Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil
Autor(a) principal: | |
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Data de Publicação: | 2020 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UFRGS |
Texto Completo: | http://hdl.handle.net/10183/223218 |
Resumo: | 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). |
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Silva, Guilherme Figueiredo daSimmonds, Nicholas J.Dalcin, Paulo de Tarso Roth2021-07-06T04:45:58Z20201471-2466http://hdl.handle.net/10183/223218001126906Background: 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).application/pdfengBMC Pulmonary medicine. London. Vol, 20 (2020), 194, 8 p.Fibrose císticaAnálise de sobrevidaPneumopatiasPorto Alegre (RS)Cystic fibrosisFEV1Advanced lung diseaseSurvivalClinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern BrazilEstrangeiroinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFRGSinstname:Universidade Federal do Rio Grande do Sul (UFRGS)instacron:UFRGSTEXT001126906.pdf.txt001126906.pdf.txtExtracted Texttext/plain39443http://www.lume.ufrgs.br/bitstream/10183/223218/2/001126906.pdf.txt7a5c3dcf9278b5f9277bd2182381bc09MD52ORIGINAL001126906.pdfTexto completo (inglês)application/pdf676843http://www.lume.ufrgs.br/bitstream/10183/223218/1/001126906.pdfc868327108a7be769a32f7f23aeab8d0MD5110183/2232182021-08-04 04:42:32.905271oai:www.lume.ufrgs.br:10183/223218Repositório de PublicaçõesPUBhttps://lume.ufrgs.br/oai/requestopendoar:2021-08-04T07:42:32Repositório Institucional da UFRGS - Universidade Federal do Rio Grande do Sul (UFRGS)false |
dc.title.pt_BR.fl_str_mv |
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil |
title |
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil |
spellingShingle |
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil Silva, Guilherme Figueiredo da Fibrose cística Análise de sobrevida Pneumopatias Porto Alegre (RS) Cystic fibrosis FEV1 Advanced lung disease Survival |
title_short |
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil |
title_full |
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil |
title_fullStr |
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil |
title_full_unstemmed |
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil |
title_sort |
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil |
author |
Silva, Guilherme Figueiredo da |
author_facet |
Silva, Guilherme Figueiredo da Simmonds, Nicholas J. Dalcin, Paulo de Tarso Roth |
author_role |
author |
author2 |
Simmonds, Nicholas J. Dalcin, Paulo de Tarso Roth |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Silva, Guilherme Figueiredo da Simmonds, Nicholas J. Dalcin, Paulo de Tarso Roth |
dc.subject.por.fl_str_mv |
Fibrose cística Análise de sobrevida Pneumopatias Porto Alegre (RS) |
topic |
Fibrose cística Análise de sobrevida Pneumopatias Porto Alegre (RS) Cystic fibrosis FEV1 Advanced lung disease Survival |
dc.subject.eng.fl_str_mv |
Cystic fibrosis FEV1 Advanced lung disease Survival |
description |
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). |
publishDate |
2020 |
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2020 |
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2021-07-06T04:45:58Z |
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1471-2466 |
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001126906 |
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1471-2466 001126906 |
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http://hdl.handle.net/10183/223218 |
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BMC Pulmonary medicine. London. Vol, 20 (2020), 194, 8 p. |
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