Brazilian guidelines for the diagnosis and treatment of cystic fibrosis
Autor(a) principal: | |
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Data de Publicação: | 2017 |
Outros Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Jornal Brasileiro de Pneumologia (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017000300219 |
Resumo: | ABSTRACT Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions. |
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Brazilian guidelines for the diagnosis and treatment of cystic fibrosisCystic fibrosis/diagnosisCystic fibrosis/therapyCystic fibrosis/complicationsPractice guidelineABSTRACT Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions.Sociedade Brasileira de Pneumologia e Tisiologia2017-06-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017000300219Jornal Brasileiro de Pneumologia v.43 n.3 2017reponame:Jornal Brasileiro de Pneumologia (Online)instname:Sociedade Brasileira de Pneumologia e Tisiologia (SBPT)instacron:SBPT10.1590/s1806-37562017000000065info:eu-repo/semantics/openAccessAthanazio,Rodrigo AbensurSilva Filho,Luiz Vicente Ribeiro Ferreira daVergara,Alberto AndradeRibeiro,Antônio FernandoRiedi,Carlos AntônioProcianoy,Elenara da Fonseca AndradeAdde,Fabíola VillacReis,Francisco José CaldeiraRibeiro,José DirceuTorres,Lídia AliceFuccio,Marcelo Bicalho deEpifanio,MatiasFirmida,Mônica de CássiaDamaceno,NeivaLudwig-Neto,NorbertoMaróstica,Paulo José CauduroRached,Samia ZahiMelo,Suzana Fonseca de OliveiraPinto,Leonardo AraújoMonte,Luciana Freitas VellosoHiga,Laurinda Yoko ShinzatoFolescu,Tania WrobelMarson,Fernando Augusto de LimaSad,IsabelaServidoni,Maria de Fátima Correa PimentaKussek,PauloRaskin,SalmoZuana,Adriana DellaAugustin,AlbinHoffmann,AnnelieseBarbisan,BeatrizHochhegger,BrunoLevy,Carlos EmilioVeiga,Claudine Sarmento daRicachinevsky,ClaudioEsposito,ConcettaEscuissato,DanteBrandemburgo,DiegoMarques,ElisabethAquino,Evanirso deFischer,Gilberto BuenoRodrigues,Joaquim CarlosMachado,LeticiaMuramato,LuciaCosta,Lusmaia Damasceno CamargoDonadio,MarcioCastro,Marcos César Santos deRibeiro,Maria AngelaSantana,Maria AngélicaCanan,MarianeAlmeida,Marina Buarque deBritto,MuriloDalcin,Paulo Roth TarsoRamos,Regina Terse TrindadeChiba,SoniaMartins,Valéria de CarvalhoLacerda,ClaudineBarbosa,ElianaGuimarães,Elizabet VilarHessel,GabrielGurmini,JocemaraNeri,LenyciaNogueira,Marcelo CoelhoWayhs,Mônica ChangSimon,Miriam Isabel SantosFernandes,Arlene Gonçalves dos SantosSilva,Claudia de Castro deAlbuquerque,Cristiano Túlio MacielSouza,Edna LúciaSilva,Fernando Antonio de Abreu eDalcin,Paulo de TarsoNoronha,Renata Maria deTeixeira,RicardoMachado,Sandra HelenaCamargo,Spencer MarcantonioRozov,TatianaRodrigues,Ticiana da Costaeng2021-03-19T00:00:00Zoai:scielo:S1806-37132017000300219Revistahttp://www.jornaldepneumologia.com.br/default.aspONGhttps://old.scielo.br/oai/scielo-oai.php||jbp@jbp.org.br|| jpneumo@jornaldepneumologia.com.br1806-37561806-3713opendoar:2021-03-19T00:00Jornal Brasileiro de Pneumologia (Online) - Sociedade Brasileira de Pneumologia e Tisiologia (SBPT)false |
dc.title.none.fl_str_mv |
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis |
title |
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis |
spellingShingle |
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis Athanazio,Rodrigo Abensur Cystic fibrosis/diagnosis Cystic fibrosis/therapy Cystic fibrosis/complications Practice guideline |
title_short |
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis |
title_full |
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis |
title_fullStr |
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis |
title_full_unstemmed |
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis |
title_sort |
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis |
author |
Athanazio,Rodrigo Abensur |
author_facet |
Athanazio,Rodrigo Abensur Silva Filho,Luiz Vicente Ribeiro Ferreira da Vergara,Alberto Andrade Ribeiro,Antônio Fernando Riedi,Carlos Antônio Procianoy,Elenara da Fonseca Andrade Adde,Fabíola Villac Reis,Francisco José Caldeira Ribeiro,José Dirceu Torres,Lídia Alice Fuccio,Marcelo Bicalho de Epifanio,Matias Firmida,Mônica de Cássia Damaceno,Neiva Ludwig-Neto,Norberto Maróstica,Paulo José Cauduro Rached,Samia Zahi Melo,Suzana Fonseca de Oliveira Pinto,Leonardo Araújo Monte,Luciana Freitas Velloso Higa,Laurinda Yoko Shinzato Folescu,Tania Wrobel Marson,Fernando Augusto de Lima Sad,Isabela Servidoni,Maria de Fátima Correa Pimenta Kussek,Paulo Raskin,Salmo Zuana,Adriana Della Augustin,Albin Hoffmann,Anneliese Barbisan,Beatriz Hochhegger,Bruno Levy,Carlos Emilio Veiga,Claudine Sarmento da Ricachinevsky,Claudio Esposito,Concetta Escuissato,Dante Brandemburgo,Diego Marques,Elisabeth Aquino,Evanirso de Fischer,Gilberto Bueno Rodrigues,Joaquim Carlos Machado,Leticia Muramato,Lucia Costa,Lusmaia Damasceno Camargo Donadio,Marcio Castro,Marcos César Santos de Ribeiro,Maria Angela Santana,Maria Angélica Canan,Mariane Almeida,Marina Buarque de Britto,Murilo Dalcin,Paulo Roth Tarso Ramos,Regina Terse Trindade Chiba,Sonia Martins,Valéria de Carvalho Lacerda,Claudine Barbosa,Eliana Guimarães,Elizabet Vilar Hessel,Gabriel Gurmini,Jocemara Neri,Lenycia Nogueira,Marcelo Coelho Wayhs,Mônica Chang Simon,Miriam Isabel Santos Fernandes,Arlene Gonçalves dos Santos Silva,Claudia de Castro de Albuquerque,Cristiano Túlio Maciel Souza,Edna Lúcia Silva,Fernando Antonio de Abreu e Dalcin,Paulo de Tarso Noronha,Renata Maria de Teixeira,Ricardo Machado,Sandra Helena Camargo,Spencer Marcantonio Rozov,Tatiana Rodrigues,Ticiana da Costa |
author_role |
author |
author2 |
Silva Filho,Luiz Vicente Ribeiro Ferreira da Vergara,Alberto Andrade Ribeiro,Antônio Fernando Riedi,Carlos Antônio Procianoy,Elenara da Fonseca Andrade Adde,Fabíola Villac Reis,Francisco José Caldeira Ribeiro,José Dirceu Torres,Lídia Alice Fuccio,Marcelo Bicalho de Epifanio,Matias Firmida,Mônica de Cássia Damaceno,Neiva Ludwig-Neto,Norberto Maróstica,Paulo José Cauduro Rached,Samia Zahi Melo,Suzana Fonseca de Oliveira Pinto,Leonardo Araújo Monte,Luciana Freitas Velloso Higa,Laurinda Yoko Shinzato Folescu,Tania Wrobel Marson,Fernando Augusto de Lima Sad,Isabela Servidoni,Maria de Fátima Correa Pimenta Kussek,Paulo Raskin,Salmo Zuana,Adriana Della Augustin,Albin Hoffmann,Anneliese Barbisan,Beatriz Hochhegger,Bruno Levy,Carlos Emilio Veiga,Claudine Sarmento da Ricachinevsky,Claudio Esposito,Concetta Escuissato,Dante Brandemburgo,Diego Marques,Elisabeth Aquino,Evanirso de Fischer,Gilberto Bueno Rodrigues,Joaquim Carlos Machado,Leticia Muramato,Lucia Costa,Lusmaia Damasceno Camargo Donadio,Marcio Castro,Marcos César Santos de Ribeiro,Maria Angela Santana,Maria Angélica Canan,Mariane Almeida,Marina Buarque de Britto,Murilo Dalcin,Paulo Roth Tarso Ramos,Regina Terse Trindade Chiba,Sonia Martins,Valéria de Carvalho Lacerda,Claudine Barbosa,Eliana Guimarães,Elizabet Vilar Hessel,Gabriel Gurmini,Jocemara Neri,Lenycia Nogueira,Marcelo Coelho Wayhs,Mônica Chang Simon,Miriam Isabel Santos Fernandes,Arlene Gonçalves dos Santos Silva,Claudia de Castro de Albuquerque,Cristiano Túlio Maciel Souza,Edna Lúcia Silva,Fernando Antonio de Abreu e Dalcin,Paulo de Tarso Noronha,Renata Maria de Teixeira,Ricardo Machado,Sandra Helena Camargo,Spencer Marcantonio Rozov,Tatiana Rodrigues,Ticiana da Costa |
author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Athanazio,Rodrigo Abensur Silva Filho,Luiz Vicente Ribeiro Ferreira da Vergara,Alberto Andrade Ribeiro,Antônio Fernando Riedi,Carlos Antônio Procianoy,Elenara da Fonseca Andrade Adde,Fabíola Villac Reis,Francisco José Caldeira Ribeiro,José Dirceu Torres,Lídia Alice Fuccio,Marcelo Bicalho de Epifanio,Matias Firmida,Mônica de Cássia Damaceno,Neiva Ludwig-Neto,Norberto Maróstica,Paulo José Cauduro Rached,Samia Zahi Melo,Suzana Fonseca de Oliveira Pinto,Leonardo Araújo Monte,Luciana Freitas Velloso Higa,Laurinda Yoko Shinzato Folescu,Tania Wrobel Marson,Fernando Augusto de Lima Sad,Isabela Servidoni,Maria de Fátima Correa Pimenta Kussek,Paulo Raskin,Salmo Zuana,Adriana Della Augustin,Albin Hoffmann,Anneliese Barbisan,Beatriz Hochhegger,Bruno Levy,Carlos Emilio Veiga,Claudine Sarmento da Ricachinevsky,Claudio Esposito,Concetta Escuissato,Dante Brandemburgo,Diego Marques,Elisabeth Aquino,Evanirso de Fischer,Gilberto Bueno Rodrigues,Joaquim Carlos Machado,Leticia Muramato,Lucia Costa,Lusmaia Damasceno Camargo Donadio,Marcio Castro,Marcos César Santos de Ribeiro,Maria Angela Santana,Maria Angélica Canan,Mariane Almeida,Marina Buarque de Britto,Murilo Dalcin,Paulo Roth Tarso Ramos,Regina Terse Trindade Chiba,Sonia Martins,Valéria de Carvalho Lacerda,Claudine Barbosa,Eliana Guimarães,Elizabet Vilar Hessel,Gabriel Gurmini,Jocemara Neri,Lenycia Nogueira,Marcelo Coelho Wayhs,Mônica Chang Simon,Miriam Isabel Santos Fernandes,Arlene Gonçalves dos Santos Silva,Claudia de Castro de Albuquerque,Cristiano Túlio Maciel Souza,Edna Lúcia Silva,Fernando Antonio de Abreu e Dalcin,Paulo de Tarso Noronha,Renata Maria de Teixeira,Ricardo Machado,Sandra Helena Camargo,Spencer Marcantonio Rozov,Tatiana Rodrigues,Ticiana da Costa |
dc.subject.por.fl_str_mv |
Cystic fibrosis/diagnosis Cystic fibrosis/therapy Cystic fibrosis/complications Practice guideline |
topic |
Cystic fibrosis/diagnosis Cystic fibrosis/therapy Cystic fibrosis/complications Practice guideline |
description |
ABSTRACT Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions. |
publishDate |
2017 |
dc.date.none.fl_str_mv |
2017-06-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017000300219 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017000300219 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/s1806-37562017000000065 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
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text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Pneumologia e Tisiologia |
publisher.none.fl_str_mv |
Sociedade Brasileira de Pneumologia e Tisiologia |
dc.source.none.fl_str_mv |
Jornal Brasileiro de Pneumologia v.43 n.3 2017 reponame:Jornal Brasileiro de Pneumologia (Online) instname:Sociedade Brasileira de Pneumologia e Tisiologia (SBPT) instacron:SBPT |
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Sociedade Brasileira de Pneumologia e Tisiologia (SBPT) |
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SBPT |
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SBPT |
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Jornal Brasileiro de Pneumologia (Online) |
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Jornal Brasileiro de Pneumologia (Online) |
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Jornal Brasileiro de Pneumologia (Online) - Sociedade Brasileira de Pneumologia e Tisiologia (SBPT) |
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