Brazilian guidelines for the diagnosis and treatment of cystic fibrosis

Brazilian guidelines for the diagnosis and treatment of cystic fibrosis

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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Main Authors: 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
Format: Digital revista
Language:English
Published: Sociedade Brasileira de Pneumologia e Tisiologia 2017
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017000300219
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Summary: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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