92.5 ± 10.4), and OUES (99.4 ± 24.4), as well as for the gas exchange rate (1.2 ± 0.1). The correlation between VO2peak and OUES was significant (r = 0.747; p < 0.0001). The correlation between HGS and VO2peak (r = 0.734; pX< 0.0001) was more significant than was that between HGS and OUES (r = 0.453; p < 0.05). Similar results were found regarding the correlations of VO2peak and OUES with MIP. Although LACpeak correlated significantly with VO2peak (r = −0.731; p < 0.0001), only LACpeak/maximum power correlated significantly with OUES (r = −0.605; p = 0.004). Conclusions: Our findings suggest that, in mild-to-moderate COPD, VO2 determinants other than overall muscle strength have a greater impact on OUES than on VO2peak.

Keywords: Respiratory function tests; Pulmonary disease, chronic obstructive; Muscle strength; Oxygen consumption.

Objectives: To evaluate the self-reported degree of adherence to treatment in patients with cystic fibrosis (CF), investigating associations with characteristics of the disease and with the degree of adherence perceived by health professionals. Methods: This was a prospective, cross-sectional study involving patients with CF monitored at a Program for Adults with CF. The degree of adherence was evaluated using a questionnaire. Patients were divided into two groups: greater degree of adherence and moderate/poor degree of adherence. Clinical data, Shwachman-Kulczycki clinical score, Brasfield radiographic score and spirometry data were obtained for all patients. Results: Out of 38 patients studied, 31 (81.6%) were classified as presenting a greater degree of adherence and 7 (18.4%) as presenting a moderate/poor degree of adherence. The self-reported patient adherence score correlated with the clinical score (r = −0.36, p = 0.028). The self-reported patient adherence score (median = 0.79) was higher than that perceived by health professionals (median = 0.71, p = 0.003). A greater degree of adherence was self-reported for respiratory therapy (by 84.2%), exercise (by 21.1%), prescribed diet (by 65.8%), pancreatic enzymes (by 96.3%), vitamins (by 79.4%), inhaled antibiotic therapy (by 76.7%) and inhaled DNase (by 79.4%). Conclusions: Self-reported adherence of patients attending a Program for Adults with CF was good. The self-reported patient adherence score correlated inversely with the clinical score. Self-reported patient adherence was greater than the adherence perceived by health professionals.

Keywords: Cystic fibrosis; Patient compliance; Therapeutics.