| Autor: |
Abensur Athanazio, Rodrigo, Ribeiro Ferreira da Silva Filho, Luiz Vicente, Andrade Vergara, Alberto, Fernando Ribeiro, Antônio, Riedi, Carlos Antônio, Andrade Procianoy, Elenara da Fonseca, Villac Adde, Fabíola, Caldeira Reis, Francisco José, Dirceu Ribeiro, José, Torres, Lídia Alice, Bicalho de Fuccio, Marcelo, Epifanio, Matias, de Cássia Firmida, Mônica, Damaceno, Neiva, Ludwig-Neto, Norberto, Cauduro Maróstica, Paulo José, Rached, Samia Zahi, de Oliveira Melo, Suzana Fonseca |
| Předmět: |
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| Zdroj: |
Brazilian Journal of Pulmonology / Jornal Brasileiro de Pneumologia; mai/jun2017, Vol. 43 Issue 3, p219-245, 27p |
| Abstrakt: |
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 cur rent 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 ive 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. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Supplemental Index |
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