Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil.

BACKGROUND: Advanced lung disease in adult cystic fibrosis (CF) drives most clinical care requirements. The aim was to evaluate outcome (time to death while in the study) in a cohort of adult CF patients with severe lung disease, and to determine the association among baseline patient characteristics and outcome. METHODS: A retrospective cohort study was performed and clinical records between 2000 and 2015 were reviewed. Severe lung disease was defined as forced expiratory volume in the first second (FEV1) < 30% of predicted. Outcomes of all patients, including their date of death or transplantation, were determined till January 1st, 2016. Clinical data were recorded at the entry date. RESULTS: Among 39 subjects included in the study, 20 (51.3%) died, 16 (41.0%) underwent bilateral lung transplantation, and 3 were alive at the end of the study period. Two variables were independently associated with death: body mass index (BMI ≥ 18.5 kg/m2) (HR = 0.78, 95% CI = 0.64-0.96 and p = 0.017) and use of tobramycin inhalation therapy (HR = 3.82, 95% CI = 1.38-10.6 and p = 0.010). Median survival was 37 (95% CI = 16.4-57.6) months. The best cut-off point for BMI was 18.5 kg/m2. Median survival in patients with BMI < 18.5 kg/m2 was 36 months (95% CI = 18.7-53.3). CONCLUSION: Median survival of CF subjects with FEV1 < 30% was 37 months. BMI and tobramycin inhalation therapy were independently associated with death. Median survival in patients with BMI < 18.5 kg/m2 was significantly lower than in patients with BMI ≥ 18.5 kg/m2. The association of tobramycin inhalation with death was interpreted as confounding by severity (use was reserved for advanced lung disease).

Peripheral muscle strength is associated with lung function and functional capacity in patients with cystic fibrosis.

PURPOSE: Cystic fibrosis (CF) is an inherited disease that causes important multisystemic impairments. The present study aimed to evaluate the association of peripheral muscle strength with lung function and functional capacity in adolescents and adults with CF. METHODS: Cross-sectional study with prospective data of patients enrolled in the Program for Adults with CF at Hospital de Clínicas de Porto Alegre. The testing procedures included peripheral muscle strength testing, pulmonary function tests, and the 6-minute walk test. RESULTS: The sample consisted of 41 subjects (27 women) with a mean age of 24.6. Upper extremity muscle strength was associated with forced vital capacity and forced expiratory volume in the first second, and lower extremity muscle strength was associated with the distance covered in the 6-minute walk test, oxygen saturation, forced expiratory volume in the first second, and forced vital capacity. CONCLUSIONS: Muscle strength was positively associated with lung function variable and functional capacity in patients with CF.

Prognostic Factors in Adult Patients with Non-Cystic Fibrosis Bronchiectasis.

BACKGROUND: Non-cystic fibrosis bronchiectasis (NCFB) is a heterogeneous disease. There are few studies about prognostic factors in these patients. Our study aims to assess mortality rates and related factors in a cohort of patients and test the ability of the BSI and FACED scores in predicting mortality in this cohort. METHODS: This was a prospective cohort analysis of 70 patients with NCFB recruited from May 2008 to August 2010. At baseline, patients underwent clinical evaluation, pulmonary function tests, 6-min walk test, and quality of life assessment. Outcomes were defined as favorable (survivors) and unfavorable (survivors who underwent lung transplantation and death from all causes). Baseline records provided data for determination of BSI and FACED. RESULTS: Twenty-seven patients (38.57%) died and 1 (1.43%) underwent lung transplantation. Mean time for occurrence of unfavorable outcomes was 74.67 ± 4.00 months. Main cause of death was an acute infectious exacerbation of bronchiectasis (60.7). Cox regression identified age (p = 0.035; HR 1.04; CI 1.01-1.08), FEV1 % of predicted (p = 0.045; HR 0.97; CI 0.93-0.99), and MEP (p = 0.016; HR 0.96; CI 0.94-0.99) as independent predictors of unfavorable outcomes. FACED was better at predicting unfavorable outcomes in our cohort (log-rank test, FACED p = 0.001 and BSI p = 0.286). In ROC analysis, both scores were similar in predicting unfavorable outcomes (BSI 0.65; FACED 0.66). CONCLUSIONS: Older age, lower FEV1 % of predicted, and lower MEP were independently linked to unfavorable outcomes. FACED and BSI were not accurate in predicting mortality in our cohort.