Objective: To compare the absolute serum von Willebrand factor (vWF) levels and relative serum vWF activity in patients with clinically stable COPD, smokers without airway obstruction, and healthy never-smokers. Methods: The study included 57 subjects, in three groups: COPD (n = 36); smoker (n = 12); and control (n = 9). During the selection phase, all participants underwent chest X-rays, spirometry, and blood testing. Absolute serum vWF levels and relative serum vWF activity were obtained by turbidimetry and ELISA, respectively. The modified Medical Research Council scale (cut-off score = 2) was used in order to classify COPD patients as symptomatic or mildly symptomatic/asymptomatic. Results: Absolute vWF levels were significantly lower in the control group than in the smoker and COPD groups: 989 ± 436 pg/mL vs. 2,220 ± 746 pg/mL (p < 0.001) and 1,865 ± 592 pg/mL (p < 0.01). Relative serum vWF activity was significantly higher in the COPD group than in the smoker group (136.7 ± 46.0% vs. 92.8 ± 34.0%; p < 0.05), as well as being significantly higher in the symptomatic COPD subgroup than in the mildly symptomatic/asymptomatic COPD subgroup (154 ± 48% vs. 119 ± 8%; p < 0.05). In all three groups, there was a negative correlation between FEV1 (% of predicted) and relative serum vWF activity (r2 = −0.13; p = 0.009). Conclusions: Our results suggest that increases in vWF levels and activity contribute to the persistence of systemic inflammation, as well as increasing cardiovascular risk, in COPD patients.

Keywords: von Willebrand factor; Pulmonary disease, chronic obstructive; Endothelial cells.

Objective: To determine the prevalence of atopy and to evaluate clinical, laboratory, and radiological profiles in patients with COPD. Methods: This was a cross-sectional study involving outpatients with stable COPD (defined by the clinical history and a post-bronchodilator FEV1/FVC < 70% of the predicted value). The patients completed a questionnaire regarding clinical characteristics and atopy, after which they underwent nasal lavage cytology, skin prick testing, chest X-rays, arterial blood gas analyses, and determination of total serum IgE. Results: Of the 149 subjects studied, 53 (35.6%), 49 (32.8%), and 88 (59.1%) presented with nasal eosinophilia, a positive skin prick test result, and symptoms of allergic rhinitis, respectively. Correspondence analysis confirmed these findings, showing two distinct patterns of disease expression: atopy in patients with COPD that was less severe; and no evidence of atopy in those with COPD that was more severe (reduced FEV1 and hyperinflation). There was a statistically significant association between nasal eosinophilia and a positive bronchodilator response. Conclusions: Using simple and reproducible methods, we were able to show that there is a high frequency of atopy in patients with COPD. Monitoring inflammation in the upper airways can be a useful tool for evaluating respiratory diseases in the elderly and in those with concomitant asthma and COPD, a clinical entity not yet fully understood.

Keywords: Pulmonary disease, chronic obstructive; Allergy and immunology; Nasal lavage fluid; Asthma; Rhinitis, allergic, perennial.

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 identify symptoms of dysphagia in individuals with COPD, based on their responses on a self-perception questionnaire. Methods: The study comprised 35 individuals with COPD and 35 healthy individuals, matched for age and gender. The study group was assessed regarding COPD severity; sensation of dyspnea; body mass index (BMI); and symptoms of dysphagia. The control group was assessed regarding BMI and symptoms of dysphagia. Results: The most common symptoms of dysphagia in the study group were pharyngeal symptoms/airway protection (p < 0.001); esophageal symptoms/history of pneumonia (p < 0.001); and nutritional symptoms (p < 0.001). Positive correlations were found between the following pairs of variables: FEV1 and BMI (r = 0.567; p < 0.001); pharyngeal symptoms/airway protection and dyspnea (r = 0.408; p = 0.015); and esophageal symptoms/history of pneumonia and pharyngeal symptoms/airway protection (r = 0.531; p = 0.001). There was a negative correlation between nutritional symptoms and BMI (r = −0.046; p < 0.008). Conclusions: Our results show that the individuals with COPD presented with symptoms of dysphagia that were associated with the pharyngeal and esophageal phases of swallowing, as well as with the mechanism of airway protection, a history of pneumonia, and nutritional symptoms.

Keywords: Deglutition disorders; Pulmonary disease, chronic obstructive; Pathological conditions, signs and symptoms.

Objective: To measure exhaled carbon monoxide (COex) levels in smokers with and without COPD. Methods: Smokers treated at outpatient clinics of São Lucas Hospital in the city of Porto Alegre, Brazil, between September of 2007 and March of 2009 were invited to participate in this study. The participants completed a questionnaire regarding demographic and epidemiologic characteristics and were submitted to spirometry, as well as to determination of COex and urinary cotinine levels. The participants were divided into two groups: those with COPD and those without COPD. Results: The study involved 294 smokers, of whom 174 (59.18%) had been diagnosed with COPD. All of the participants presented with urinary cotinine levels > 50 ng/mL. Smokers with COPD presented significantly higher median values for age and pack-years than did those without COPD (p < 0.001 and p = 0.026, respectively). No other statistically significant differences were found. When adjusted for gender, age at smoking onset, number of cigarettes/day and urinary cotinine level, the mean values of COex were higher, but not statistically so, in the COPD group than in the non-COPD group (17.8 ± 0.6 ppm and 16.6 ± 0.7 ppm, respectively; p = 0.200). The differences remained nonsignificant when plotted logarithmically. A wide dispersion of COex values was found when the participants were classified by FEV1 level (r = −0.06; p = 0.53) or by Global Initiative for Chronic Obstructive Lung Disease classification (r = 0.08; p = 0.34). The proportions of false-negative results for smoking were 18.4% and 6.7%, respectively, in the COPD and non-COPD groups (p = 0.007). Conclusions: Since COex values did not differ significantly between smokers with COPD and those without, there seem to be no major contraindications to their use in smokers with COPD.

Keywords: Carbon monoxide; Smoking cessation; Pulmonary disease, chronic obstructive.

Bronchoscopy with bronchoalveolar lavage, bronchial brush, and transbronchial biopsy are useful methods for the diagnosis of pulmonary diseases in patients infected with the human immunodeficiency virus (HIV). However, data on the accuracy of these techniques are conflicting. In order to estimate the sensitivity of the bronchoalveolar lavage, bronchial and transbronchial biopsy in pulmonary disease diagnosis in patients infected with HIV, the authors studied 71 consecutive patients, most of them with respiratory symptoms up to 15 days (58%) and radiologic signs of interstitial infiltrates (62%). The sensitivity was calculated taking as reference a constructed gold-standard, represented by the sum of all three techniques combined. Sensitivities for bronchoalveolar lavage, transbronchial biopsy, and bronchial brush were 86%, 43% and 5%, respectively. The association of bronchoalveolar lavage and transbronchial biopsy reached the sensitivity to 100%, reflecting how the gold-standard was constructed. The complication rate of the procedures was 3%. These results suggest that, in this population: (1) bronchoscopy with bronchoalveolar lavage, bronchial brush, and transbronchial biopsy are secure procedures with good diagnostic performance; (2) bronchoalveolar lavage alone has reached the highest sensitivity, whereas both bronchoalveolar lavage and transbronchial biopsy can be considered complementary techniques; (3) the association of bronchoalveolar lavage and transbronchial biopsy performed better than bronchoalveolar lavage alone; and (4) the use of bronchial brush in the sequence used in this protocol did not have any influence on the performance of diagnosis.

Keywords: Human immunodeficiency virus. Lung diseases. Diagnostic techniques. HIV. Lung infections.

Objective: To verify if there are differences in Chronic Obstructive Pulmonary Disease (COPD) patient exacerbator and non-exacerbator phenotypes undergoing a Pulmonary Rehabilitation Program (PRP). Methods: A real life retrospective study included outpatients with COPD from public primary care who completed a 12-weeks PRP, three times a week. All were assessed before and after PRP using the six minute walk test (6MWT), the modified Medical Research Council (mMRC) dyspnea index, quality of life and Body mass Index, airflow Obstruction, Dyspnea and Exercise (BODE index). Results: A total of 151 patients were analyzed and mean age was 65.0 ± 8.1 years and mean Forced Expiratory Volume (FEV) 1% of predicted was 39.8 ± 15.9. The predominant gender was male (66.9%). Of these patients 31 (20.5%) were exacerbator phenotype There was a significant improvement in the mean distance in the 6MWT in both groups, with the largest change observed in the exacerbator group [mΔ (95% CI): 84.9 (57.1 112.6) vs. 48.6 (37-60.2) p= 0.018]. Significant reduction in dyspnea on the mMRC scale occurred in both groups, with the highest intensity in the exacerbator group [mΔ (95% CI): - 0.8 (-1.11 to 0.51) vs. -1.6 (-2.20 to -1.13) p = 0.006]. Improvement in the BODE index occurred in both groups, but the mean variation was also significantly greater in the exacerbator group [mΔ (95% CI): -1.44 (-2.17 to -0.70) p= 0.045]. Conclusion: Patients with COPD exacerbator phenotype had a greater magnitude of response to PRP (36 meters) when compared to non-exacerbator phenotype regardless the severity of airflow obstruction, also showing improvement in prognosis measured by the BODE index.

Keywords: Chronic obstructive pulmonary disease; Phenotype; Rehabilitation.

Objective: To compare religious coping (RC) in patients with COPD and healthy individuals, as well as to determine whether RC is associated with demographic characteristics, quality of life, depression, and disease severity in the patients with COPD. Methods: This was a cross-sectional study conducted between 2014 and 2016, involving outpatients with moderate to severe COPD seen at one of two hospitals in Fortaleza, Brazil, as well as gender- and age-matched healthy controls. The Brief RCOPE scale assessed RC in all of the participants. We also evaluated the COPD group patients regarding symptoms, quality of life, and depression, as well as submitting them to spirometry and a six-minute walk test. Results: A total of 100 patients were evaluated. The mean age was 67.3 ± 6.8 years, and 54% were men. In the COPD group, the mean positive RC score was significantly higher than was the mean negative RC score (27.17 ± 1.60 vs. 8.21 ± 2.12; p = 0.001). The mean positive RC score was significantly higher in women than in men (27.5 ± 1.1 vs. 26.8 ± 2.8; p = 0.02). Negative RC scores were significantly higher in the COPD group than in the control group (p = 0.01). Negative RC showed an inverse association with six-minute walk distance (6MWD; r = −0.3; p < 0.05) and a direct association with depressive symptoms (r = 0.2; p < 0.03). Positive RC correlated with none of the variables studied. Multiple regression analysis showed that negative RC was associated with 6MWD (coefficient = −0.009; 95% CI: −0.01 to −0.003). 6MWD explained the variance in negative RC in a linear fashion. Conclusions: Patients with COPD employ negative RC more often than do healthy individuals. Exercise capacity and depressive symptoms are associated with negative RC.

Keywords: Religion; Spirituality; Adaptation, psychological; Quality of life; Pulmonary disease, chronic obstructive.

Objective: The rapid differentiation between Mycobacterium tuberculosis and nontuberculous mycobacteria is fundamental for patients co-infected with tuberculosis and HIV. To that end, we use two methods in our laboratory: detection of cord factor and PCR-restriction enzyme analysis (PRA). The objective of this study was to evaluate the accuracy of a screening test on solid medium as a rapid method for the presumptive identification of M. tuberculosis complex, considering costs and turnover time. Methods: A total of 152 strains were submitted to a combined screening test, consisting of the detection of cord factor under microscopy (Ziehl-Neelsen staining) and evaluation of the macroscopic aspect of colonies, as well as to PRA, which was used as the gold standard. Costs were estimated by calculating the price of all of the materials needed for each test. Results: The overall accuracy of cord factor detection alone was 95.4% (95% CI: 90.7-98.1%), and that of the combined screening test was 99.3% (95% CI: 96.4-100%). Cord factor detection costs US$ 0.25, whereas the PRA costs US$ 7.00. Results from cord factor detection are ready in 2 days, whereas PRA requires 4 days to yield results. Conclusions: The presumptive identification of M. tuberculosis using the macroscopic evaluation of colonies combined with cord factor detection under microscopy is a simple, rapid and inexpensive test. We recommend the combined screening test to rapidly identify M. tuberculosis in resource-poor settings and in less well-equipped laboratories while awaiting a definite identification by molecular or biochemical methods.

Keywords: Tuberculosis; Mycobacterium/classification; Polymerase chain reaction; Diagnostic tests, routine.

Objective: To evaluate the accuracy of bacteriological methods and of polymerase chain reaction (with primers specific for IS6110 of the Mycobacterium tuberculosis complex) in testing sputum samples from indigenous (Amerindian) and non-indigenous patients. Methods: A total of 214 sputum samples (154 from indigenous patients and 60 from non-indigenous patients) were analyzed in order to determine the accuracy of smear microscopy (direct and concentrated versions) for acid-fast bacilli, culture, and polymerase chain reaction. Results: Both microscopy methods presented low sensitivity in comparison with culture and polymerase chain reaction. Specificity ranged from 91% to 100%, the concentrated acid-fast smear technique being the least specific. Nontuberculous mycobacteria were isolated three times more frequently in samples from indigenous patients than in those from non-indigenous patients. False-positive and false-negative polymerase chain reaction results were more common in the indigenous population. Conclusion: Positivity and isolation of nontuberculous mycobacteria in the acid-fast smear in conjunction with polymerase chain reaction positivity raise the following hypotheses: nontuberculous mycobacteria species with DNA regions homologous to, or even still possessing, the M. tuberculosis IS6110 exist in the Amazon; colonization of the oropharynx or of a tuberculous lesion accelerates the growth of the nontuberculous mycobacteria present in the sputum samples, making it impossible to isolate M. tuberculosis; A history of tuberculosis results in positivity for M. tuberculosis DNA. The absence of bacteriological positivity in the presence of polymerase chain reaction positivity raises questions regarding the inherent technical characteristics of the bacteriological methods or regarding patient history of tuberculosis.

Keywords: Tuberculosis, pulmonary/diagnosis; Mycobacterium tuberculosis, Polymerase chain reaction/methods; Indians, south american

Objective: To study the clinical, radiological, and histopathological patterns of transbronchial biopsy (TBB) used in order to confirm the diagnosis in patients with clinical suspicion of interstitial lung disease (ILD) treated at a tertiary-care university hospital. Methods: We reviewed the medical records, radiology reports, and reports of transbronchial biopsies from all patients with suspected ILD who underwent TBB between January of 1999 and December of 2006 at the Hospital das Clínicas de Botucatu, located in the city of Botucatu, Brazil. Results: The study included 56 patients. Of those, 11 (19.6%) had a definitive diagnosis of idiopathic pulmonary fibrosis (IPF), the rate of which was significantly higher in the patients in which ILD was a possible diagnosis in comparison with those in which ILD was the prime suspect (p = 0.011), demonstrating the contribution of TBB to the diagnostic confirmation of these diseases. The histopathological examination of the biopsies revealed that 27.3% of the patients with IPF showed a pattern of organizing pneumonia, which suggests greater disease severity. The most common histological pattern was the indeterminate pattern, reflecting the peripheral characteristic of IPF. However, the fibrosis pattern showed high specificity and high negative predictive value. For CT scan patterns suggestive of IPF, the ROC curve showed that the best relationship between sensitivity and specificity occurred when five radiological alterations were present. Honeycombing was found to be strongly suggestive of IPF (p = 0.01). Conclusions: For ILDs, chest CT should always be performed, and TBB should be used in specific situations, according to the suspicion and distribution of lesions.

Keywords: Lung diseases, interstitial; Diagnosis, differential; Bronchoscopy.

Objective: To present epidemiological data on patients diagnosed with tuberculosis, as well as on associated factors, and to determine the efficacy of the National Tuberculosis Control Program in Bagé, Brazil. Methods: A retrospective study was carried out at the Pablo Barcellos Center, analyzing cases of tuberculosis reported from January 2001 to December 2004. Data were collected through the review of clinical charts and from the National Case Registry database. Results: During this period, of the 4468 sputum smear microscopies performed, 131 were positive, with higher prevalence among males aged 26 to 35 years old. Prevalence was lower among those aged 65 and above. Over 50% of the patients were Caucasian, had only 1 to 3 years of schooling and worked in low-income jobs (mean salary, 265 Brazilian reals/month). There was no significant difference between smokers and former smokers/nonsmokers, and only one of the 131 cases was HIV positive. Conclusion: The number of sputum smear microscopies performed in Bagé increased in the past four years. In 2003 and 2004, it exceeded the goal of the National Tuberculosis Control Program. However, the number of new cases decreased, demonstrating the efficacy of the active search for tuberculosis cases in the city.

Keywords: Tuberculosis; Epidemiology; Communicable disease control.

Objective: To identify characteristics related to smoking in hospitalized patients and to assess the knowledge that such patients have regarding the relationship between nicotine dependence and smoking-related diseases. Methods: The study included 186 patients (males, 59%; mean age, 51.3 ± 16.8 years) who were evaluated regarding demographic characteristics, diagnosis at admission, smoking history and passive smoke exposure. All of the patients completed a questionnaire regarding their knowledge of the relationship between smoking and disease. Results: Of the 186 patients, 42 (22.6%) were smokers, 64 (34.4%) were former smokers and 80 (43%) stated they were never smokers; 136 (73%) reported passive smoke exposure. In the sample as a whole, 21.5% of the patients were diagnosed with a smoking-related disease at admission, compared with 39% of those who were smokers or former smokers. The proportion of individuals who were unaware of the relationship between smoking and the cause of hospitalization was similar among current smokers and former smokers (56% and 65%, respectively). Only 19% of the current smokers believed that smoking might have affected their health, compared with 32% of the former smokers (p = 0.22). The proportion of individuals who believed that quitting smoking depends on willpower was significantly higher among former smokers and never smokers than among current smokers (64% and 53%, respectively, vs. 24%; p < 0.001 and p = 0.008). Although 96% of the patients believed that smoking causes dependence, only 60% identified smoking as a disease. Conclusions: This study shows the disconnect between the recognition of smoking as a cause of dependence and the recognition of smoking as a disease, as well as the general lack of awareness that former and current smoking constitute a risk factor for the development and progression of disease.

Keywords: Smoking; Tobacco use disorder; Smoking cessation.

The authors analyze the nutritional status of patients with lung cancer. 131 patients, 96 (73.3%) male and 35 (26.7%) female, were classified into three groups: I - malnourished patients; II - eutrophic patients and III - obese patients. Most of the malnourished had anorexia (59.7%), which was not seen in the eutrophic (25%) and obese patients (26.1%). More than 70% of patients reported some degree of previous weight loss at the initial evaluation, weight loss being higher among malnourished patients. Weight loss was observed in more than 60% of the patients during the follow-up period of 3 months after the first evaluation. Patients with squamous carcinoma neoplasms prevailed in the malnourished group whereas adenocarcinoma was more prevalent in the other two groups. Most patients had advanced stages of the disease.

Keywords: Lung cancer. Nutritional status.

Objective: To derive reference values from white race adults, for DCO in a sample from different sites in Brazil, through the same equipment model (Sensormedics), and compare the results with the derivatives from Crapo, Miller, Neder equations and from the Global Lung Initiative (GLI) proposal. Methods: The tests were performed according to the norms suggested by ATS/ERS in 2005 in six Brazilian cities, with 120 adult volunteers of each gender, non-smokers, without referred anemia and without lung or cardio diseases. The expected values were derived from linear regressions and the differences between the values forecasted by some authors and the ones observed in the current study were calculated. Results: Among men, the age varied between 25 and 88 years old, and the height varied between 140 and 176 cm. DCO was correlated significantly and positively with the height and negatively with the age. The values forecasted by Crapo, Neder, and Miller equations were higher in comparison with the ones obtained by the current study (p<0.01) in both genders. Among men, the values did not differ when compared to the ones calculated by GLI (p=0.29); among women, the values derived by GLI were slightly higher: 0.99 ml/min/mmHg (p<0.01). Conclusion: new values forecasted for DCO were derived in a sample of white adults in Brazil. The forecasted values are similar to the ones complied by GLI equations and differ from the previously proposed equations.

Keywords: Transfer factor; Pulmonary diffusing capacity; Diffusion; Carbon monoxide; Reference values; Lung function tests.

Objective: To test a prediction model for sleep apnea based on clinical and sociodemographic variables in a population suspected of having sleep disorders and submitted to polysomnography. Methods: We included 323 consecutive patients submitted to polysomnography because of the clinical suspicion of having sleep disorders. We used a questionnaire with sociodemographic questions and the Epworth sleepiness scale. Blood pressure, weight, height, and SpO2 were measured. Multiple linear regression was used in order to create a prediction model for sleep apnea, the apnea-hypopnea index (AHI) being the dependent variable. Multinomial logistic regression was used in order to identify factors independently associated with the severity of apnea (mild, moderate, or severe) in comparison with the absence of apnea. Results: The prevalence of sleep apnea in the study population was 71.2%. Sleep apnea was more prevalent in men than in women (81.2% vs. 56.8%; p < 0.001). The multiple linear regression model, using log AHI as the dependent variable, was composed of the following independent variables: neck circumference, witnessed apnea, age, BMI, and allergic rhinitis. The best-fit linear regression model explained 39% of the AHI variation. In the multinomial logistic regression, mild apnea was associated with BMI and neck circumference, whereas severe apnea was associated with age, BMI, neck circumference, and witnessed apnea. Conclusions: Although the use of clinical prediction models for sleep apnea does not replace polysomnography as a tool for its diagnosis, they can optimize the process of deciding when polysomnography is indicated and increase the chance of obtaining positive polysomnography findings.

Keywords: Sleep apnea syndromes; Polysomnography; Sleep apnea, obstructive; Body mass index.

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.