Donald R VanDevanter1, David J Pasta. 1. Case Western Reserve University School of Medicine, Cleveland, OH, United States. Electronic address: enigmaster@comcast.net.
Abstract
BACKGROUND: Many infants with cystic fibrosis (CF) exhibit airway inflammation, gas trapping, bronchiectasis, and/or reduced flow, but by age 6 years have forced vital capacities (FVC) and expiratory volumes in 1 second (FEV1) within the variability range of the normal population. We sought evidence of diminished FVC and FEV1 in 6-year-olds with CF. METHODS: GLI 2012 FVC and FEV1 Z-scores for 6-year-olds from the European CF Patient Registry were plotted against theoretical values from the Normal distribution. RESULTS: Mean FVC and FEV1 Z-scores for 681 patients (322 females) were -0.43 (SD=1.41) and -0.65 (1.40). Z-scores were consistently lower than expected for the normative population by quantile-quantile plot. CONCLUSIONS: Diminished FEV1, and to a lesser extent FVC, is found in a large majority of this population, consistent with an established body of evidence that loss of lung function begins early in life for most, if not all, children with CF.
BACKGROUND: Many infants with cystic fibrosis (CF) exhibit airway inflammation, gas trapping, bronchiectasis, and/or reduced flow, but by age 6 years have forced vital capacities (FVC) and expiratory volumes in 1 second (FEV1) within the variability range of the normal population. We sought evidence of diminished FVC and FEV1 in 6-year-olds with CF. METHODS: GLI 2012 FVC and FEV1 Z-scores for 6-year-olds from the European CF Patient Registry were plotted against theoretical values from the Normal distribution. RESULTS: Mean FVC and FEV1 Z-scores for 681 patients (322 females) were -0.43 (SD=1.41) and -0.65 (1.40). Z-scores were consistently lower than expected for the normative population by quantile-quantile plot. CONCLUSIONS: Diminished FEV1, and to a lesser extent FVC, is found in a large majority of this population, consistent with an established body of evidence that loss of lung function begins early in life for most, if not all, children with CF.
Authors: Andrea Hahn; Aszia Burrell; Emily Ansusinha; Diane Peng; Hollis Chaney; Iman Sami; Geovanny F Perez; Anastassios C Koumbourlis; Robert McCarter; Robert J Freishtat; Keith A Crandall; Edith T Zemanick Journal: Heliyon Date: 2020-06-01