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Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil
dc.contributor.author | Silva, Guilherme Figueiredo da | pt_BR |
dc.contributor.author | Simmonds, Nicholas J. | pt_BR |
dc.contributor.author | Dalcin, Paulo de Tarso Roth | pt_BR |
dc.date.accessioned | 2021-07-06T04:45:58Z | pt_BR |
dc.date.issued | 2020 | pt_BR |
dc.identifier.issn | 1471-2466 | pt_BR |
dc.identifier.uri | http://hdl.handle.net/10183/223218 | pt_BR |
dc.description.abstract | 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). | en |
dc.format.mimetype | application/pdf | pt_BR |
dc.language.iso | eng | pt_BR |
dc.relation.ispartof | BMC Pulmonary medicine. London. Vol, 20 (2020), 194, 8 p. | pt_BR |
dc.rights | Open Access | en |
dc.subject | Cystic fibrosis | en |
dc.subject | Fibrose cística | pt_BR |
dc.subject | Análise de sobrevida | pt_BR |
dc.subject | FEV1 | en |
dc.subject | Advanced lung disease | en |
dc.subject | Pneumopatias | pt_BR |
dc.subject | Porto Alegre (RS) | pt_BR |
dc.subject | Survival | en |
dc.title | Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil | pt_BR |
dc.type | Artigo de periódico | pt_BR |
dc.identifier.nrb | 001126906 | pt_BR |
dc.type.origin | Estrangeiro | pt_BR |
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