Objective: To evaluate whether anthropometric and dietary intake indicators are predictors of pulmonary function in cystic fibrosis (CF) patients. Methods: This was a cross-sectional study involving 69 patients (age range, 5.4-16.5 years) diagnosed with CF under follow-up at the Hospital de Clínicas de Porto Alegre, located in the city of Porto Alegre, Brazil. Anthropometric assessment was based on body mass index (BMI), mid-arm muscle circumference (MAMC), and triceps skinfold thickness (TST). Dietary intake was assessed by using recall data, which were compared with the recommended dietary allowances. Pulmonary function was assessed by ventilatory capacity, expressed as FEV1. Prevalence ratios for the outcome studied (FEV1 < 80% of predicted) were calculated by indicator. Results: In patients with MAMC and TST below the 25th percentile, the prevalence of FEV1 < 80% of predicted was significantly higher than in those with higher MAMC and TST (p < 0.001 and p = 0.011, respectively). In comparison with other patients, those with a BMI below the 50th percentile showed a 4.43 times higher prevalence of FEV1 < 80% of predicted (95% CI: 1.58-12.41), and that prevalence was 2.54 times higher in those colonized with methicillin-resistant Staphylococcus aureus (MRSA) than in those not so colonized (95% CI: 1.43-4.53). The association between dietary intake and the prevalence of FEV1 < 80% of predicted was of only borderline significance (95% CI: 0.95-3.45). Conclusions: Not being colonized with MRSA and having a BMI above the 50th percentile appear to preserve pulmonary function in CF patients.

Keywords: Cystic fibrosis; Respiratory function tests; Nutrition assessment; Energy intake.

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.

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.

Objective: To analyze the effects of Pilates mat exercises in patients with cystic fibrosis (CF). Methods: This was a clinical trial involving 19 CF patients recruited from either the CF Outpatient Clinic of the State University at Campinas Hospital de Clínicas or the Children's Institute of the University of São Paulo School of Medicine Hospital das Clínicas. All of the patients performed Pilates mat exercises for four months (one 60-min session per week). The variables studied (before and after the intervention) were respiratory muscle strength, MIP, MEP, FVC, and FEV1. Results: After the intervention, MIP was significantly higher in the male patients (p = 0.017), as were MIP and MEP in the female patients (p = 0.005 and p = 0.007, respectively). There were no significant differences between the pre- and post-intervention values of FVC or FEV1, neither in the sample as a whole nor among the patients of either gender. Conclusions: Our results show that Pilates mat exercises have beneficial effects on respiratory muscle strength in CF patients. (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR-86vp8x [http://www.ensaiosclinicos.gov.br])

Keywords: Cystic fibrosis; Muscle strength; Exercise movement techniques; 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 study correlations among pulmonary function, chest radiology and clinical status in cystic fibrosis. Methods: A retrospective cross-sectional study was performed to evaluate chest X-rays and clinical charts of patients treated at the Hospital de Clínicas de Porto Alegre. Spirometry findings, Shwachman-Kulczycki (S-K) scores and Brasfield scores were analyzed. Results: The final sample consisted of 40 patients (mean age 9.72 ± 3.27). The following mean S-K scores were obtained: total, 80.87 ± 10.24; general activity, 24.75 ± 1.1; physical examination, 18.87 ± 4.59; nutrition, 21.87 ± 4.18; radiology, 15.37 ± 5.23. The mean Brasfield score was 18.2 ± 4. The pulmonary function test results, in percentage of predicted, were as follows: forced vital capacity (FVC), 82.99 ± 14.36%; forced expiratory volume in one second (FEV1), 83.62 ± 18.26%; and forced expiratory flow between 25 and 75% of FVC (FEF25-75), 74.63 ± 2.53%. The S-K score correlated moderately with FVC, whereas it correlated strongly with FEV1 and FEF25-75. The Brasfield score correlated strongly with the S-K total and radiology score, whereas it correlated moderately with pulmonary function. Physical examination correlated moderately with FVC, FEV1 and FEF25-75; as did nutrition with FEF25-75; and radiology with FEV1 and FEF25-75. General activity was the domain that had the greatest influence on the total S-K score. Conclusions: These two scoring systems are complementary, correlating with each other, as well as with pulmonary function tests. The radiology domain of the S-K scoring system is a good alternative to the Brasfield score.

Keywords: Cystic fibrosis; Spirometry; Lung/radiography.

Objective: To evaluate the association between nutritional status measurements and pulmonary function in children and adolescents with cystic fibrosis. Methods: We evaluated the nutritional status of 48 children and adolescents (aged 6-18 years) with cystic fibrosis based on body mass index (BMI) and body composition measurements-mid-arm muscle circumference (MAMC) and triceps skinfold thickness (TST)-at a referral center in the city of Rio de Janeiro, Brazil. Pulmonary function was assessed by means of spirometry, using FEV1 to classify the severity of airway obstruction. We used Student's t-tests for comparisons between proportions and linear regression analysis for associations between continuous variables. The level of significance was set at p < 0.05. Results: The evaluation of nutritional status based on BMI identified a smaller number of malnourished patients than did that based on MAMC (14 vs. 25 patients, respectively). Most of the patients presented mild pulmonary disease. Mean FEV1 was 82.5% of predicted. Pulmonary function was found to correlate significantly with BMI, MAMC and TST (p = 0.001, p = 0.001 and p = 0.03, respectively). All subjects with moderate or severe pulmonary involvement were considered malnourished based on BMI and body composition parameters. Of the 25 patients considered malnourished based on body composition (MAMC), 19 were considered well-nourished based on their BMI. Conclusions: In the present study, all nutritional status measurements correlated directly with the pulmonary function of children and adolescents with cystic fibrosis. However, body composition measurements allowed earlier detection of nutritional deficiencies.

Keywords: Cystic fibrosis; Body composition; Spirometry; Nutrition assessment.

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 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: Thymidine-dependent small-colony variants (TD-SCVs) of Staphylococcus aureus are being isolated with increasing frequency from patients with cystic fibrosis (CF). The aim of this study was to evaluate the relationship between TD-SCV isolation and pulmonary function in patients with CF, as well as to determine whether the emergence of TD-SCVs was associated with trimethoprim-sulfamethoxazole (TMP-SMX) use and with coinfection with other microorganisms. Methods: This was a retrospective case-control study including patients with CF who visited the Clinical Hospital Complex of the Federal University of Paraná, in Curitiba, Brazil, between 2013 and 2022. Demographic, clinical, and spirometric data, as well as information on TD-SCVs and other isolated microorganisms, were collected from the medical records of patients with CF and TD-SCVs (TD-SCV group; n = 32) and compared with those of a matched group of patients with CF without TD-SCVs (control group; n = 64). Results: Isolation of TD-SCVs was positively associated with TMP-SMX use (p = 0.009), hospitalization (p < 0.001), and impaired pulmonary function (p = 0.04). Conclusions: The use of TMP-SMX seems to contribute to the emergence of TD-SCVs, the isolation of which was directly associated with worse pulmonary function in our sample.

Keywords: Staphylococcus aureus/drug effects; Thymidine/metabolism; Trimethoprim, sulfamethoxazole drug combination/adverse effects; Lung/physiopathology; Cystic fibrosis/complications.

Objective: To correlate tomographic findings with pulmonary function parameters in patients with idiopathic pulmonary fibrosis (IPF). Methods: A cross-sectional study was carried out, in which 30 nonsmoking patients with IPF were evaluated. Using a semiquantitative scoring system, the following high-resolution computerized tomography (HRCT) findings were quantified: total interstitial disease (TID), reticular abnormality/honeycombing, and ground-glass opacity (GGO). The functional variables were measured by spirometry, forced oscillation technique (FOT), helium dilution method, as well as the single-breath method of measuring diffusion capacity of the lung for carbon monoxide (DLCO). Results: Of the 30 patients studied, 18 were female, and 12 were male, with a mean age of 70.9 years. We found that TID and reticular abnormality and honeycombing correlated significantly (negative correlations) with the measurements of forced vital capacity (FVC), total lung capacity (TLC), DLCO, and dynamic respiratory compliance were found, as well as that GGO correlated significantly (and positively) with residual volume/TLC. The ratio of forced expiratory flow between 25 and 75% of FVC to FVC (FEF25-75%/FVC) correlated positively with TID, reticular abnormality/honeycombing, and GGO. Conclusion: In IPF patients, the measurements of volume, diffusion, and dynamic compliance are the physiological variables which best reflect the extent of the interstitial disease on HRCT scans.

Keywords: Lung diseases, interstitial; Pulmonary fibrosis; Tomography, X-ray computed; Respiratory function tests.

Objective: Pulmonary disease in cystic fibrosis (CF) is characterised by recurrent episodes of pulmonary exacerbations (PExs), with acute and long-term declines in lung function (FEV1). The study sought to determine whether routine spirometry increases the frequency of PEx diagnosis, resulting in benefits to long-term pulmonary function. Methods: CF patients in the 5- to 18-year age bracket were followed for 1 year, during which they underwent spirometry before every medical visit. The main variables were the frequency of PEx diagnosis and use of antibiotics; the use of spirometry as a criterion for PEx diagnosis (a decline = 10% in baseline FEV1); and median percent predicted FEV1 over time. The data were compared with those for the previous 24-month period, when spirometry was performed electively every 6 months. Results: The study included 80 CF patients. PExs were diagnosed in 27.5% of the visits, with a mean frequency of 1.44 PExs per patient/year in 2014 vs. 0.88 PExs per patient/year in 2012 (p = 0.0001) and 1.15 PExs per patient/year in 2013 (p = 0.05). FEV1 was used as a diagnostic feature in 83.5% of PExs. In 21.9% of PExs, the decision to initiate antibiotics was solely based on an acute decline in FEV1. The median percent predicted FEV1 during the follow-up year was 85.7%, being 78.5% in 2013 and 76.8% in 2012 (p > 0.05). The median percent predicted FEV1 remained above 80% during the two years after the study. Conclusions: Routine spirometry is associated with higher rates of diagnosis and treatment of PExs, possibly impacting long-term pulmonary function.

Keywords: Cystic fibrosis; Respiratory function tests; Respiratory tract infections; Spirometry.

Objective: To evaluate whether respiratory therapy followed by the use of inhaled albuterol modifies the pulmonary deposition of inhaled tobramycin in patients with cystic fibrosis (CF) and whether pulmonary deposition correlates with disease severity or genotype. Methods: A prospective study was carried out including patients with CF older than 6 years of age and colonized with Pseudomonas aeruginosa. Exclusion criteria were pulmonary exacerbation, changes in therapy between the study phases and FEV1 < 25%. All patients were submitted to pulmonary scintigraphy by means of a scintillation camera equipped with a low-energy all-purpose collimator in order to evaluate drug penetration following the administration of inhaled 99mTc-tobramycin, as well as to pulmonary perfusion with 99mTc-macroaggregated albumin (phase 1). One month later, the same procedure was performed following respiratory therapy and administration of inhaled albuterol (phase 2). Results: We included 24 patients (12 males) aged 5-27 years (mean ± SD: 12.85 ± 6.64 years). The Shwachman score (SS) was excellent/good in 8 patients, moderate/fair in 16 and poor in 0. Genotyping revealed that 7 patients were ΔF508 homozygotes, 13 were ΔF508 heterozygotes; and 4 presented other mutations. In all patients, lung deposition of tobramycin decreased in phase 2, especially in those with moderate/fair SS (p = 0.017) and in heterozygotes (p = 0.043). Conclusions: The use of a respiratory therapy technique and the administration of inhaled albuterol immediately prior to the use of inhaled tobramycin decreased the pulmonary deposition of the latter in CF patients, and this reduction correlates with disease severity and genotype.

Keywords: Cystic fibrosis; Tobramycin; Respiratory therapy; Albuterol; Radionuclide imaging.

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 whether nebulizers are a source of microbial contamination in patients with cystic fibrosis, as well as whether the technique and frequency of disinfection of these devices is appropriate. Methods: This was a cross-sectional, uncontrolled observational study. Samples were collected from 28 patients with cystic fibrosis. Samples were collected at the homes of the patients, who were not previously informed of the purpose of the visit. Three samples were collected from each patient: one from the nebulizer chamber, one from the mask/mouthpiece, and one from the patient (oropharyngeal swab/sputum). The samples were properly stored and taken for analyses. The patients, their parents, or their legal guardians completed a questionnaire regarding nebulizer cleaning and disinfecting methods. Results: We collected 84 samples from the 28 patients. Of those 28 patients, 15 (53.5%) were male. The median age of the patients was 11 years (range, 1-27 years). Of the 28 patients, 15 presented with positive oropharyngeal swab/sputum sample cultures. The most common bacterial isolates were Staphylococcus aureus (in 8 patients) and Pseudomonas aeruginosa (in 4 patients). Although the samples obtained from the nebulizers presented with various pathogens in culture, no specific species predominated. In 27 cases (96.7%), there were no associations between the samples obtained from the nebulizers and those obtained from the patients in terms of the results of the cultures. Cleaning and disinfection of nebulizers were inappropriate in 22 cases (78.6%). Conclusions: In this sample of patients, despite the inappropriate disinfection techniques, nebulizers were not found to be a source of microbial contamination.

Keywords: Cystic fibrosis; Nebulizers and vaporizers; Disinfection.

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.

Background: The risk for nosocomial transmission of tuberculosis exists in health care institutions. Objective: To evaluate indicators of transmission risk among patients with pulmonary tuberculosis treated at a university hospital. Method: A retrospective study covering the January 1997 to September of 1999 period and evaluating patients admitted to the Hospital de Clínicas of the Universidade Estadual de Campinas with pulmonary tuberculosis. Three intervals were determined: from admission to collection of sputum for acid-fast bacilli microscopy; from admission to implementation of airborne infection control measures; from sputum collection to the initiation of treatment. Results: The final sample included 63 cases. Concomitant human immunodeficiency virus-positivity was found in 31.7%. Forty patients (63.5%) were admitted through the emergency room. In 42 (66.7%) patients, TB was suspected at admission. The interval between admission and sputum collection exceeded 12 hours in 27.5% of cases admitted through the emergency room and in 30.4% of those admitted directly to wards (p = 0.803). Delayed respiratory isolation occurred in 31 cases (49.2%). The delay in isolation was correlated to no diagnosis of tuberculosis at admission (p < 0.000) and lower bacillary load in the sputum (p = 0.032). Co-infection with human immunodeficiency virus (p = 0.530), hospitalization ward (p = 0.284) and underlying diseases (p = 0.541) were not correlated with delayed isolation. The interval between sputum collection and initiation of treatment was greater than 24 hours in 15.9% of the cases. Conclusion: Delayed isolation was observed in many cases. Policies of continuing education are called for, especially in high-risk areas.

Keywords: Tuberculosis. Infection Control. Brazil. Delay. Diagnosis.

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 determine the effects that mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene and deletion of the glutathione S-transferase (GST) genes mu-1 (GSTM1) and theta-1 (GSTT1) have on the clinical course of cystic fibrosis (CF) in patients residing in the southeastern region of Brazil. Methods: The study sample consisted of all consecutive CF patients treated at the Hospital de Clínicas School of Medical Sciences of the State University at Campinas between March of 2002 and March of 2005. We included 66 CF patients. Genomic DNA was analyzed by polymerase chain reaction and restriction endonuclease digestion for the identification of the genotypes. Results: The F508 mutation of the CFTR gene was found in 44 patients (66.7%). The null genotypes GSTM1, GSTT1 and GSTM1/GSTT1 were found in 40.9%, 15.2%, and 3.0% of the patients, respectively. The F508 CFTR mutation was more common in patients diagnosed with CF before 2.5 years of age than in those diagnosed later (75.5% vs. 41.2%; p = 0.008). The frequency of the F508 CFTR mutation, as well as of the GSTM1 and GSTT1 genotypes, was not found to be associated with gender, ethnicity, pulmonary disease status, or pancreatic disease status. Conclusions: When the patients were stratified by clinical and epidemiological features, the frequencies of the GSTM1 and GSTT1 null genotypes were similar, suggesting that the inherited absence of these enzymatic pathways does not alter the course of CF. However, the high frequency of the F508 CFTR mutation found in younger children suggests that it influences the age at diagnosis of CF in this region of Brazil.

Keywords: : Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator; Glutathione transferase.