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Brazilian guidelines for the diagnosis and treatment of cystic fibrosis

Overview of attention for article published in Jornal de Pneumologia, June 2017
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Title
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis
Published in
Jornal de Pneumologia, June 2017
DOI 10.1590/s1806-37562017000000065
Pubmed ID
Authors

Rodrigo Abensur Athanazio, Luiz Vicente Ribeiro Ferreira da Silva Filho, Alberto Andrade Vergara, Antônio Fernando Ribeiro, Carlos Antônio Riedi, Elenara da Fonseca Andrade Procianoy, Fabíola Villac Adde, Francisco José Caldeira Reis, José Dirceu Ribeiro, Lídia Alice Torres, Marcelo Bicalho de Fuccio, Matias Epifanio, Mônica de Cássia Firmida, Neiva Damaceno, Norberto Ludwig-Neto, Paulo José Cauduro Maróstica, Samia Zahi Rached, Suzana Fonseca de Oliveira Melo, Leonardo Araújo Pinto, Luciana Freitas Velloso Monte, Laurinda Yoko Shinzato Higa, Tania Wrobel Folescu, Fernando Augusto de Lima Marson, Isabela Sad, Maria de Fátima Correa Pimenta Servidoni, Paulo Kussek, Salmo Raskin, Adriana Della Zuana, Albin Augustin, Anneliese Hoffmann, Beatriz Barbisan, Bruno Hochhegger, Carlos Emilio Levy, Claudine Sarmento da Veiga, Claudio Ricachinevsky, Concetta Esposito, Dante Escuissato, Diego Brandemburgo, Elisabeth Marques, Evanirso de Aquino, Gilberto Bueno Fischer, Joaquim Carlos Rodrigues, Leticia Machado, Lucia Muramato, Lusmaia Damasceno Camargo Costa, Marcio Donadio, Marcos César Santos de Castro, Maria Angela Ribeiro, Maria Angélica Santana, Mariane Canan, Marina Buarque de Almeida, Murilo Britto, Paulo Roth Tarso Dalcin, Regina Terse Trindade Ramos, Sonia Chiba, Valéria de Carvalho Martins, Claudine Lacerda, Eliana Barbosa, Elizabet Vilar Guimarães, Gabriel Hessel, Jocemara Gurmini, Lenycia Neri, Marcelo Coelho Nogueira, Mônica Chang Wayhs, Miriam Isabel Santos Simon, Arlene Gonçalves dos Santos Fernandes, Claudia de Castro de Silva, Cristiano Túlio Maciel Albuquerque, Edna Lúcia Souza, Fernando Antonio de Abreu e Silva, Paulo de Tarso Dalcin, Renata Maria de Noronha, Ricardo Teixeira, Sandra Helena Machado, Spencer Marcantonio Camargo, Tatiana Rozov, Ticiana da Costa Rodrigues

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. RESUMO A fibrose cística (FC) é uma doença genética autossômica recessiva caracterizada pela disfunção do gene CFTR. Trata-se de uma doença multissistêmica que ocorre mais frequentemente em populações descendentes de caucasianos. Nas últimas décadas, diversos avanços no diagnóstico e tratamento da FC mudaram drasticamente o cenário dessa doença, com aumento expressivo da sobrevida e qualidade de vida. Atualmente, o Brasil dispõe de um programa de ampla cobertura para a triagem neonatal de FC e centros de referência distribuídos na maior parte desses estados para seguimento dos indivíduos. Antigamente confinada à faixa etária pediátrica, tem-se observado um aumento de pacientes adultos com FC tanto pelo maior número de diagnósticos de formas atípicas, de expressão fenotípica mais leve, assim como pelo aumento da expectativa de vida com os novos tratamentos. Entretanto, ainda se observa uma grande heterogeneidade no acesso aos métodos diagnósticos e terapêuticos para FC entre as diferentes regiões brasileiras. O objetivo dessas diretrizes foi reunir as principais evidências científicas que norteiam o manejo desses pacientes. Um grupo de 18 especialistas em FC elaborou 82 perguntas clínicas relevantes que foram divididas em cinco categorias: características de um centro de referência; diagnóstico; tratamento da doença respiratória; tratamento gastrointestinal e nutricional; e outros aspectos. Diversos profissionais brasileiros atuantes na área da FC foram convidados a responder as perguntas formuladas pelos coordenadores. A literatura disponível foi pesquisada na base de dados PubMed com palavras-chave, buscando-se as melhores respostas às perguntas dos autores.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 342 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 342 100%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 62 18%
Student > Master 31 9%
Student > Doctoral Student 20 6%
Other 18 5%
Student > Postgraduate 18 5%
Other 58 17%
Unknown 135 39%
Readers by discipline Count As %
Medicine and Dentistry 77 23%
Nursing and Health Professions 42 12%
Biochemistry, Genetics and Molecular Biology 13 4%
Sports and Recreations 12 4%
Agricultural and Biological Sciences 9 3%
Other 39 11%
Unknown 150 44%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 3. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 18 August 2017.
All research outputs
#15,151,839
of 25,461,852 outputs
Outputs from Jornal de Pneumologia
#236
of 719 outputs
Outputs of similar age
#171,723
of 330,714 outputs
Outputs of similar age from Jornal de Pneumologia
#1
of 1 outputs
Altmetric has tracked 25,461,852 research outputs across all sources so far. This one is in the 40th percentile – i.e., 40% of other outputs scored the same or lower than it.
So far Altmetric has tracked 719 research outputs from this source. They receive a mean Attention Score of 3.7. This one has gotten more attention than average, scoring higher than 67% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 330,714 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 47th percentile – i.e., 47% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 1 others from the same source and published within six weeks on either side of this one. This one has scored higher than all of them