Spirometric caracterization of cystic fibrosis patients

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 FEV1 (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 multiethnic 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 FEV1 and FEV1/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.