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.

Objective: Depression and anxiety have been documented in people with cystic fibrosis (CF), jeopardizing treatment adherence. To date, no studies have assessed the prevalence of psychosocial issues in adolescents with CF in Brazil. We sought to assess the prevalence of depression and anxiety in adolescents with CF in Brazil, as well as the impact of depression and anxiety on treatment adherence. Methods: This was a multicenter, prospective, observational, longitudinal study conducted between 2017 and 2019 at 14 CF referral centers in Brazil. We used standardized tools such as the nine-item Patient Health Questionnaire (for depression), the seven-item Generalized Anxiety Disorder scale (for anxiety), and the eight-item Morisky Medication Adherence Scale (for treatment adherence) in order to collect data on 218 CF patients at two different time points. Results: The prevalence of depression was 19.1% at time point 1 and 15.4% at time point 2. The prevalence of anxiety was 19.1% at time point 1 and 18.0% at time point 2. Depression and anxiety were significantly higher in female patients and lower in those who underwent home physiotherapy or had psychological support. Significant correlations were found between depression and anxiety at both time points, the associations being strongest at time point 1 (r = 0.68; p < 0.001). Most (66.7%) of the study participants reported low adherence to treatment, and the remainder reported either average adherence (in 28%) or high adherence (in 5.3%). Depression and anxiety showed inverse correlations with treatment adherence. Conclusions: The prevalence of depression and anxiety in adolescents with CF in Brazil appears to be similar to that reported in other countries, being higher in females and lower in those undergoing home physiotherapy or receiving psychological care. Depression and anxiety appear to correlate with lower treatment adherence. Treating psychosocial issues may effectively improve rates of treatment adherence in adolescents with CF.

Keywords: Cystic fibrosis; Anxiety; Depression; Treatment adherence and compliance; Adolescent.

Objective: To evaluate the perception of disease severity in patients with cystic fibrosis (CF), investigating its relationship with clinical score, radiographic score, respiratory function tests, adherence to treatment and perception of self-care practices. Methods: Prospective, cross-sectional study involving CF patients treated in a program for adults with CF. The perception of disease severity, adherence to treatment and reported self-care practices were evaluated by means of questionnaires. Clinical data, Shwachman-Kulczycki clinical score, Brasfield radiographic score and spirometry were obtained for all of the patients. Results: Of the 38 patients studied, 3 (7.9%) patients rated their perception of health status as well below average; 5 (13.2%), as below average; 15 (39.5%), as average; 10 (26.3%), as above average; and 5 (13.2%), as well above average. The perception of disease severity correlated significantly with clinical score (r = 0.43, p = 0.007), FVC (r = 0.34, p = 0.034), FEV1 (r = 0.38, p = 0.019) and self-care practices (r = 0.33, p = 0.044), but not with degree of adherence (r = -0.03, p = 0.842) and radiographic score (r = 0.33, p = 0.51). Conclusions: The perception of disease severity correlated with objective measurements of disease severity (clinical score and respiratory function tests) and with reported self-care practices, but not with adherence to treatment.

Keywords: Severity of illness index; Cystic fibrosis; Patient compliance; Respiratory function tests.

Evidence-based techniques have been increasingly used in the creation of clinical guidelines and the development of recommendations for medical practice. The use of levels of evidence allows the reader to identify the quality of scientific information that supports the recommendations made by experts. The objective of this review was to address current concepts related to the clinical impact, diagnosis, and treatment of Pseudomonas aeruginosa infections in patients with cystic fibrosis. For the preparation of this review, the authors defined a group of questions that would be answered in accordance with the principles of PICO-an acronym based on questions regarding the Patients of interest, Intervention being studied, Comparison of the intervention, and Outcome of interest. For each question, a structured review of the literature was performed using the Medline database in order to identify the studies with the methodological design most appropriate to answering the question. The questions were designed so that each of the authors could write a response. A first draft was prepared and discussed by the group. Recommendations were then made on the basis of the level of scientific evidence, in accordance with the classification system devised by the Oxford Centre for Evidence-Based Medicine, as well as the level of agreement among the members of the group.

Keywords: Cystic fibrosis/diagnosis; Cystic fibrosis/drug therapy; Pseudomonas aeruginosa; Evidence-based medicine.

Objective: To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life. Methods: This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores. Results: We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048). Conclusions: Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.

Keywords: Cystic fibrosis; Patient compliance; Physical therapy modalities; Quality of life.

Abstract Objective: To determine the rate of adherence to treatment with inhaled corticosteroids in patients with severe asthma, to identify predictive factors for adherence and to evaluate the relationship between adherence to treatment and parameters of clinical and functional response. Methods: Prospective cohort study of patients enrolled in the Program for the Control of Asthma and Allergic Rhinitis in the state of Bahia, Brazil. The study comprised 160 patients with severe asthma, monitored for 180 days in order to evaluate adherence (dependent variable) to the prescribed inhaled corticosteroid. Independent variables were assessed at baseline and for a six-month follow-up period by means of interviews and the completion of a standardized questionnaire. Patients recorded the missed doses in a diary. Results: Of the 160 patients, 158 completed the study. Adherence rate was 83.8%. Of the 158 patients, 112 (70.9%) were considered adherent (cut-off point: 80% of prescribed doses administered). There was a significant association between asthma control and adherence to treatment. Predictors of poor adherence were adverse effects, living far from the referral center, limited resources to pay for transportation and dose schedule. Other factors, such as depressive symptoms, religion and economic status, were not associated with poor adherence. Conclusions: Adherence to asthma treatment was high and was associated with the clinical response to treatment, in a sample of patients with severe asthma enrolled in a public program that provides free medication and the assistance of a multiprofessional specialized team in a referral center.

Keywords: Asthma; Directly observed therapy; Treatment outcome; Patient compliance; Pharmacy.

Objective: To determine the prevalence of malnutrition in patients attending an adult cystic fibrosis (CF) program and to investigate the associations of malnutrition with the clinical characteristics of those patients. Methods: This was a cross-sectional study involving patients with clinically stable CF patients (16 years of age or older). The patients underwent clinical assessment, nutritional assessments, pulmonary function tests, and pancreatic function assessment. They also completed a questionnaire regarding diet compliance. On the basis of their nutritional status, the patients were classified divided into three groups: adequate nutrition; at nutritional risk; and malnutrition. Results: The study has included 73 patients (mean age, 25.6 ± 7.3 years), 40 of whom (54.8%) were female. The mean body mass index was 21.0 ± 3.0 kg/m2 and the mean FEV1 was 59.7 ± 30.6% of predicted. In this sample of patients, 32 (43.8%), 23 (31.5%), and 18 (24.7%) of the patients were allocated to the adequate nutrition, nutritional risk, and malnutrition groups, respectively. The logistic regression analysis identified three independent factors associated with the risk of malnutrition: Shwachman-Kulczycki score, percent predicted FEV1; and age. Conclusions: Malnutrition remains a common complication in adolescents and adults with CF, despite dietary advice. Malnutrition is associated with age, clinical severity, and lung function impairment.

Keywords: Cystic fibrosis; Malnutrition; Exocrine pancreatic insufficiency; Respiratory function tests.

Objective: To profile the characteristics of adult patients with bronchiectasis, drawing comparisons between cystic fibrosis (CF) patients and those with bronchiectasis from other causes in order to determine whether it is rational to extrapolate the bronchiectasis treatment given to CF patients to those with bronchiectasis from other causes. Methods: A retrospective analysis of the medical charts of 87 patients diagnosed with bronchiectasis and under follow-up treatment at our outpatient clinic. Patients who had tuberculosis (current or previous) were excluded. We evaluated the clinical, functional, and treatment data of the patients. Results: Of the 87 patients with bronchiectasis, 38 (43.7%) had been diagnosed with CF, through determination of sweat sodium and chloride concentrations or through genetic analysis, whereas the disease was due to another etiology in 49 (56.3%), of whom 34 (39.0%) had been diagnosed with idiopathic bronchiectasis. The mean age at diagnosis was lower in the patients with CF than in those without (14.2 vs. 24.2 years; p < 0.05). The prevalence of symptoms (cough, expectoration, hemoptysis, and wheezing) was similar between the groups. Colonization by Pseudomonas aeruginosa or Staphylococcus aureus was more common in the CF patients (82.4 vs. 29.7% and 64.7 vs. 5.4%, respectively). Conclusions: The causes and clinical manifestations of bronchiectasis are heterogeneous, and it is important to identify the differences. It is crucial that these differences be recognized so that new strategies for the management of patients with bronchiectasis can be developed.

Keywords: Cystic fibrosis; Bronchiectasis/diagnosis; Bronchiectasis/therapy; Respiratory function tests.

Cystic fibrosis-related diabetes (CFRD) is the principal extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adult cystic fibrosis patients. The number of cystic fibrosis patients who develop diabetes is increasing in parallel with increases in life expectancy. The aim of this study was to review the physiopathology, clinical presentation, diagnosis and treatment of CFRD. A bibliographic search of the Medline and Latin American and Caribbean Health Sciences Literature databases was made. Articles were selected from among those published in the last twenty years. Insulin deficiency, caused by reduced β-cell mass, is the main etiologic mechanism, although insulin resistance also plays a role. Presenting features of type 1 and type 2 diabetes, CFRD typically affects individuals of approximately 20 years of age. It can also be accompanied by fasting, non-fasting or intermittent hyperglycemia. Glucose intolerance is associated with worsening of nutritional status, increased morbidity, decreased survival and reduced pulmonary function. Microvascular complications are always present, although macrovascular complications are rarely seen. An oral glucose tolerance test is recommended annually for patients ≥ 10 years of age and for any patients presenting unexplained weight loss or symptoms of diabetes. Patients hospitalized with severe diseases should also be screened. If fasting hyperglycemia persists for more than 48 h, insulin therapy is recommended. Insulin administration remains the treatment of choice for diabetes and fasting hyperglycemia. Calories should not be restricted, and patients with CFRD should be managed by a multidisciplinary team.

Keywords: Cystic fibrosis; Diabetes mellitus.

Objective: To test the hypothesis that disease severity in patients with cystic fibrosis (CF) is correlated with an increased risk of sleep apnea. Methods: A total of 34 CF patients underwent clinical and functional evaluation, as well as portable polysomnography, spirometry, and determination of IL-1β levels. Results: Mean apnea-hypopnea index (AHI), SpO2 on room air, and Epworth Sleepiness Scale score were 4.8 ± 2.6, 95.9 ± 1.9%, and 7.6 ± 3.8 points, respectively. Of the 34 patients, 19 were well-nourished, 6 were at nutritional risk, and 9 were malnourished. In the multivariate model to predict the AHI, the following variables remained significant: nutritional status (β = −0.386; p = 0.014); SpO2 (β = −0.453; p = 0.005), and the Epworth Sleepiness Scale score (β = 0.429; p = 0.006). The model explained 51% of the variation in the AHI. Conclusions: The major determinants of sleep apnea were nutritional status, SpO2, and daytime sleepiness. This knowledge not only provides an opportunity to define the clinical risk of having sleep apnea but also creates an avenue for the treatment and prevention of the disease.

Keywords: Cystic fibrosis; Oxygenation; Sleep apnea, obstructive.

Background: Pneumonia is a common disease with a high mortality rate, being the sixth leading cause of death among elderly people in the USA and the fifth among those in Brazil. Initial treatment of pneumonia is usually empirical since the ethiological agent is identified in only approximately 50% of cases. Therefore, several scientific societies have defined some guidelines for initial antimicrobial therapies. Objectives: This study evaluated adherence to the guidelines set forth by the Consenso Brasileiro sobre Pneumonias (Brazilian Consensus on Pneumonia) for treatment of community-acquired pneumonia in hospitalized elderly patients. Method: Fifty-four patients, aged 60 or over, hospitalized at Londrina University Hospital with community-acquired pneumonia between 2 August 1999 and 2 August 2000 were evaluated. Whether their treatment adhered or did not adhere to guidelines, the patients were compared in terms of 30-day mortality, average time for clinical stabilization, average length of hospital stay, cost of treatment and severity score. Results: The average age was 74.1, and 61.1% of the patients were treated in accordance with the Brazilian guidelines for treatment of community-acquired pneumonia. There were no differences in length of hospital stays, cost of treatment, time to clinical stability and severity score between the two groups (adherent and non-adherent). However, there was a difference in mortality. The rate of mortality was higher in patients with pneumonia severity index (PSI) scores of IV or V who were treated according to the guidelines than in those with the same score whose treatment was non-adherent (p = 0.04). In general, PSI score was related to mortality. The mortality rate among patients with scores of II and III was 9.5%, compared with 30.3% in patients with scores of IV and V. Conclusion: Adherence to the Brazilian guidelines for treatment of community-acquired pneumonia in elderly patients was satisfactory, and there was no difference in results between both groups, except for the higher mortality rate found for patients with higher PSI scores who were treated according to the guidelines. A positive correlation was found between PSI score and mortality.

Keywords: Pneumonia. Health Planning Guidelines. Community-Acquired Infections. Health Services for the Aged.

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.

Objective: To assess the quality of life (QoL) of patients with cystic fibrosis (CF) followed at a university referral center for CF. Methods: A cross-sectional study involving application of the Cystic Fibrosis Questionnaire (CFQ) and Shwachman score in CF patients between April of 2008 and June of 2009. Results: The sample consisted of 75 patients. The mean age was 12.5 ± 5.1 years (range, 6.1-26.4 years). The patients were divided into three groups by age in years: group I (< 12), II (12-14), and III (≥ 14). The highest and lowest CFQ scores were for the nutrition domain in group III (89.3 ± 16.2) and the social domain in group II (59.5 ± 22.3), respectively. Groups I and III differed significantly regarding the treatment domain (p = 0.001). Regarding Shwachman scores, there were significant differences between patients scoring ≤ 70 and those scoring > 70 in the social (group I; p = 0.045), respiratory (group II; p = 0.053), and digestive (p = 0.042) domains. In group III, severity did not correlate with QoL. In groups I and II, patients with an FEV1 < 80% of predicted did not differ from other patients for any CFQ domain. However, in group III, values for the following domains were significantly lower in patients with an FEV1 < 80%: physical (p = 0.012); body image (p = 0.031); respiratory (p = 0.023), emotional (p = 0.041); and social role (p = 0.024). Conclusions: It is important to assess QoL in CF patients, because it can improve treatment compliance.

Keywords: Cystic fibrosis; Quality of life; Questionnaires.

Objective: Cystic fibrosis (CF) is a chronic multisystemic hereditary disease for which a multidisciplinary approach must be taken. The objective of this study was to show the evolution of a group of patients with CF after the implementation of multidisciplinary treatment. Methods: A retrospective study involving 19 patients (6-29 years of age) under clinical follow-up treatment at the University of São Paulo at Ribeirão Preto School of Medicine Hospital das Clínicas, located in the city of Ribeirão Preto, Brazil. The patients were divided into two groups: 6-12(6-12 years of age) and 13+ (> 12 years of age). We collected data regarding body mass index (BMI), Zscore, Shwachman score (SS), number of exacerbations/year, chronic colonization by Pseudomonas aeruginosa, and spirometric measurements (FVC, FEV1, FEV1%, and FEF25-75%). Data were collected at two different time points (before and after the implementation of the multidisciplinary treatment) and were analyzed with the Wilcoxon signed rank test. Results: The median age at the onset of symptoms was 10 months. In the 6-12 group, only BMI and FVC increased significantly. Although the other spirometric values increased, the differences were not significant. In the 13+ group, there were no significant differences between the two time points. There was a borderline significant decrease in SS and less than significant decreases in the spirometric measurements. However, the number of patients with alterations in volumes and flows decreased in both groups. Conclusions: Although our patient sample was small, the lack of changes in the spirometric parameters might reflect clinical and functional stability. In all of the patients evaluated, clinical, functional, and nutritional parameters remained stable throughout the study period. The implementation of a multidisciplinary approach might have contributed to this result.

Keywords: Cystic fibrosis; Spirometry; Body mass index; Physical therapy modalities.

Objective: Although various strategies have been proposed for eradicating Pseudomonas aeruginosa in patients with cystic fibrosis (CF), only a few employ multistep treatment in children colonized by that pathogen for the first time. The aim of this study was to describe the effectiveness of a three-phase eradication protocol, initiated after the first isolation of P. aeruginosa, in children with CF in Brazil. Methods: This was a retrospective real-life study in which we reviewed the medical records of pediatric CF patients in whom the eradication protocol was applied between June of 2004 and December of 2012. The three-phase protocol was guided by positive cultures for P. aeruginosa in airway secretions, and the treatment consisted of inhaled colistimethate and oral ciprofloxacin. Success rates were assessed after each phase, as well as cumulatively. Results: During the study period, 47 episodes of P. aeruginosa colonization, in 29 patients, were eligible for eradication. Among the 29 patients, the median age was 2.7 years, 17 (59%) were male, and 19 (65%) had at least one F508del allele. All 29 patients completed the first phase of the protocol, whereas only 12 and 6 completed the second and third phases, respectively. Success rates for eradication in the three treatment phases were 58.6% (95% CI: 40.7-74.5), 50.0% (95% CI: 25.4-74.6), and 66.7% (95% CI: 30.0-90.3), respectively. The cumulative success rate was 93.1% (95% CI: 78.0-98.1). Treatment failure in all three phases occurred in only 2 patients. Conclusions: In this sample of patients, the multistep eradication protocol was effective and had a high success rate.

Keywords: Cystic fibrosis/therapy; Cystic fibrosis/prevention & control; Pseudomonas aeruginosa; Treatment outcome.

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.

Objetivo: Medir a resistência de vias aéreas utilizando a técnica de resistência do interruptor (Rint) em pacientes com fibrose cística (FC) e correlacioná-la com parâmetros espirométricos, assim como avaliar a acurácia de Rint para determinar a resposta das vias aéreas a um broncodilatador. Métodos: Estudo transversal com 38 crianças e adolescentes com FC acompanhados no Ambulatório de FC do Hospital São Lucas, em Porto Alegre (RS). Após a determinação de Rint, os pacientes foram submetidos à espirometria. Para a avaliação da resposta ao broncodilatador, as medições foram repetidas após o uso de salbutamol inalatório. Resultados: Houve uma forte correlação entre o inverso de Rint e VEF1 (r = 0,8; p < 0,001) e moderadas correlações entre o inverso de Rint e FEF25-75% (r = 0,74; p < 0,001) e entre o inverso de Rint e índice de massa corpórea (r = 0,62; p < 0,001). A curva ROC foi utilizada na comparação da resposta ao broncodilatador determinada por Rint com aquela determinada por valores espirométricos. Para um ponto de corte de −28% para Rint, a área sob a curva foi de 0,75, com uma sensibilidade de 66% e uma especificidade de 82%. Conclusões: Nossos achados indicam que Rint apresenta uma boa correlação com parâmetros espirométricos, embora a técnica Rint não tenha sido suficientemente acurada para substituir a espirometria na avaliação da resposta ao broncodilatador.

Keywords: Testes de função respiratória; Fibrose cística; Espirometria; Resistência das vias respiratórias.