Objective: To determine the submaximal exercise capacity of patients with cystic fibrosis (CF) by means of the 6-minute walk test (6MWT), correlating the results with clinical score, nutritional status, radiographic score, and pulmonary function tests. Methods: This was a prospective, cross-sectional study involving patients aged 16 or older enrolled in a program for adults with CF. The patients were submitted to clinical evaluation, determination of maximal respiratory pressures, 6MWT, spirometry, and chest X-ray. Results: The study comprised 41 patients. The mean age was 23.7 ± 6.5 years, and the mean forced expiratory volume in one second (FEV1) was 55.1 ± 27.8%. On the 6MWT, 30 (73.2%) of the patients covered a distance (mean, 556.7 ± 76.5 m) that was less than the predicted normal value. The distance walked did not correlate significantly with body mass index, clinical score, radiographic score, maximal respiratory pressures, peripheral oxygen saturation at rest, desaturation during the 6MWT, sensation of dyspnea, or fatigue, although it did so with age at diagnosis, FEV1 in liters, and forced vital capacity in liters. Worsening of pulmonary function was associated with greater desaturation during the 6MWT. Conclusion: This study showed that most patients attending an adult CF program had reduced submaximal exercise capacity. The 6MWT can be valuable for identifying patients who might experience oxygen desaturation and physical impairment in daily activities.

Keywords: Cystic fibrosis; Respiratory function tests; Exercise tolerance.

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 determine the prevalence of pulmonary hypertension (PH) in patients with cystic fibrosis (CF), to compare clinical characteristics, radiographic scores, pulmonary function, and echocardiographic parameters in patients with and without PH, and to correlate echocardiographic findings with clinical characteristics, radiographic scores, and pulmonary function. Methods: This was a prospective, cross-sectional study involving clinically stable patients (aged 16 or older) enrolled in an adult CF program. The patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, and chest X-rays. Results: Tricuspid regurgitant jet velocity (TRV) was obtained in 37 of the 40 patients studied. The prevalence of PH was 49% with a TRV cut-off of 2.5 m/s (18 patients) and 30% with a TRV cut-off of 2.8 m/s (11 patients). Peripheral oxygen saturation (SpO2) at rest, clinical score, forced expiratory volume in one second (FEV1), and forced vital capacity (FVC) were significantly lower in the group with PH. The TRV was found to correlate significantly with SpO2 at rest (p < 0.001), clinical score (p < 0.001), radiographic score (p = 0.030), FEV1 in liters (p < 0.001) and in % of predicted (p < 0.001), and FCV in liters (p = 0.008) and in % of predicted (p = 0.001). The single best predictor of TRV was SpO2 at rest (p < 0.001). Conclusion: The high prevalence of PH in the CF patients studied suggests that PH should be considered in the evaluation and follow-up treatment of such patients. The best predictor of PH was SpO2 at rest.

Keywords: Cystic fibrosis; Tricuspid valve/physiopathology; Hypertension, pulmonary; Echocardiography, Doppler.

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.

Objective: To identify the predictive factors of oxygen desaturation during the six-minute walk test (6MWT) in patients with cystic fibrosis (CF). Methods: Prospective cross-sectional study involving clinically stable patients with CF aged ≥ 10 years. The patients were submitted to nutritional evaluations, oral glucose tolerance tests, pulmonary function tests, chest X-rays and 6MWTs. Results: The study included 88 patients (43 females and 45 males; mean age, 19.9 ± 7.2 years; mean FEV1, 65.4 ± 28.4%). We observed oxygen desaturation in 13 patients (OD+ group) and no oxygen desaturation in 75 (OD− group). In comparison with OD− group patients, OD+ group patients presented higher mean age (p = 0.004), worse clinical score (p < 0.001), worse radiological score (p < 0.001), higher incidence of glucose intolerance (p = 0.004), lower incidence of methicillin-sensitive Staphylococcus aureus infection (p < 0.001), higher incidence of methicillin-resistant S. aureus infection (p = 0.016), higher incidence of Pseudomonas aeruginosa infection (p = 0.008), lower mean resting SpO2 (p < 0.001) and lower mean FEV1 (p < 0.001). In the logistic regression analysis, oxygen desaturation during the 6MWT correlated with resting SpO2 (OR = 0.305, p < 0.001) and FEV1 (OR = 0.882, p = 0.025). The parameters maximizing the predictive value for oxygen desaturation were resting SpO2 < 96% and FEV1 < 40%. In this sample, 15% of the patients with CF aged ≥ 10 years presented oxygen desaturation during the 6MWT. Conclusions: Resting SpO2 < 96% and FEV1 < 40% can predict oxygen desaturation during the 6MWT.

Keywords: Cystic fibrosis; Respiratory function tests; Exercise tolerance.

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 determine whether the variation in lung function over one year is associated with worse clinical outcomes, as well as with a decline in lung function in the following years, in patients with cystic fibrosis (CF). Methods: This was a retrospective study involving CF patients (4-19 years of age), evaluated over a three-year period. We evaluated demographic characteristics, chronic Pseudomonas aeruginosa infection, antibiotic use, hospitalization, six-minute walk distance (6MWD), and lung function. The inclusion criterion was having undergone pulmonary function testing at least three times in the first year and at least once in each of the next two years. Results: We evaluated 35 CF patients. The variation in FEV1 in the first year (ΔFEV1) was greater among those who, in the third year, showed reduced FEV1, had a below-average 6MWD, or were hospitalized than among those with normal FEV1, normal 6MWD, or no hospital admissions, in that same year (p < 0.05), although no such difference was found for antibiotic use in the third year. Subjects showing a ΔFEV1 ≥ 10% also showed a greater decline in FEV1 over the two subsequent years (p = 0.04). The ΔFEV1 also showed an inverse correlation with absolute FEV1 in the third year (r = −0.340, p = 0.04) and with the rate of FEV1 decline (r = −0.52, p = 0.001). Linear regression identified ΔFEV1 as a predictor of FEV1 decline (coefficient of determination, 0.27). Conclusions: Significant variation in lung function over one year seems to be associated with a higher subsequent rate of FEV1 decline and worse clinical outcomes in CF patients. Short-term ΔFEV1 might prove useful as a predictor of CF progression in clinical practice.

Keywords: Cystic fibrosis; Respiratory function tests; Disease progression; Hospitalization; Forced expiratory volume.

Pneumothorax is a common complication in cystic fibrosis and is associated with worsening of lung function. However, bilateral simultaneous pneumothorax in cystic fibrosis is a rare condition. We describe the case of a 17-year-old female with cystic fibrosis who presented with spontaneous pneumothorax. Clinically, she presented right-sided chest pain and progressive breathlessness. The pneumothorax failed to resolve after the initial treatment (chest drainage). However, the patient was later successfully treated with additional chest drainage and talc pleurodesis. We also discuss the etiology and management of pneumothorax in patients with cystic fibrosis, since pneumothorax is associated with increased morbidity and mortality among such patients.

Keywords: Cystic fibrosis/physiopathology; Pneumothorax; Pleurodesis.

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: Pulmonary rehabilitation is widely recommended for the treatment of chronic obstructive pulmonary disease. Objective: To evaluate the efficacy of pulmonary rehabilitation in improving exercise capacity, respiratory muscle strength and quality of life of chronic obstructive pulmonary disease patients. Method: This was an open, non-randomized clinical trial involving 27 clinically stable ex-smokers with chronic obstructive pulmonary disease who were enrolled in a pulmonary rehabilitation program. All were evaluated before and after pulmonary rehabilitation. Results: Mean age was 65 ± 5 years, mean body mass index was 25 ± 4 kg/m2, mean forced expiratory volume in one second was 55 ± 25% of predicted, mean ratio between forced expiratory volume in one second and forced vital capacity was 50 ± 12%, and mean arterial oxygen tension was 70 ± 7 mmHg. Comparison of pre- and post-pulmonary rehabilitation values revealed improvement in the distance walked in the 6-minute walk test (513 ± 99 m vs. 570 ± 104 m), maximum upper limb load (2 ± 1 kg vs. 3 ± 1 kg) and maximal inspiratory pressure (-89 ± 23 cmH2O vs. -102 ± 23 cmH2O), as well as in the activity domain, impact domain and total score on the Saint George's Respiratory Questionnaire. Conclusion: Pulmonary rehabilitation, when performed with care and with a focus on physical training, is efficacious in increasing not only the distance walked in the 6-minute walk test but maximum upper limb load, maximal inspiratory pressure and quality of life as well.

Keywords: Key words: Pulmonary rehabilitation. Chronic obstructive pulmonary disease. Quality of life. Respiratory muscles.

Objective: To investigate the validity of field walking tests to identify exercise-induced hypoxemia and to compare cardiorespiratory responses and perceived effort between laboratory-based and field-based exercise tests in subjects with bronchiectasis. Methods: This was a cross-sectional study involving 72 non-oxygen-dependent participants (28 men; mean age = 48.3 ± 14.5 years; and mean FEV1 = 54.1 ± 23.4% of the predicted value). The participants underwent cardiopulmonary exercise testing (CPET) on a treadmill and constant work-rate exercise testing (CWRET) on the same day (1 h apart). In another visit, they underwent incremental shuttle walk testing (ISWT) and endurance shuttle walk testing (ESWT; 1 h apart). Desaturation was defined as a reduction in SpO2 ≥ 4% from rest to peak exercise. Results: CPET results were compared with ISWT results, as were CWRET results with ESWT results. There was no difference in the magnitude of desaturation between CPET and ISWT (−7.7 ± 6.3% vs. −6.6 ± 5.6%; p = 0.10) and between CWRET and ESWT (−6.8 ± 5.8% vs. −7.2 ± 6.3%; p = 0.50). The incremental tests showed an agreement in the magnitude of desaturation in the desaturation and no desaturation groups (42 and 14 participants, respectively; p < 0.01), as did the endurance tests (39 and 16 participants; p < 0.01). The magnitude of desaturation was similar among the participants who did or did not reach at least 85% of the maximum predicted HR. Conclusions: Field exercise tests showed good precision to detect desaturation. Field tests might be an alternative to laboratory tests when the clinical question is to investigate exercise-induced desaturation in subjects with bronchiectasis.

Keywords: Bronchiectasis; Oxygen; Hypoxia; Exercise test.

Step tests are typically used to assess exercise capacity. Given the diversity of step tests, the aim of this review was to describe the protocols that have been used in healthy subjects and in patients with chronic lung disease. Step tests for use in healthy subjects have undergone a number of modifications over the years. In most step tests, the duration is variable (90 s-10 min), but the step height (23.0-50.8 cm) and stepping rate (22.5-35.0 steps/min) remain constant throughout the test. However, the use of a fixed step height and constant stepping rate might not provide adequate work intensity for subjects with different levels of fitness, the workload therefore being above or below individual capacity. Consequently, step test protocols have been modified by introducing changes in step heights and stepping rates during the test. Step tests have been used in patients with chronic lung diseases since the late 1970s. The protocols are quite varied, with adjustments in step height (15-30 cm), pacing (self-paced or externally paced), and test duration (90 s-10 min). However, the diversity of step test protocols and the variety of outcomes studied preclude the determination of the best protocol for use in individuals with chronic lung disease. Shorter protocols with a high stepping rate would seem to be more appropriate for assessing exercise-related oxygen desaturation in chronic lung disease. Symptom-limited testing would be more appropriate for evaluating exercise tolerance. There is a need for studies comparing different step test protocols, in terms of their reliability, validity, and ability to quantify responses to interventions, especially in individuals with lung disease.

Keywords: Pulmonary disease, chronic obstructive; Asthma; Cystic fibrosis; Idiopathic pulmonary fibrosis; Exercise tolerance; Exercise test.

Objectives: To examine the relationship between chronic bacterial infection and pulmonary hypertension, using Doppler echocardiography, in patients with cystic fibrosis (CF). Methods: A prospective cross-sectional study involving CF patients (≥ 16 years of age) admitted to a program for adults with the disease. The study included 40 patients with a mean age of 23.7 ± 6.3 years. Patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, chest X-rays and sputum cultures of Pseudomonas aeruginosa and Burkholderia cepacia. Results: In terms of the following variables, no significant differences were found between P. aeruginosapositive patients and P. aeruginosa-negative patients: clinical score (p = 0.472); forced expiratory volume in one second (FEV1; p = 0.693); radiological score (p = 0.760); tricuspid regurgitant jet velocity (TRV, p = 0.330); diameter of the right ventricle (DRV, p = 0.191); and right ventricular/pulmonary artery (RV/PA) systolic acceleration time (SAT, p = 0.330). B. cepacia-positive patients presented significantly lower FEV1 than did B. cepacia-negative patients (p = 0.011). No significant differences were found between B. cepacia-positive patients and B. cepacia-negative patients regarding the following variables: clinical score (p = 0.080); radiological score (p = 0.760); TRV (p = 0.613); DRV (p = 0.429); and RV/PA SAT (p = 0.149). Conclusions: Chronic infection with P. aeruginosa or B. cepacia presented no association with pulmonary hypertension in adult CF patients. Pulmonary function was worse in B. cepacia-positive patients than in P. aeruginosa-positive patients.

Keywords: Cystic fibrosis; Bacterial infections; Hypertension, pulmonary; Echocardiography, Doppler.

Objective: To determine the relationship between nutritional status and dietary intake in patients with cystic fibrosis. Methods: Cross-sectional study involving 85 cystic fibrosis patients between 6 and 18 years of age. Dietary intake was evaluated by the 3-day diet record (weighing the food consumed). The outcome measures were the following nutritional status indicators: weight/height (W/H%) percentage, body mass index (BMI) percentiles, Z score for weight/age (W/A), Z score for height/age (H/A) and percentage of dietary intake compared with the Recommended Dietary Allowance (RDA). Results: The prevalence of well-nourished patients was 77.7%, using BMI above the 25th percentile as the cut-off value, and the W/H% was above 90% in 83.5%. The mean dietary intake, evaluated in 82 patients, was 124.5% of the RDA. In the univariate logistic regression analyses, we found a significant association between the independent variable calorie intake and the Z score for W/A. The multivariate analysis, based on the Z score for H/A and adjusted for FEV1, methicillin-resistant Staphylococcus aureus colonization and number of hospitalizations, demonstrated that a 1% increase in the calorie intake decreases the chance of having short stature by 2% (OR: 0.98; 95% CI: 0.96-1.00). Maternal level of education showed a borderline association (p = 0.054). Conclusions: The prevalence of malnutrition was low in this sample of patients. The study model demonstrated an association between dietary intake and nutritional status. Dietary intake was a predictive factor of statural growth in patients with cystic fibrosis.

Keywords: Cystic fibrosis; Nutritional status; Diet records; Child; Adolescent.

Background: Cystic fibrosis (CF) is a disease marked by airway inflammation and airflow obstruction, resulting in air trapping in the lungs. Objective: To assess the associations between airflow limitation, pulmonary volume and X-ray findings in patients with cystic fibrosis. Method: A cross-sectional retrospective study. Review of spirometric, plethysmographic, and chest X-ray findings of outpatients (age ³ 16 years). The airflow findings were classified as within normal limits or as airflow obstruction: mild, moderate or severe obstructive alteration. Results: A total of 23 patients (15 male and eight female; mean age, 21 ± 5.9 years) were studied. Six of them were within normal limits, four had a mild, five had a moderate, and eight had a severe obstructive alteration. There was an association between airflow limitation and the increase of residual volume (p = 0.006) and also with the Brasfield score (p = 0.001), but not with the total lung capacity (p = 0.33). There was a correlation between residual volume and Brasfield score (r = 0,73, p = 0,002), but not with the total pulmonary capacity. Moreover, according to X-ray criteria, the air trapping was correlated only with the residual volume (p = 0.006). Conclusion: In patients with cystic fibrosis (age ³ 16 years), the progressive airflow limitation is accompanied by an increase in residual volume, while the total pulmonary capacity remains normal or tends to decrease. The X-ray score was associated with airflow limitation and residual volume, but not with total lung capacity.

Keywords: Cystic fibrosis. Pulmonary disease. Lung volume measurements. Radiography, thoracic.

Objetivo: Analisar as características funcionais pulmonares, a resposta farmacodinâmica a um broncodilatador e sua prescrição em pacientes com diagnóstico de fibrose cística (FC). Métodos: Estudo de coorte retrospectivo de pacientes (6-18 anos) com diagnóstico de FC acompanhados em um centro de referência, capazes de realizar testes de função pulmonar (TFP) entre 2008 e 2010. Foram analisados CVF, VEF1 e FEF25-75%, em percentual do previsto, antes e após prova broncodilatadora (pré-BD e pós-BD, respectivamente) de 312 TFP. Foram realizadas ANOVA para medidas repetidas e comparações múltiplas. Resultados: Foram incluídos no estudo 56 pacientes. Desses, 37 e 19, respectivamente, tinham resultados de TFP entre 2008 e 2010 e apenas em 2009-2010, formando dois grupos. No grupo com TFP nos três anos estudados, houve redução significativa em VEF1 pós-BD em 2008-2010 (p = 0,028) e 2009-2010 (p = 0,036) e em FEF25-75% pré-BD e pós-BD em todas as comparações múltiplas (2008 vs. 2009; 2008 vs. 2010; e 2009 vs. 2010). No grupo com TFP apenas em 2009-2010, não houve diferenças significativas em nenhuma das comparações das variáveis estudadas. Dos 312 TFP, somente 24 (7,7%) apresentaram resposta significativa ao broncodilatador e pertenciam a pacientes sem prescrição de broncodilatador durante o período estudado. Conclusões: Houve perda funcional, com indicação de doença pulmonar progressiva, nos pacientes com FC estudados. Houve maiores alterações no FEF25-75%, sugerindo o comprometimento inicial de vias aéreas menores.

Keywords: Fibrose cística; Testes de função respiratória; Broncodilatadores.

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: Bone disease is a common comorbidity in patients with cystic fibrosis (CF). We sought to determine risk factors and identify potential biochemical markers for CF-related bone disease (CFBD) in a unique cohort of CF patients with end-stage lung disease undergoing lung transplantation (LTx) evaluation. Methods: All of the CF patients who were evaluated for LTx at our center between November of 1992 and December of 2010 were included in the study. Clinical data and biochemical markers of bone turnover, as well as bone mineral density (BMD) at the lumbar spine and femoral neck, were evaluated. Spearman's rho and multivariate logistic regression analysis were used. Results: A total of 102 adult CF patients were evaluated. The mean age was 28.1 years (95% CI: 26.7-29.5), and the mean body mass index was 17.5 kg/m2 (95% CI: 17.2-18.2). Mean T-scores were −2.3 and −1.9 at the lumbar spine and femoral neck, respectively, being lower in males than in females (−2.7 vs. −2.0 at the lumbar spine and −2.2 vs. −1.7 at the femoral neck). Overall, 52% had a T-score of < −2.5 at either skeletal site. The homozygous Phe508del genotype was found in 57% of patients without osteoporosis and in 60% of those with low BMD. Mean T-scores were not particularly low in patients with severe CFTR mutations. Although the BMI correlated with T-scores at the femoral neck and lumbar spine, serum 25-hydroxyvitamin D and parathyroid hormone levels did not. Conclusions: CFBD is common in CF patients with end-stage lung disease, particularly in males and patients with a low BMI. It appears that CF mutation status does not correlate with CFBD. In addition, it appears that low BMD does not correlate with other risk factors or biochemical parameters. The prevalence of CFBD appears to have recently decreased, most likely reflecting increased efforts at earlier diagnosis and treatment.

Keywords: Lung transplantation; Cystic fibrosis; Bone density; Osteoporosis.

Objective: To determine whether the quality of life of adolescents with asthma correlates with parameters obtained prior to and after the six-minute step test (6MST); spirometric results after the 6MST; and level of physical activity. Methods: Nineteen adolescents with asthma, ranging from 11-15 years of age, were assessed with spirometry, 6MST, the International Physical Activity Questionnaire (IPAQ), the Pediatric Asthma Quality of Life Questionnaire (PAQLQ), and the 10-point Borg category-ratio scale. Results: Sensation of dyspnea correlated negatively with the total PAQLQ score (r = −0.54) and with the scores of its activity limitation (AL) and symptoms domains (r = −0.64 and r = −0.63, respectively), leg fatigue also correlating negatively with those same domains (r = −0.49 and r = −0.56, respectively). The total IPAQ score correlated with the total PAQLQ score (r = 0.47) and with the PAQLQ AL domain (r = 0.51); IPAQ time spent walking correlated with the PAQLQ symptoms domain (r = 0.45); and IPAQ time spent in vigorous activity correlated with the AL domain (r = 0.50). In the regression analysis, only sensation of dyspnea remained significantly correlated with the total PAQLQ score and the PAQLQ AL domain; leg fatigue remained significantly correlated with the symptoms domain. Conclusions: Higher levels of physical activity indicate better quality of life, as do lower perception of dyspnea and less leg fatigue. The 6MST proved to be a viable option for evaluating exercise capacity in adolescents with asthma, because it reflects the discomfort that asthma causes during activities of daily living.