Benzbromarone as adjuvant therapy for cystic fibrosis lung disease: a pilot clinical trial
Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing CF transmembrane conductance regulator-specific treatments for CF lung disease, exploring alternative CF-targeted medications seems reasonable. We sought...
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Published in | Jornal brasileiro de pneumologia Vol. 50; no. 3; p. e20230292 |
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Sociedade Brasileira de Pneumologia e Tisiologia
2024
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Abstract | Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing CF transmembrane conductance regulator-specific treatments for CF lung disease, exploring alternative CF-targeted medications seems reasonable. We sought to evaluate the potential beneficial effects of oral benzbromarone as an adjuvant therapy in CF patients with reduced lung function.
This was a prospective open-label pilot study of oral benzbromarone (100 mg/day) administered once daily for 90 days. Patients were followed at a tertiary referral center in southern Brazil. Safety was assessed by the number of reported adverse events. Secondary objectives included percent predicted FEV1 (FEV1%) and pulmonary exacerbations.
Ten patients were enrolled. Benzbromarone was found to be safe, with no serious drug-related adverse events. Eight patients completed the study; the median relative change in FEV1% tended to increase during the treatment, showing an 8% increase from baseline at the final visit. However, a nonparametric test showed that the change was not significant (p = 0.06). Of a total of ten patients, only one experienced at least one pulmonary exacerbation during the study.
Oral benzbromarone appears to be safe, and improved FEV1% has been observed in patients with CF. Further assessment in larger trials is warranted to elucidate whether oral benzbromarone can be a potential adjuvant therapy for CF. |
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AbstractList | Objective: Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing CF transmembrane conductance regulator-specific treatments for CF lung disease, exploring alternative CF-targeted medications seems reasonable. We sought to evaluate the potential beneficial effects of oral benzbromarone as an adjuvant therapy in CF patients with reduced lung function. Methods: This was a prospective open-label pilot study of oral benzbromarone (100 mg/day) administered once daily for 90 days. Patients were followed at a tertiary referral center in southern Brazil. Safety was assessed by the number of reported adverse events. Secondary objectives included percent predicted FEV1 (FEV1%) and pulmonary exacerbations. Results: Ten patients were enrolled. Benzbromarone was found to be safe, with no serious drug-related adverse events. Eight patients completed the study; the median relative change in FEV1% tended to increase during the treatment, showing an 8% increase from baseline at the final visit. However, a nonparametric test showed that the change was not significant (p = 0.06). Of a total of ten patients, only one experienced at least one pulmonary exacerbation during the study. Conclusions: Oral benzbromarone appears to be safe, and improved FEV1% has been observed in patients with CF. Further assessment in larger trials is warranted to elucidate whether oral benzbromarone can be a potential adjuvant therapy for CF. ABSTRACT Objective: Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing CF transmembrane conductance regulator-specific treatments for CF lung disease, exploring alternative CF-targeted medications seems reasonable. We sought to evaluate the potential beneficial effects of oral benzbromarone as an adjuvant therapy in CF patients with reduced lung function. Methods: This was a prospective open-label pilot study of oral benzbromarone (100 mg/day) administered once daily for 90 days. Patients were followed at a tertiary referral center in southern Brazil. Safety was assessed by the number of reported adverse events. Secondary objectives included percent predicted FEV1 (FEV1%) and pulmonary exacerbations. Results: Ten patients were enrolled. Benzbromarone was found to be safe, with no serious drug-related adverse events. Eight patients completed the study; the median relative change in FEV1% tended to increase during the treatment, showing an 8% increase from baseline at the final visit. However, a nonparametric test showed that the change was not significant (p = 0.06). Of a total of ten patients, only one experienced at least one pulmonary exacerbation during the study. Conclusions: Oral benzbromarone appears to be safe, and improved FEV1% has been observed in patients with CF. Further assessment in larger trials is warranted to elucidate whether oral benzbromarone can be a potential adjuvant therapy for CF. Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing CF transmembrane conductance regulator-specific treatments for CF lung disease, exploring alternative CF-targeted medications seems reasonable. We sought to evaluate the potential beneficial effects of oral benzbromarone as an adjuvant therapy in CF patients with reduced lung function. This was a prospective open-label pilot study of oral benzbromarone (100 mg/day) administered once daily for 90 days. Patients were followed at a tertiary referral center in southern Brazil. Safety was assessed by the number of reported adverse events. Secondary objectives included percent predicted FEV1 (FEV1%) and pulmonary exacerbations. Ten patients were enrolled. Benzbromarone was found to be safe, with no serious drug-related adverse events. Eight patients completed the study; the median relative change in FEV1% tended to increase during the treatment, showing an 8% increase from baseline at the final visit. However, a nonparametric test showed that the change was not significant (p = 0.06). Of a total of ten patients, only one experienced at least one pulmonary exacerbation during the study. Oral benzbromarone appears to be safe, and improved FEV1% has been observed in patients with CF. Further assessment in larger trials is warranted to elucidate whether oral benzbromarone can be a potential adjuvant therapy for CF. Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing CF transmembrane conductance regulator-specific treatments for CF lung disease, exploring alternative CF-targeted medications seems reasonable. We sought to evaluate the potential beneficial effects of oral benzbromarone as an adjuvant therapy in CF patients with reduced lung function.OBJECTIVECystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing CF transmembrane conductance regulator-specific treatments for CF lung disease, exploring alternative CF-targeted medications seems reasonable. We sought to evaluate the potential beneficial effects of oral benzbromarone as an adjuvant therapy in CF patients with reduced lung function.This was a prospective open-label pilot study of oral benzbromarone (100 mg/day) administered once daily for 90 days. Patients were followed at a tertiary referral center in southern Brazil. Safety was assessed by the number of reported adverse events. Secondary objectives included percent predicted FEV1 (FEV1%) and pulmonary exacerbations.METHODSThis was a prospective open-label pilot study of oral benzbromarone (100 mg/day) administered once daily for 90 days. Patients were followed at a tertiary referral center in southern Brazil. Safety was assessed by the number of reported adverse events. Secondary objectives included percent predicted FEV1 (FEV1%) and pulmonary exacerbations.Ten patients were enrolled. Benzbromarone was found to be safe, with no serious drug-related adverse events. Eight patients completed the study; the median relative change in FEV1% tended to increase during the treatment, showing an 8% increase from baseline at the final visit. However, a nonparametric test showed that the change was not significant (p = 0.06). Of a total of ten patients, only one experienced at least one pulmonary exacerbation during the study.RESULTSTen patients were enrolled. Benzbromarone was found to be safe, with no serious drug-related adverse events. Eight patients completed the study; the median relative change in FEV1% tended to increase during the treatment, showing an 8% increase from baseline at the final visit. However, a nonparametric test showed that the change was not significant (p = 0.06). Of a total of ten patients, only one experienced at least one pulmonary exacerbation during the study.Oral benzbromarone appears to be safe, and improved FEV1% has been observed in patients with CF. Further assessment in larger trials is warranted to elucidate whether oral benzbromarone can be a potential adjuvant therapy for CF.CONCLUSIONSOral benzbromarone appears to be safe, and improved FEV1% has been observed in patients with CF. Further assessment in larger trials is warranted to elucidate whether oral benzbromarone can be a potential adjuvant therapy for CF. |
Author | Pieta, Marina Puerari Antunes, Marcos Otávio Brum Friedrich, Frederico Petry, Lucas Montiel Meneses, Amanda da Silva Bittencourt, Luana Braga Xavier, Luiza Fernandes Grun, Lucas Kich Lumertz, Magali Kunzelmann, Karl Garcia, Laura de Castro E Pinto, Leonardo Araujo |
AuthorAffiliation | 1 . Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil 2 . Laboratório de Imunobiologia, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil 3 . Physiological Institute, University of Regensburg, Regensburg, Germany |
AuthorAffiliation_xml | – sequence: 0 name: University of Regensburg – sequence: 0 name: Pontifícia Universidade Católica do Rio Grande do Sul – name: 3 . Physiological Institute, University of Regensburg, Regensburg, Germany – name: 1 . Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – name: 2 . Laboratório de Imunobiologia, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil |
Author_xml | – sequence: 1 givenname: Frederico orcidid: 0000-0001-6939-0340 surname: Friedrich fullname: Friedrich, Frederico organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 2 givenname: Lucas Montiel orcidid: 0000-0002-9692-7593 surname: Petry fullname: Petry, Lucas Montiel organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 3 givenname: Laura de Castro E orcidid: 0000-0003-2339-8675 surname: Garcia fullname: Garcia, Laura de Castro E organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 4 givenname: Marina Puerari orcidid: 0000-0002-9965-3954 surname: Pieta fullname: Pieta, Marina Puerari organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 5 givenname: Amanda da Silva orcidid: 0000-0002-1008-5998 surname: Meneses fullname: Meneses, Amanda da Silva organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 6 givenname: Luana Braga orcidid: 0000-0002-2646-081X surname: Bittencourt fullname: Bittencourt, Luana Braga organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 7 givenname: Luiza Fernandes orcidid: 0000-0001-8848-2806 surname: Xavier fullname: Xavier, Luiza Fernandes organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 8 givenname: Marcos Otávio Brum orcidid: 0000-0001-8873-2385 surname: Antunes fullname: Antunes, Marcos Otávio Brum organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 9 givenname: Lucas Kich orcidid: 0000-0002-1446-6552 surname: Grun fullname: Grun, Lucas Kich organization: Laboratório de Imunobiologia, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 10 givenname: Magali orcidid: 0000-0001-9282-272X surname: Lumertz fullname: Lumertz, Magali organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil – sequence: 11 givenname: Karl orcidid: 0000-0002-4583-7037 surname: Kunzelmann fullname: Kunzelmann, Karl organization: Physiological Institute, University of Regensburg, Regensburg, Germany – sequence: 12 givenname: Leonardo Araujo orcidid: 0000-0002-3906-5456 surname: Pinto fullname: Pinto, Leonardo Araujo organization: Escola de Medicina, Pontifícia Universidade Católica do Rio Grande do Sul - PUCRS - Porto Alegre (RS) Brasil |
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Snippet | Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing CF... Objective: Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in developing... ABSTRACT Objective: Cystic fibrosis (CF) affects multiple organs, the most severe consequences being observed in the lungs. Despite significant progress in... |
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SubjectTerms | Adolescent Adult Benzbromarone - administration & dosage Benzbromarone - therapeutic use Chemotherapy, Adjuvant Cystic Fibrosis - drug therapy Cystic Fibrosis - physiopathology Cystic fibrosis/therapy Female Forced Expiratory Volume - drug effects Humans Male Mucociliary clearance Original Pilot Projects Prospective Studies RESPIRATORY SYSTEM respiratory tract diseases Statistics, Nonparametric Time Factors Treatment Outcome Uricosuric Agents - therapeutic use Young Adult |
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Title | Benzbromarone as adjuvant therapy for cystic fibrosis lung disease: a pilot clinical trial |
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