6 documents found, page 1 of 1
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis
Athanazio,Rodrigo Abensur; Silva Filho,Luiz Vicente Ribeiro Ferreira da; Vergara,Alberto Andrade; Ribeiro,Antônio Fernando; Riedi,Carlos AntônioABSTRACT 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 ...
Effects of Pilates mat exercises on muscle strength and on pulmonary function i...
Franco,Caroline Buarque; Ribeiro,Antonio Fernando; Morcillo,André Moreno; Zambon,Mariana Porto; Almeida,Marina Buarque; Rozov,TatianaOBJECTIVE: To analyze the effects of Pilates mat exercises in patients with cystic fibrosis (CF). METHODS: This was a clinical trial involving 19 CF patients recruited from either the CF Outpatient Clinic of the State University at Campinas Hospital de Clínicas or the Children's Institute of the University of São Paulo School of Medicine Hospital das Clínicas. All of the patients performed Pilates mat exercises...
A first-year dornase alfa treatment impact on clinical parameters of patients w...
Rozov,Tatiana; Silva,Fernando Antônio A. e; Santana,Maria Angélica; Adde,Fabíola Villac; Mendes,Rita HeloisaOBJECTIVE: To describe the clinical impact of the first year treatment with dornase alfa, according to age groups, in a cohort of Brazilian Cystic Fibrosis (CF) patients. METHODS: The data on 152 eligible patients, from 16 CF reference centers, that answered the medical questionnaires and performed laboratory tests at baseline (T0), and at six (T2) and 12 (T4) months after dornase alfa initiation, were analyzed...
Validação lingüística dos questionários de qualidade de vida em fibrose cística
Rozov,Tatiana; Cunha,Maristela T.; Nascimento,Oliver; Quittner,Alexandra L.; Jardim,José R.OBJETIVO: O propósito deste estudo foi validar em português as quatro versões de questionários de qualidade de vida em fibrose cística, desenvolvidos para pacientes com fibrose cística de 6 a 11 anos, de 12 a 13 e mais de 14 anos, e para os pais de pacientes de 6 a 13 anos. MATERIAL E MÉTODOS: A validação das quatro versões de questionários de qualidade de vida em fibrose cística (de 35 e 50 questões, abrangend...
Displasia broncopulmonar
Monte,Luciana F. Velloso; Silva Filho,Luiz Vicente F. da; Miyoshi,Milton Harumi; Rozov,TatianaOBJETIVO: Apresentar uma ampla revisão da literatura sobre displasia broncopulmonar, abordando novas definições, fisiopatologia, prevenção, tratamento, prognóstico e evolução. FONTE DOS DADOS: Foram selecionados os artigos mais relevantes sobre o tema, desde a sua descrição inicial, em 1967, pesquisados na MEDLINE. SÍNTESE DOS DADOS: A displasia broncopulmonar é considerada uma das principais causas de doença p...
Cystic fibrosis with normal sweat chloride concentration: case report
Silva Filho,Luiz Vicente Ferreira da; Bussamra,Maria Helena de Carvalho Ferreira; Nakaie,Cleyde Miriam Aversa; Adde,Fabíola VillacCystic fibrosis is a genetic disease usually diagnosed by abnormal sweat testing. We report a case of an 18-year-old female with bronchiectasis, chronic P. aeruginosa infection, and normal sweat chloride concentrations who experienced rapid decrease of lung function and clinical deterioration despite treatment. Given the high suspicion ofcystic fibrosis, broad genotyping testing was performed, showing a compoun...