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BACKGROUND: Availability of clinically effective and cost-effective treatments for severe asthma would be beneficial to patients and national healthcare systems. The aim of this study was to evaluate clinical outcomes and healthcare expenditure after incorporating benralizumab into the standard treatment of refractory eosinophilic asthma. METHODS: This was a cross-sectional multicentre study of consecutive patients with refractory eosinophilic asthma who received treatment with benralizumab during at least 12 months. Patient follow-up was performed in specialised severe asthma units. The main effectiveness parameters measured were: the avoidance of one asthma exacerbation, a 3-point increase in the asthma control test (ACT) score, and the difference in utility scores (health-related quality of life) between a 1-year baseline treatment and 1-year benralizumab treatment. The health economic evaluation included direct costs and incremental cost-effectiveness ratios (ICERs). RESULTS: After 1 year of treatment with benralizumab, patients with refractory eosinophilic asthma showed an improvement in all the effectiveness parameters analysed: improvement of asthma control and lung function, and decrease in the number of exacerbations, oral corticosteroid (both as corticosteroid courses and maintenance therapy), and inhaled corticosteroid use. The total annual cost per patient for the baseline and benralizumab treatment periods were 11,544 and 14,043, respectively, reflecting an increase in costs due to the price of the biological agent but a decrease in costs for the remaining parameters. The ICER was 602 per avoided exacerbation and 983.86 for every 3-point increase in the ACT score. CONCLUSIONS: All the pharmacoeconomic parameters analysed show that treatment with benralizumab is a cost-effective option as an add-on therapy in patients with refractory eosinophilic asthma.
Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Asma/tratamento farmacológico , Custos de Medicamentos , Antiasmáticos/economia , Antiasmáticos/uso terapêutico , Anticorpos Monoclonais Humanizados/economia , Asma/economia , Asma/fisiopatologia , Análise Custo-Benefício , Estudos Transversais , Progressão da Doença , Feminino , Seguimentos , Volume Expiratório Forçado/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Gravidade do Paciente , Resultado do TratamentoRESUMO
BACKGROUND: Benralizumab is a monoclonal antibody that binds to the human interleukin-5 (IL-5) receptor (IL-5R), thereby preventing IL-5 from binding to its receptor and inhibiting differentiation and maturation of eosinophils in the bone marrow. Because of its recent marketing approval, sufficient real-life evidence is lacking to confirm the efficacy and safety data from clinical trials. The purpose of this study was to evaluate the efficacy and safety of benralizumab for the treatment of severe refractory eosinophilic asthma in a real-world cohort of patients. METHODS: This was a cross-sectional multicentre study of consecutive patients with severe refractory eosinophilic asthma who received treatment with benralizumab during at least 6 months. Patient follow-up was performed in specialised severe asthma units. RESULTS: A total of 42 patients were enrolled and treated with benralizumab. Asthma control, as measured by the asthma control test (ACT), improved in all patients both at 3 months of treatment compared with baseline (13.9 ± 4 vs 20.1 ± 3.7, p < 0.001) and at 6 months of treatment compared with the results obtained at 3 months (20.1 ± 3.7 vs 21 ± 2.7, p = 0.037). Similarly, the number of emergency department visits decreased both at 3 months compared with baseline (1 [IR:0.7] vs 0 [IR:0.75], p < 0.001) and at 6 months compared with the results at 3 months (0 [IR:0.75] vs 0 [IR:0], p = 0.012). Reductions in the number of oral corticosteroid cycles, percentage of corticosteroid-dependent patients, and mean daily dose of oral or inhaled corticosteroid were also evidenced. Finally, mean lung function improvement was 291 mL (p < 0.001), and FEV1% improved both at 3 months compared with baseline (64.4 ± 9.3 vs 73.1 ± 9.1, p < 0.001) and at 6 months compared to 3 months (73.1 ± 9.1 vs 76.1 ± 12, p = 0.002). Side effects were mild and did not lead to treatment discontinuation. CONCLUSIONS: This study confirms the efficacy and safety of benralizumab in a real-life setting with improved asthma control and lung function, and a reduced oral and inhaled corticosteroid use as well as fewer emergency department visits. In addition to a rapid initial improvement, it appears that patients continue to improve during the first 6 months of treatment.
Assuntos
Antiasmáticos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Asma/tratamento farmacológico , Eosinofilia/tratamento farmacológico , Corticosteroides/administração & dosagem , Corticosteroides/efeitos adversos , Adulto , Idoso , Estudos Transversais , Progressão da Doença , Eosinófilos/efeitos dos fármacos , Feminino , Volume Expiratório Forçado/efeitos dos fármacos , Humanos , Masculino , Pessoa de Meia-Idade , EspanhaRESUMO
BACKGROUND: Some studies have reported a high prevalence of bronchiectasis in patients with uncontrolled asthma, but the factors associated with this condition are unknown. The objective of this study was to determine the prevalence of bronchiectasis in uncontrolled moderate-to-severe asthma and to identify risk factors and their correlation with bronchiectasis in these patients. METHODS: This is a prospective study of data from consecutive patients with uncontrolled moderate-to-severe asthma. Diagnosis of bronchiectasis was based on high-resolution computed tomography. A prognostic score was developed using a logistic regression model, which was used to determine the factors associated with bronchiectasis. RESULTS: A total of 398 patients (60% with severe asthma) were included. The prevalence of bronchiectasis was 28.4%. The presence of bronchiectasis was associated with a higher frequency of chronic expectoration (OR, 2.95; 95% CI, 1.49-5.84; p = 0.002), greater severity of asthma (OR, 2.43; 95% CI, 1.29-4.57; p = 0.006), at least one previous episode of pneumonia (OR, 2.42; 95% CI, 1.03-5.69; p = 0.044), and lower levels of FeNO (OR, 0.98; 95% CI, 0.97-0.99; p = 0.016). The NOPES score was developed on the basis of these variables (FeNO[cut off point 20.5 ppb], Pneumonia, Expectoration and asthma Severity), and it ranges from 0 to 4 points, where 0 means "no risk" and 4 corresponds to "high risk". The NOPES score yielded an AUC-ROC of 70% for the diagnosis of bronchiectasis, with a specificity of 95%. CONCLUSIONS: Almost a third of the patients with uncontrolled moderate-to-severe asthma had bronchiectasis. Bronchiectasis was related to the severity of asthma, the presence of chronic expectoration, a previous history of pneumonia, and lower levels of FeNO. The NOPES score is an easy-to-use scoring system with a high prognostic value for bronchiectasis in patients with uncontrolled moderate-to-severe asthma.
Assuntos
Asma/diagnóstico , Asma/fisiopatologia , Bronquiectasia/diagnóstico , Bronquiectasia/fisiopatologia , Índice de Gravidade de Doença , Adulto , Idoso , Asma/epidemiologia , Testes Respiratórios/métodos , Bronquiectasia/epidemiologia , Feminino , Seguimentos , Volume Expiratório Forçado/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Escarro/fisiologiaRESUMO
BACKGROUND: Data on the prevalence of and factors associated with nontuberculous mycobacteria (NTM) in patients with non-cystic fibrosis (CF) bronchiectasis are limited. Our aim was to determine the prevalence and factors associated with isolation of NTM in this population. METHODS: We performed a multicenter observational study of historical cohorts comprising consecutive patients with non-CF bronchiectasis and at least 2 sputum samples cultured for mycobacteria over a period of 5 years. RESULTS: The study population included 218 adult patients (61.9 % women) with a mean (SD) age of 55.7 (16) years and a mean (SD) of 5.1 (3.3) cultures/patient. NTM was isolated from sputum in 18 patients (8.3 %). Of these, 5 patients (28 %) met the American Thoracic Society criteria for NTM disease. Mycobacterium avium complex was the most frequently isolated microorganism (9 patients, 4.1 %). The variables independently associated with isolation of NTM were FVC ≥ 75 % predicted (OR, 4.84; 95 % CI 1.47 to 15.9; p < 0.05), age ≥ 50 years (OR, 4.74; 95 % CI 1.25 to 17.97; p < 0.05), and body mass index (BMI) ≤ 23 kg/m(2) (OR, 2.97; 95 % CI 1.03-8.58; p < 0.05). Patients with these three characteristics had a 40 % probability of having at least one isolation of NMT. CONCLUSIONS: A significant number of patients with non-CF bronchiectasis are positive for the isolation of NTM. M. avium complex is the most frequently isolated mycobacteria. FVC ≥ 75 % predicted, age ≥ 50 years, and a BMI ≤ 23 kg/m(2) were independently associated with the presence of NTM in patients with non-CF bronchiectasis.
Assuntos
Bronquiectasia/complicações , Infecções por Mycobacterium não Tuberculosas/diagnóstico , Micobactérias não Tuberculosas/isolamento & purificação , Adulto , Fatores Etários , Idoso , Índice de Massa Corporal , Bronquiectasia/diagnóstico por imagem , Fibrose Cística/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Infecções por Mycobacterium não Tuberculosas/complicações , Infecções por Mycobacterium não Tuberculosas/microbiologia , Complexo Mycobacterium avium/isolamento & purificação , Razão de Chances , Prevalência , Escarro/microbiologia , Tomografia Computadorizada por Raios XRESUMO
RATIONALE: The baseline value of eosinophils in peripheral blood (BEC) has been associated with different degrees of severity, prognosis and response to treatment in patients with bronchiectasis. It is not known, however, if this basal value remains constant over time. OBJECTIVES: The aim of this study was to assess whether the BEC remains stable in the long term in patients with bronchiectasis. METHODS AND MEASUREMENTS: Patients from the RIBRON registry of bronchiectasis diagnosed by computed tomography with at least 2 BEC measurements one year apart were included in the study. Patients with asthma and those taking anti-eosinophilic drugs were excluded. Reliability was assessed using the intra-class correlation coefficient (ICC). A patient with a BEC of at least 300 cells/uL or less than 100 cells/uL was considered eosinophilic or eosinopenic, respectively. Group changes over time were also calculated. MAIN RESULTS: Seven hundred and thirteen patients were finally included, with a mean age of 66.5 (13.2) years (65.8 % women). A total of 2701 BEC measurements were performed, with a median number of measurements per patient of 4 (IQR: 2-5) separated by a median of 12.1 (IQR: 10.5-14.3) months between two consecutive measurements. The ICC was good (>0.75) when calculated between two consecutive measurements (approximately one year apart) but had dropped significantly by the time of the next annual measurements. Similarly, the change from an eosinophilic or eosinopenic patient to a non-eosinophilic or non-eosinopenic patient, respectively, was less than 30 % during the first year with respect to the baseline value but was close to 50 % in later measurements. CONCLUSIONS: Given the significant changes observed in the baseline value of the BEC over time, its monitoring is necessary in patients with bronchiectasis in order to more reliably assess its usefulness.
RESUMO
OBJECTIVES: The objective of this study was to analyse lung function decline over time in bronchiectasis, along with the factors associated with it. METHODS: Spirometry was measured every year in this observational, prospective study in 849 patients from the Spanish Bronchiectasis Registry (RIBRON). The main outcome was the decline in the rate of forced expiratory volume during the first second (FEV1). To be included in this study, patients needed a baseline assessment and at least one subsequent assessment. FEV1 decline was analysed using a mixed-effects linear regression model adjusted for clinically significant variables. RESULTS: We recruited 849 bronchiectasis patients with at least two annual lung function measurements (follow-up range 1-4 years). A total of 2262 lung function tests were performed (mean 2.66 per patient, range 2-5). Mean baseline FEV1 was 1.78 L (standard deviation (SD) 0.76; 71.3% predicted). Mean age was 69.1 (SD 15.4) years; 543 (64% women. The adjusted rates of FEV1 decline were -0.98% predicted/year (95% confidence interval (CI) -2.41 to -0.69) and -31.6 (95% CI -44.4 to -18.8) mL. The annual FEV1 decline was faster in those patients with chronic bronchial infection by Pseudomonas aeruginosa (-1.37% (52.1 mL) vs -0.37% (-24.6 mL); p < 0.001), greater age, increased number of severe exacerbations in the previous year and higher baseline FEV1 value. DISCUSSION: In patients with bronchiectasis, the annual rate of FEV1 decline was -31.6 mL/year and it was faster in older patients and those with chronic bronchial infection by P. aeruginosa, increased number of previous severe exacerbations and higher baseline FEV1 value.
Assuntos
Bronquiectasia/complicações , Bronquiectasia/microbiologia , Infecções por Pseudomonas/complicações , Infecções por Pseudomonas/microbiologia , Pseudomonas aeruginosa , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes de Função RespiratóriaRESUMO
The prevalence of osteopenia/osteoporosis has not been sufficiently studied in people with bronchiectasis not due to cystic fibrosis (BC), nor has its relationship with other variables (clinical, body composition and bone turnover and inflammation markers) been sufficiently studied. Our aim was to determine the prevalence of osteopenia and osteoporosis and related factors in patients with BC. We did a cross-sectional study in people with BC in a clinically stable state. Spirometric parameters, annual exacerbations and analysis with bone turnover markers (BTM) and inflammation markers were evaluated. Densitometry (DXA) was performed for body composition, bone mineral density (BMD) and handgrip strength. 123 patients were studied (65% women, mean age 49.6 ± 18.8, Body Mass Index (BMI) 24.8 ± 4.7 kg/m2). 62.8% and 62.5% of men and women, respectively, had normal bone mineral density, 30.2% and 22.2% osteopenia and 7% and 15% osteoporosis. 52 patients (56.2%) had low fat-free mass: 68.9% women and 28.6% men. Patients with decreased bone mass had significantly lower muscle strength, maximum expiratory volume in the first second (FEV1%), vitamin D, higher levels of C-terminal telopeptide of type 1 collagen (CTX) and total osteocalcin and underarboxylated osteocalcin (ucOC). We observed significant and negative correlations between BMD and the number of serious exacerbations per year CTX and undercarboxylated osteocalcin. We observed significant positive correlations between BMD, fat free mass index (FFMI) and handgrip dynamometry. The study suggest that the prevalence of osteoporosis was high in relation to the demographic characteristics. Respiratory parameters, body composition, muscle strength and bone remodeling markers were associated with a lower bone mineral density.
Assuntos
Biomarcadores/metabolismo , Doenças Ósseas Metabólicas/epidemiologia , Bronquiectasia/epidemiologia , Osteoporose/epidemiologia , Adulto , Idoso , Composição Corporal/fisiologia , Doenças Ósseas Metabólicas/complicações , Doenças Ósseas Metabólicas/metabolismo , Doenças Ósseas Metabólicas/fisiopatologia , Remodelação Óssea/genética , Bronquiectasia/complicações , Bronquiectasia/metabolismo , Bronquiectasia/fisiopatologia , Feminino , Força da Mão/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Força Muscular/genética , Força Muscular/fisiologia , Osteoporose/complicações , Osteoporose/metabolismo , Osteoporose/fisiopatologiaRESUMO
BACKGROUND: Although the FACED score has demonstrated a great prognostic capacity in bronchiectasis, it does not include the number or severity of exacerbations as a separate variable, which is important in the natural history of these patients. OBJECTIVE: Construction and external validation of a new index, the E-FACED, to evaluate the predictive capacity of exacerbations and mortality. METHODS: The new score was constructed on the basis of the complete cohort for the construction of the original FACED score, while the external validation was undertaken with six cohorts from three countries (Brazil, Argentina, and Chile). The main outcome was the number of annual exacerbations/hospitalizations, with all-cause and respiratory-related deaths as the secondary outcomes. A statistical evaluation comprised the relative weight and ideal cut-off point for the number or severity of the exacerbations and was incorporated into the FACED score (E-FACED). The results obtained after the application of FACED and E-FACED were compared in both the cohorts. RESULTS: A total of 1,470 patients with bronchiectasis (819 from the construction cohorts and 651 from the external validation cohorts) were followed up for 5 years after diagnosis. The best cut-off point was at least two exacerbations in the previous year (two additional points), meaning that the E-FACED has nine points of growing severity. E-FACED presented an excellent prognostic capacity for exacerbations (areas under the receiver operating characteristic curve: 0.82 for at least two exacerbations in 1 year and 0.87 for at least one hospitalization in 1 year) that was statistically better than that of the FACED score (0.72 and 0.78, P<0.05, respectively). The predictive capacities for all-cause and respiratory mortality were 0.87 and 0.86, respectively, with both being similar to those of the FACED. CONCLUSION: E-FACED score significantly increases the FACED capacity to predict future yearly exacerbations while maintaining the score's simplicity and prognostic capacity for death.
Assuntos
Bronquiectasia/diagnóstico , Indicadores Básicos de Saúde , Nível de Saúde , Pulmão/fisiopatologia , Adulto , Fatores Etários , Idoso , Área Sob a Curva , Argentina , Brasil , Bronquiectasia/mortalidade , Bronquiectasia/fisiopatologia , Bronquiectasia/terapia , Causas de Morte , Chile , Progressão da Doença , Dispneia/fisiopatologia , Feminino , Volume Expiratório Forçado , Hospitalização , Humanos , Pulmão/microbiologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Infecções por Pseudomonas/diagnóstico , Infecções por Pseudomonas/microbiologia , Pseudomonas aeruginosa/isolamento & purificação , Curva ROC , Reprodutibilidade dos Testes , Infecções Respiratórias/diagnóstico , Infecções Respiratórias/microbiologia , Fatores de Risco , Índice de Gravidade de Doença , Fatores de TempoRESUMO
BACKGROUND: Cystic fibrosis (CF) is a chronic disease with an impact on the quality of life. Self-reported symptoms of depression and anxiety were assessed in the Spanish cohort of the International Epidemiological Study on Depression and Anxiety in patients with CF (International Depression-Anxiety Epidemiological Study) and their relationship with health status and health-related quality of life (HRQoL) was evaluated. METHODS: This cross-sectional study recruited adult patients with CF at 10 Spanish centers. Patients completed the Hospital Anxiety and Depression Scale (HADS) and the Revised Cystic Fibrosis Questionnaire. Demographic and health data were recorded from medical charts. Logistic regression was used to determine the predictors of elevated symptoms of depression and anxiety (HADS≥8). RESULTS: Of the 336 participants recruited (mean age, 28.1years; 48.2% women), 41 (12.2%) had elevated depression-related scores, and 100 (29.7%) had elevated anxiety-related scores (HADS≥8). After adjusting for confounders, only less education, intravenous antibiotics, psychiatric medications and psychotherapy were significantly associated with elevated psychological symptoms. Specifically, regardless of lung function, patients who were depressed or anxious reported worse HRQoL. CONCLUSIONS: The prevalence of elevated symptoms of depression and anxiety was high in Spanish adult patients with CF, and these symptoms were associated with a decreased HRQoL.
Assuntos
Ansiedade/diagnóstico , Fibrose Cística/psicologia , Depressão/diagnóstico , Qualidade de Vida , Adolescente , Adulto , Ansiedade/epidemiologia , Estudos Transversais , Fibrose Cística/epidemiologia , Depressão/epidemiologia , Feminino , Humanos , Masculino , Espanha/epidemiologia , Adulto JovemRESUMO
OBJECTIVES: To assess the prevalence of impaired glucose tolerance and diabetes mellitus in a group of patients with cystic fibrosis and to compare insulin secretion, haemoglobin A1c, age, gender, genotype, and clinical status related variables between the groups with abnormal (impaired glucose tolerance and diabetes mellitus) and normal carbohydrate metabolism. PATIENTS AND METHODS: 66 patients with cystic fibrosis (age 1-38 years). Plasma glucose, insulin and C peptide determinations during an oral glucose tolerance test (OGTT) in 65 patients (one was previously known as diabetic). Based on the Expert Committee from the American Diabetes Association (1997), patients were classified as having impaired glucose tolerance and diabetes mellitus. Haemoglobin A1c, presence of delta F508 mutation, date of diagnosis and first sputum colonization, scores of National Institutes of Health, Schwachman and Chrispin-Norman, pancreatic enzyme intake, weight, body mass index, forced expiratory volume in one second and forced vital capacity determinations in every patient. Comparative analysis of these variables in both groups of patients was performed by Student test. RESULTS: Nine patients (13.6%) showed impaired tolerance glucose and one diabetes mellitus following OGTT, so we have two diabetics in our cystic fibrosis group (3.0%). When compared to cystic fibrosis patients with normal glucose tolerance, tolerance glucose and diabetes mellitus patients had significantly reduced basal insulin levels (8.6 [3.8] vs 15.0 [22.2] microU/ml; p < 0.0001), increased glucose stimulated insulin and C peptide levels (50.2 [19.3] vs 21.4 [19.3] microU/ml; p < 0.0001, and 9.0 [5.9] vs 4.4 [3.2] ng/ml; p < 0.0001), they were older (18.0 [7.5] vs 12.7 [7.3] years old; p < 0.05) and had longer time since diagnosis and since first sputum colonization. The remaining variables did not differ between the two groups. All patients with exocrine pancreatic sufficiency showed normal glucose tolerance. CONCLUSIONS: Abnormalities in carbohydrate metabolism were present in 16.6% of cystic fibrosis patients. These patients had reduced basal but increased glucose stimulated insulin levels. Age, time since diagnosis of cystic fibrosis, time since first sputum colonization and exocrine pancreatic insufficiency are the variables being associated with carbohydrate metabolism abnormalities.
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Metabolismo dos Carboidratos , Fibrose Cística/complicações , Doenças Metabólicas/complicações , Doenças Metabólicas/diagnóstico , Adolescente , Adulto , Glicemia/metabolismo , Criança , Pré-Escolar , Estudos Transversais , Complicações do Diabetes , Diabetes Mellitus/epidemiologia , Feminino , Teste de Tolerância a Glucose , Hemoglobina A/metabolismo , Humanos , Lactente , Insulina/metabolismo , Secreção de Insulina , Masculino , Doenças Metabólicas/epidemiologia , Plasma , Prevalência , Índice de Gravidade de DoençaAssuntos
Processamento Alternativo/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/genética , Deleção de Sequência/genética , Alelos , Fibrose Cística/patologia , DNA/química , DNA/genética , Análise Mutacional de DNA , Europa (Continente) , Feminino , Genótipo , Haplótipos , Homozigoto , Humanos , Masculino , Fenótipo , Mutação Puntual , Índice de Gravidade de DoençaRESUMO
1. The in vitro production of ferrous-induced lipid peroxidation was 5.71 times higher in rat lung tissue than in human lung membranes. 2. The pyrimido-pyrimidine derivative RA-642 shows a more potent inhibition of ferrous-induced lipid peroxidation than dipyridamole; mopidamol had no effect. All the compounds showed higher anti-peroxidative effect in rat than in human lung tissue.
Assuntos
Antioxidantes/farmacologia , Dipiridamol/farmacologia , Compostos Ferrosos/antagonistas & inibidores , Peroxidação de Lipídeos/efeitos dos fármacos , Pulmão/metabolismo , Mopidamol/farmacologia , Pirimidinas/farmacologia , Adulto , Idoso , Animais , Feminino , Compostos Ferrosos/farmacologia , Humanos , Técnicas In Vitro , Pulmão/efeitos dos fármacos , Masculino , Malondialdeído/metabolismo , Pessoa de Meia-Idade , Ratos , Ratos Wistar , Substâncias Reativas com Ácido Tiobarbitúrico/metabolismo , Vitamina E/farmacologiaRESUMO
While all patients with cystic fibrosis (CF) have mutations in both CFTR alleles, often only one CFTR change is detected in patients with other lung disorders. The aim of this study was to investigate whether heterozygosity for CFTR mutations could be a determinant risk factor in the development of bronchiectasis in adult patients. We have performed the CFTR gene analysis in a cohort of 55 bronchiectasis adult patients with unknown etiology. The 5T variant (TG)m and the M470V polymorphisms were also analyzed. A general population in which the same molecular analysis was previously performed was used as the control group. The mutational spectrum of patients was also compared with that found in our CF population. CFTR mutations/variants were found in 20 patients (36%), 14 with only one mutant gene (25%). All six patients colonized by Staphylococcus aureus presented with at least one CFTR change (p = 0.001). No statistical significance was observed between patients with and without mutations for other clinical features. The 5T variant was found in four patients. Additionally, 90% of patients with mutations had the more functional M470 allele (p < 0.001). These results suggest the involvement of the CFTR gene in bronchiectasis of unknown etiology in adult patients.