5 documents found, page 1 of 1
Effectiveness of a multistep Pseudomonas aeruginosa eradication treatment proto...
Riquena,Barbara; Silva Filho,Luiz Vicente Ribeiro Ferreira da; Nakaie,Cleyde Myriam Aversa; Almeida,Marina Buarque de; Rodrigues,Joaquim CarlosABSTRACT Objective: Although various strategies have been proposed for eradicating Pseudomonas aeruginosa in patients with cystic fibrosis (CF), only a few employ multistep treatment in children colonized by that pathogen for the first time. The aim of this study was to describe the effectiveness of a three-phase eradication protocol, initiated after the first isolation of P. aeruginosa, in children with CF in ...
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 ...
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...
Discinesia ciliar primária: quando o pediatra deve suspeitar e como diagnosticar?
Olm,Mary Anne K.; Adde,Fabíola Villac; Silva Filho,Luiz Vicente F. da; Rodrigues,Joaquim CarlosOBJETIVO: Revisar a discinesia ciliar primária (DCP) quanto aos seus aspectos ultra-estruturais, discriminar os defeitos ciliares primários dos secundários, descrever o quadro clínico, os testes laboratoriais de triagem e de diagnóstico disponíveis, bem como seu manejo clínico. FONTE DE DADOS: Pesquisa nas bases de dados Medline, Lilacs e SciELO, no período de 1980 a 2007. SÍNTESE DOS DADOS: A DCP é uma doença ...
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...