Caracterización espirométrica de pacientes con fibrosis quística

Abstract

Introduction: The prognosis of patients with cystic fibrosis (CF) has remarkably improved. The assessment of the disease progression is based on the measurement of the FEV 1 (Forced Expiratory Volume in one second). Objectives: 1. To describe forced expiratory flows and volumes and compare their interpretation according to different reference standards (Knudson, Gutiérrez, and multi-ethnic GLI); 2. To describe bronchodilator response. Patients and Method: The medical records and spirometries of all patients with CF controlled at the Dr. Sotero del Rio Hospital were reviewed. Demographic background, sweat test results, genetic study , and bacteriological study were obtained. In addition, Forced Vital Capacity (FVC) was recorded as well as FEV 1 and FEV 1 /FVC ratio. Results: Data from 14 patients, were analyzed, seven males, aged 6-24 years, median 15 years, median BMI 18.15 (range 14.6-23.3), median sweat chloride test 76 mEq/l (range 50,2-119 mEq/l), seven patients with at least one F508del mutation. Using multi-ethnic and Gutierrez predictive formulas, lung function involvement occurred previously in relation to the use of Knudson equations. None of the patients had a significant bronchodilator response. Conclusion: The group of patients described mostly presents functional respiratory involvement and had no bronchodilator response. The interpretation of functional respiratory involvement varies according to the theoretical values used.