Brazilian guidelines for the pharmacological treatment of the pulmonary symptoms of cystic fibrosis. Official document of the Sociedade Brasileira de Pneumologia e Tisiologia (SBPT, Brazilian Thoracic Association) Article Swipe
Rodrigo Abensur Athanazio
,
Suzana Érico Tanni
,
Juliana Carvalho Ferreira
,
Paulo de Tarso Roth Dalcin
,
Marcelo Bicalho de Fuccio
,
Concetta Esposito
,
Mariane Gonçalves Martynychen Canan
,
Liana Sousa Coelho
,
Mônica de Cássia Firmida
,
Marina Buarque de Almeida
,
Paulo José Cauduro Maróstica
,
Luciana de Freitas Velloso Monte
,
Edna Lúcia Souza
,
Leonardo Araújo Pinto
,
Samia Zahi Rached
,
Verônica Del Gragnano Stasiak Bednarczuk de Oliveira
,
Luiz Vicente Ribeiro Ferreira da Silva Filho
·
YOU?
·
· 2023
· Open Access
·
· DOI: https://doi.org/10.36416/1806-3756/e20230040
YOU?
·
· 2023
· Open Access
·
· DOI: https://doi.org/10.36416/1806-3756/e20230040
Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.
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- Type
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- Language
- en
- Landing Page
- https://doi.org/10.36416/1806-3756/e20230040
- https://jornaldepneumologia.com.br/export-pdf/3833/2023_49_2_3833_portugues.pdf
- OA Status
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- Cited By
- 6
- References
- 178
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4367693179
All OpenAlex metadata
Raw OpenAlex JSON
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https://openalex.org/W4367693179Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.36416/1806-3756/e20230040Digital Object Identifier
- Title
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Brazilian guidelines for the pharmacological treatment of the pulmonary symptoms of cystic fibrosis. Official document of the Sociedade Brasileira de Pneumologia e Tisiologia (SBPT, Brazilian Thoracic Association)Work title
- Type
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articleOpenAlex work type
- Language
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enPrimary language
- Publication year
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2023Year of publication
- Publication date
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2023-05-02Full publication date if available
- Authors
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Rodrigo Abensur Athanazio, Suzana Érico Tanni, Juliana Carvalho Ferreira, Paulo de Tarso Roth Dalcin, Marcelo Bicalho de Fuccio, Concetta Esposito, Mariane Gonçalves Martynychen Canan, Liana Sousa Coelho, Mônica de Cássia Firmida, Marina Buarque de Almeida, Paulo José Cauduro Maróstica, Luciana de Freitas Velloso Monte, Edna Lúcia Souza, Leonardo Araújo Pinto, Samia Zahi Rached, Verônica Del Gragnano Stasiak Bednarczuk de Oliveira, Luiz Vicente Ribeiro Ferreira da Silva FilhoList of authors in order
- Landing page
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https://doi.org/10.36416/1806-3756/e20230040Publisher landing page
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https://jornaldepneumologia.com.br/export-pdf/3833/2023_49_2_3833_portugues.pdfDirect link to full text PDF
- Open access
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YesWhether a free full text is available
- OA status
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diamondOpen access status per OpenAlex
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https://jornaldepneumologia.com.br/export-pdf/3833/2023_49_2_3833_portugues.pdfDirect OA link when available
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Ivacaftor, Medicine, Cystic fibrosis, Cystic fibrosis transmembrane conductance regulator, Life expectancy, Intensive care medicine, Quality of life (healthcare), Internal medicine, Population, Environmental health, NursingTop concepts (fields/topics) attached by OpenAlex
- Cited by
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6Total citation count in OpenAlex
- Citations by year (recent)
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2025: 3, 2024: 2, 2023: 1Per-year citation counts (last 5 years)
- References (count)
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178Number of works referenced by this work
- Related works (count)
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10Other works algorithmically related by OpenAlex
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