Objective: To determine the prevalence of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis treated at a referral center in the state of Bahia, Brazil. Methods: A cross-sectional study, with prospective data collection, carried out at the Cystic Fibrosis Referral Center of Bahia of the Octávio Mangabeira Specialized Hospital. We evaluated 74 patients diagnosed with cystic fibrosis, older than six years of age, treated between December 9, 2003 and March 7, 2005. We analyzed the following variables: gender, age, forced vital capacity, forced expiratory volume in one second, pharmacodynamic response, chest X-ray findings, facial sinus X-ray findings, wheezing, cultures for Aspergillus spp., total immunoglobulin E (IgE), specific IgE for Aspergillus fumigatus and immediate skin test reactivity to A. fumigatus antigen. Results: Of the 74 patients, 2 were diagnosed with ABPA. We found total IgE levels > 1,000 IU/mL in 17 (23%), positive immediate skin reactivity to A. fumigatus antigen in 19 (25.7%) and wheezing in 60 (81.1%). Conclusions: The prevalence of ABPA was 2.7%. The high levels of total IgE, high incidence of wheezing and high rate of immediate skin test reactivity to A. fumigatus antigen suggest that these patients should be carefully monitored due to their propensity to develop ABPA.

Keywords: Cystic fibrosis; Aspergillosis, allergic bronchopulmonary; Immunoglobulin E/diagnostic use; Hypersensitivity, immediate/diagnosis; Gliotoxin.

Objective: To report demographic and clinical characteristics of patients with asthma who evolved to death, as well as to describe the conditions related to this outcome in a subgroup of patients admitted to the Program for the Control of Asthma and Rhinitis in Bahia (ProAR). Methods: A descriptive, retrospective, observational study. Data from clinical charts and death certificates of 16 patients of 930 subjects with severe asthma monitored at the ProAR Central Reference Center from December 2003 to June 2006 were reviewed. Results: Of the 930 patients participating in the program, 16 (1.72%) died. Of these, there were 10 males and 6 females, ranging in age from 39 to 74 years (median, 55 years); 12 (75%) of the patients were black. Time since diagnosis ranged from 1 to 68 years (median, 30 years). In 43.8 and 53.8%, respectively, there was a personal or family history of atopy. Ex-smokers (<10 pack-years) accounted for 37.5% of the cases. Causes of death listed on the death certificates were as follows: asthma or asthma exacerbations in 8 (50%); respiratory failure in 3 (18.75%); acute heart infarction in 2 (12.5%); hepatitis in 1 (6.25%); hypovolemic shock in 1 (6.25%); and cardiorespiratory arrest in 1 (6.25%). Of the 16 deaths, 13 (81.25%) occurred inside hospitals. Conclusion: Asphyxia and cardiovascular diseases were the most common atributed causes of mortality in this subgroup of patients with severe asthma. Hospital-based mortality, male gender, advanced age, long-term disease and fixed airflow obstruction were the aspects most frequently observed in the cases studied.

Keywords: Mortality; Rhinitis/treatment; Asthma/treatment; Cardiovascular diseases.

Allergic bronchopulmonary aspergillosis is a lung disease occurring in patients with asthma or cystic fibrosis, triggered by a hypersensitivity reaction to the presence of Aspergillus fumigatus in the airways. We report herein the case of a patient presenting a clinical profile suggestive of asthma and meeting the clinical, laboratory testing and radiological criteria for a diagnosis of allergic bronchopulmonary aspergillosis. The importance of such findings is that early diagnosis can reduce the risk of respiratory exacerbations and fibrosis.

Keywords: Asthma; Bronchiectasis; Aspergillus fumigatus; Aspergillosis, allergic bronchopulmonary

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: To determine the prevalence of latent and active TB among detainees in a prison hospital in Bahia, Brazil. Methods: A cross-sectional study with prospective data collection was carried out comprising 237 inmates in the Bahia State Prison Hospital between July 2003 and April 2004. A standardized questionnaire was applied and completed by medical students. The detainees were systematically submitted to the following tests: tuberculin skin test, chest X-ray (anteroposterior), sputum smear microscopy and culture for mycobacteria. The events of interest were active TB and latent TB. Results: The mean age of the participants was 36.6 years, and 89.9% were male. Smoking and alcohol consumption were reported by 70.0% and 43.9% of the inmates, respectively. A history of treatment for TB was reported by 11.3% of the inmates. Of the inmates evaluated, 36.3% reported cough and 31.4% reported expectoration. Other less common symptoms were asthenia (in 26.2%), weight loss (in 23.1%), loss of appetite (in 17.7%), fever (in 11.3%) and hemoptysis (in 6.7%). Of the 86 inmates tested, none presented positive HIV serology. The prevalence of latent TB was 61.5% (96 of the 156 inmates submitted to tuberculin skin tests), whereas that of active TB was 2.5% (6 of the 237 inmates evaluated). The presence of cough was a determinant of active TB (prevalence ratio = 8.8; 95% CI: 1.04-73.9; p = 0.025). Conclusions: Active and latent TB are highly prevalent among inmates hospitalized in the Bahia State Prison Hospital. Our findings justify the need to implement public policies specifically directed towards the control of TB in this population.

Keywords: Tuberculosis; Prisons; Hospitals; Prevalence.

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.

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 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: The sweat test (ST) measures chloride levels in sweat and is considered the gold standard for the diagnosis of cystic fibrosis (CF). However, the reliability of a ST depends on their being performed by experienced technicians and in accordance with strict guidelines. Our aim was to evaluate how sweat stimulation, sweat collection, and chloride measurement are performed at 14 centers (9 public centers and 5 private centers) that routinely perform STs in the state of São Paulo, which has the highest frequency of CF in Brazil. Methods: This was a cross-sectional cohort study, using a standardized questionnaire administered in loco to the staff responsible for conducting STs. Results: No uniformity regarding the procedures was found among the centers. Most centers were noncompliant with the international guidelines, especially regarding the collection of sweat (the samples were insufficient in 10-50% of the subjects tested); availability of stimulation equipment (which was limited at 2 centers); modernity and certification of stimulation equipment (most of the equipment having been used for 3-23 years); and written protocols (which were lacking at 12 centers). Knowledge of ST guidelines was evaluated at only 1 center. Conclusions: Our results show that STs largely deviate from internationally accepted guidelines at the participating centers. Therefore, there is an urgent need for standardization of STs, training of qualified personnel, and acquisition/certification of suitable equipment. These are essential conditions for a reliable diagnosis of CF, especially with the increasing demand due to newborn screening nationwide, and for the assessment of a possible clinical benefit from the use of modulator drugs.

Keywords: Cystic fibrosis/diagnosis; Cystic fibrosis/prevention & control; Sweat.

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.

This study carries out a descriptive and comparative analysis of the various types of cystic fibrosis severity scores described in the literature and contextualizes the origin and objective of each. A total of 16 scoring systems were found: 8 are used predominantly for clinical evaluation, 5 for radiographic findings, 2 for tomographic findings and 1 for scintigraphic findings. Despite the criticism and controversy regarding these instruments of assessment, they have contributed to a better understanding of the disease and to the development of more effective therapeutic procedures.

Keywords: Severity of illness index. Cystic fibrosis. Review literature.

Objective: To estimate the prevalence of human immunodeficiency virus seropositivity among patients with active tuberculosis residing in the city of Londrina, in the state of Paraná, Brazil and treated at the Pulmonology Clinic of the 17th Regional Health Facility of the State of Paraná, and to compare those coinfected with human immunodeficiency virus to those with active tuberculosis only in terms of the clinical form of tuberculosis, sputum smear microscopy, chest X-ray and tuberculosis treatment regimen. Methods: A transversal study involving 188 active tuberculosis patients was conducted. Tuberculosis and human immunodeficiency virus infection were diagnosed based on criteria established by the Brazilian Ministry of Health. In addition to the data obtained through individual tuberculosis investigation files, clinical and epidemiological information were collected by means of questionnaires, which were completed by all participating patients. Results: The prevalence of human immunodeficiency virus seropositivity among patients with tuberculosis was 14.9%. The rate of positive sputum microscopy results was higher in the tuberculosis-only group (p = 0.0275), and the ratio for patients treatment with alternative scheme was significantly higher in the co-infected group (p = 0.042). In 32.1% of the coinfected patients, the serological diagnosis of human immunodeficiency virus infection was made simultaneously to or following that of the tuberculosis. Conclusion: The results underscore the importance of routinely testing for human immunodeficiency virus antibodies in patients diagnosed with tuberculosis.

Keywords: Tuberculosis/complications; Tuberculosis/diagnosis; Soropositivity; HIV/immunology; HIV infections/complications; HIV infections/epidemiology; HIV infections/ drug therapy; Rifampin/therapeutic use; Disease progression

Objective: To describe the profile of patients with cystic fibrosis (CF). Methods: A prospective, cross-sectional study involving CF patients ≤ 18 years of age, evaluated between March and July of 2006 at a referral center in northeastern Brazil. Nutritional assessment was performed using Z scores for height/age (H/A), weight/age (W/A) and weight/height (W/H), as well as %weight/height (%W/H) and body composition measurements. Socioeconomic and clinical data were obtained. Results: Twenty-one patients were evaluated, 12 (57.1%) of whom were female. Mean age at diagnosis was 3.8 ± 3.9 years. The principal features at diagnosis were respiratory infection (85.7%), steatorrhea (66.7%) and nutritional deficit (47.6%). The mean Z scores for W/A, H/A and W/H were 0.73 ± 0.28, 0.34 ± 0.21 and 0.73 ± 0.35, respectively. Mean %W/H was 94.52 ± 1.58. The percentage of malnourished children assessed by Z score differed from that assessed by %W/H (nutritional deficit in 66.7% and 33.3%, respectively; p > 0.05). Socioeconomic status, clinical status and Shwachman score were better among well-nourished patients than among those classified as malnourished (p < 0.05 for Shwachman score). Conclusions: Normal nutritional status was identified based on nutritional indicators (W/A, H/A and W/H), whereas nutritional deficit was identified by assessing body composition. Socioeconomic factors proved favorable, especially maternal education and per capita income. Age at diagnosis was higher than that reported in the literature, although the Shwachman score and the incidence of respiratory infections demonstrated that the patients presented good clinical status.

Keywords: Cystic fibrosis; Nutritional assessment; Symptoms, clinical; Socioeconomic factors.

Objective: To determine the prevalence of smoking in the adult population of Brazil, in order to propose recommendations for the reduction of tobacco use. Methods: This was a population-based, cross-sectional study including a sample composed of residents (≥ 18 years of age) of the capital cities of 26 Brazilian states and in the Federal District of Brasília, Brazil. For the determination of sample size, a 95% confidence interval and a 2% sample error were defined. The participants were selected and interviewed by means of the Sistema de Vigilância de Fatores de Risco e Proteção para Doenças Crônicas por Inquérito Telefônico (VIGITEL, Telephone-based System for the Surveillance of Risk and Protective Factors for Chronic Diseases).The proportion of smokers and the number of cigarettes smoked per day were estimated and stratified according to sociodemographic variables. In addition, the male/female ratio was estimated for the prevalence of smoking. Results: The prevalence of smoking was 16.1% (20.5% among males and 12.4% among females). The proportion of adults that reported smoking ≥ 20 cigarettes a day was 4.9%, being greater in males (6.5% vs. 3.6%). The prevalence of smoking was greater among individuals with a lower level of education (≤ 8 years of schooling). The number of cigarettes smoked per day by males was approximately the double that smoked by females. Conclusions: The VIGITEL estimates indicate a reduction in the prevalence of smoking, which was, however, still greater among males than among females. The VIGITEL has been fundamental to monitoring smoking, as well as to informing decisions regarding public policies for health promotion and the prevention of chronic nontransmissible diseases.

Keywords: Smoking/epidemiology; Smoking/prevention & control; Cross-sectional studies.

Objective: To evaluate, using computerized tomography, the frequency of paranasal sinus involvement in patients with allergic rhinitis. Methods: From among outpatients diagnosed with rhinitis and complaining of nasal obstruction, 60 were selected for evaluation. The patients were submitted to anterior rhinoscopy, skin prick test for reactivity to aeroallergens and computed tomography of the paranasal sinuses. In addition, questionnaires designed to evaluate symptom severity were administered. The Lund score was used to evaluate paranasal sinus involvement on computed tomography scans. Results: Computed tomography scans of the paranasal sinuses were abnormal in 31 patients (52%). The sum of the largest diameters of cutaneous reactions to the aeroallergens, symptom severity and anterior rhinoscopy findings did not differ between patients with paranasal sinus involvement and those without. All the patients with paranasal sinus abnormalities also presented osteomeatal complex abnormalities, whereas only 11 patients (38%) without paranasal sinus involvement presented such abnormalities (p < 0.01). Conclusion: In a sample of patients with allergic rhinitis, the frequency of paranasal sinus abnormalities on computed tomography scans was elevated and did not correlate with symptom severity or skin prick test reactivity but was correlated with osteomeatal complex obstruction.

Keywords: Rhinitis; Sinusitis; Tomography, X-ray computed; Paranasal sinuses/radiography

Objective: To determine the prevalence of smoking experimentation among adolescents with asthma or allergic rhinitis. Methods: This was a cross-sectional study involving adolescent students (13-14 years of age) in the city of Belo Horizonte, Brazil. The participants completed the Centers for Disease Control and Prevention and International Study of Asthma and Allergies in Childhood questionnaires, both of which have been validated for use in Brazil. We calculated the prevalence of smoking experimentation in the sample as a whole, among the students with asthma symptoms, and among the students with allergic rhinitis symptoms, as well as in subgroups according to gender and age at smoking experimentation. Results: The sample comprised 3,325 adolescent students. No statistically significant differences were found regarding gender or age. In the sample as a whole, the prevalence of smoking experimentation was 9.6%. The mean age for smoking experimentation for the first time was 11.1 years of age (range, 5-14 years). Among the adolescents with asthma symptoms and among those with allergic rhinitis symptoms, the prevalence of self-reported smoking experimentation was 13.5% and 10.6%, respectively. Conclusions: The proportion of adolescents with symptoms of asthma or allergic rhinitis who reported smoking experimentation is a cause for concern, because there is strong evidence that active smoking is a risk factor for the occurrence and increased severity of allergic diseases.

Keywords: Asthma/epidemiology; Rhinitis/epidemiology; Smoking/epidemiology.

Objective: To determine the effect that re-education of the thoracic and abdominal muscles has on the respiratory muscle strength of patients with cystic fibrosis evaluated over time at the Cystic Fibrosis Outpatient Clinic of the Universidade Católica de Brasília (Catholic University of Brasília). Methods: The sample consisted of 29 cystic fibrosis patients, characterized based on anthropometric, genetic and bacterial colonization data. The patients were submitted to physical therapy sessions, involving re-education of the respiratory muscles, twice a week for four months. Spirometry, pressure manometry and anthropometry were performed before and after each session. Results: Comparing baselines values to those obtained after physical therapy, increases in maximum inspiratory pressure and maximum expiratory pressure were observed in all patients, those without any obstructive respiratory disease and those with mild obstructive respiratory disease (p < 0.05). A positive correlation between age and maximum expiratory pressure was observed for most of the patients. Maximum inspiratory pressure correlated positively with age only in the group with mild obstructive respiratory disease (p = 0.012; r = 0.817). In female patients and in the group of patients without obstructive respiratory disease, a negative correlation was observed between maximum expiratory pressure and colonization with Pseudomonas aeruginosa (p = 0.036; r = -0.585). Conclusion: Use of the thoracic and abdominal muscle re-education technique increased respiratory muscle strength in the cystic fibrosis patients studied, a finding that underscores the importance of including physical therapy in the treatment of these patients.

Keywords: Cystic fibrosis/rehabilitation; Physical therapy technique; Respiratory muscles; Forced expiratory volume; Inspiratory capacity; Vital capacity

Epidemiologic data on idiopathic pulmonary fibrosis are relatively scarce, and its real incidence and prevalence are unknown. Recent studies suggest that idiopathic pulmonary fibrosis mortality is rising in developed countries. Objectives: To describe idiopathic pulmonary fibrosis mortality in the State of Rio Grande do Sul (RS), Brazil, from 1970 to 2000, analyzing its trend and comparing it with that from other countries. Methods: Prevalence study, using data from the Brazilian Institute of Geography and Statistics (IBGE), analyzing death certificates in which idiopathic pulmonary fibrosis was stated as ultimate cause of death. Results: Annual mortality adjusted to population was 0.22/100,000 persons in the 1970's, 0.3/100,000 persons in the 1980's, and 0.48/100,000 persons in the 1990's. Total mortality rised 36% from 1970 to 1980, and 70% from 1980 to 1990. Mortality adjusted to population rised 36% and 60% during the same periods. The rise in both total and adjusted mortality from IPF was statistically significant (p < 0.05). The mean mortality rate per 100,000 persons during 1996 to 1998, however, was 0.683, corresponding to a rise of 70% when compared to the previous three-year period, which was 0.4 (p = 0.0002), probably reflecting encoding practices. Conclusion: There was a significant increase in IPF mortality in RS from 1970 to 2000, in part due to changes in coding practices. This increase is in conformity with observations in other countries, althought mortality rates in RS are considerably lower.

Keywords: Pulmonary fibrosis. Mortality.