Brazilian guidelines for the diagnosis and treatment of cystic fibrosis
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Published:2017-06
Issue:3
Volume:43
Page:219-245
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ISSN:1806-3756
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Container-title:Jornal Brasileiro de Pneumologia
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language:
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Short-container-title:J. bras. pneumol.
Author:
Athanazio Rodrigo Abensur1, Silva Filho Luiz Vicente Ribeiro Ferreira da2, Vergara Alberto Andrade3, Ribeiro Antônio Fernando4, Riedi Carlos Antônio5, Procianoy Elenara da Fonseca Andrade6, Adde Fabíola Villac1, Reis Francisco José Caldeira3, Ribeiro José Dirceu4, Torres Lídia Alice1, Fuccio Marcelo Bicalho de7, Epifanio Matias8, Firmida Mônica de Cássia9, Damaceno Neiva10, Ludwig-Neto Norberto11, Maróstica Paulo José Cauduro12, Rached Samia Zahi1, Melo Suzana Fonseca de Oliveira3, 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
Affiliation:
1. Universidade de São Paulo, Brazil 2. Universidade de São Paulo, Brazil; Hospital Albert Einstein, Brasil 3. Rede Fundação Hospitalar do Estado de Minas Gerais, Brasil 4. Universidade Estadual de Campinas, Brazil 5. Universidade Federal do Paraná, Brazil 6. Hospital de Clínicas de Porto Alegre, Brasil 7. Fundação Hospitalar do Estado de Minas Gerais, Brasil 8. Pontifícia Universidade Católica do Rio Grande do Sul, Brazil 9. Universidade do Estado do Rio de Janeiro, Brazil 10. Irmandade da Santa Casa de Misericórdia de São Paulo, Brasil 11. Hospital Infantil Joana de Gusmão, Brasil; Secretaria Estadual de Saúde de Santa Catarina, Brasil 12. Hospital de Clínicas de Porto Alegre, Brasil; Universidade Federal do Rio Grande do Sul, Brazil
Abstract
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.
Publisher
FapUNIFESP (SciELO)
Cited by
60 articles.
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