Results: 20

Pseudomonas aeruginosa colonization in the upper and lower airways of a child with cystic fibrosis: a father's meticulous approach to successful eradication

J. bras. pneumol; 45 (6), 2019

Progresos en farmacoterapia en fibrosis quística

Cystic fibrosis is an autosomal recessive multisystemic disease caused by a mutation in the gene encoding the CFTR protein (cystic fibrosis transmembrane conductance regulator). For decades treatments were focused on pulmonary and extrapulmonary symptoms, but in recent years new treatments based on genet...

Medications used in pediatric cystic fibrosis population

Einstein (Säo Paulo); 16 (4), 2018
ABSTRACT Objective To describe the drug utilization profile used by pediatric cystic fibrosis patients. Methods A transversal study comprising the analysis of records and interviews with caregivers of pediatric patient in a reference center of Southern Brazil. We collected information about patients'...

Variants in the interleukin 8 gene and the response to inhaled bronchodilators in cystic fibrosis

J. pediatr. (Rio J.); 93 (6), 2017
Abstract Objective: Interleukin 8 protein promotes inflammatory responses, even in airways. The presence of interleukin 8 gene variants causes altered inflammatory responses and possibly varied responses to inhaled bronchodilators. Thus, this study analyzed the interleukin 8 variants (rs4073, rs2227306,...

Hearing thresholds at high frequency in patients with cystic fibrosis: a systematic review

Abstract Introduction: High-frequency audiometry may contribute to the early detection of hearing loss caused by ototoxic medications. Many ototoxic drugs are widely used in the treatment of patients with cystic fibrosis. Early detection of hearing loss should allow known harmful drugs to be identified ...

Discrepâncias entre os registros de prontuários acerca da farmacoterapia de pacientes pediátricos com fibrose cística

Clin. biomed. res; 37 (3), 2017
Introdução: Discrepâncias nos registros eletrônicos de medicamentos são frequentes e podem levar a erros relacionados a medicamentos. O objetivo deste estudo foi analisar as discrepâncias entre os registros médicos e farmacêuticos da terapia medicamentosa de pacientes com fibrose cística de um a...

Informe de evaluación científica basada en la evidencia disponible: fibrosis quística
Scientific evaluation report based on available evidence: cystic fibrosis

INTRODUCCIÓN: La fibrosis quística (FQ) es la enfermedad autosómica recesiva más frecuente entre las poblaciones caucásicas, con una frecuencia de 1 en 2000 a 3000 nacidos vivos. Esta patología es producida por la mutación del gen que codifica la proteína reguladora de la conductancia transmembra...

Clinical significance, antimicrobial susceptibility and molecular identification of Nocardia species isolated from children with cystic fibrosis

Braz. j. microbiol; 47 (3), 2016
ABSTRACT Nocardia is an opportunistic pathogen that causes respiratory infections in immunocompromised patients. The aim of this study was to analyze the epidemiology, clinical significance and antimicrobial susceptibility of Nocardia species isolated from eight children with cystic fibrosis. The isolate...

Moduladores mejoran la función pulmonar en la mutación más frecuente de la fibrosis quística

Rev. pediatr. electrón; 12 (4), 2015
Un tópico de análisis crítico es un resumen estandarizado que se organiza en torno a una pregunta clínica estructurada, realiza una revisión crítica y resalta la relevancia de sus resultados aplicados a nuestra realidad. El estudio analizado evalúa en 1100 pacientes de 12 años o más portadores d...

Informe de evaluación rápida de tecnología sobre seguridad y efectividad de la alfadornasa para fibrosis quística
Rapid evaluation report of technology on safety and effectiveness of alfadornase for cystic fibrosis

La fibrosis quística es una enfermedad hereditaria letal, más frecuente en raza blanca. Se transmite de manera autosómica recesiva, de tal modo que una pareja de portadores tiene la probabilidad de un 25% de un hijo con fibrosis quística en cada embarazo y que cada hijo sano tiene 2/3 de probabilidad...