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Lung function deterioration in school children with cystic fibrosis
Author(s) -
WalickaSerzysko Katarzyna,
Postek Magdalena,
Milczewska Justyna,
Sands Dorota
Publication year - 2020
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.25013
Subject(s) - medicine , functional residual capacity , plethysmograph , nitrogen washout , airway resistance , lung volumes , pulmonary function testing , airway obstruction , spirometry , cystic fibrosis , ventilation (architecture) , cardiology , lung , asthma , air trapping , obstructive lung disease , respiratory disease , airway , copd , anesthesia , mechanical engineering , engineering
Abstract Introduction Lung disease in cystic fibrosis (CF) begins early in life but the capabilities for detecting abnormalities of pulmonary dysfunction in children remain limited. Objective The study aimed to evaluate the early progression of lung function by the analysis of pulmonary hyperinflation, ventilation inhomogeneity (VI), trapped gas and airway obstruction with age. Methods One hundred CF children aged 7 to 18, divided into two groups aged 7 to 12 (n = 40) and 13 to 18 (n = 60), were enrolled. Patients performed multiple‐breath nitrogen washout (MBNW) tests and plethysmography for measurements of lung clearance index (LCI), functional residual capacity (FRC pleth , FRC MBNW ), volume of trapped gas (V T ), total resistance, and effective and specific effective airway resistance (R eff , sR eff ). Results We obtained a positive correlation of FRC pleth , FRC MBNW , and LCI with age. A linear correlation between FRC MBNW and FRC pleth ( P < .0001) was observed. VI was higher in the group of older patients (9.79 in the group aged 7‐12 and 11.67 in the group aged 13‐18). An increased effective specific airway resistance >2 ( z ‐score) was present in 58% of all subjects (50% and 63.3%, respectively). Pulmonary hyperinflation (FRC pleth >2 z ‐score) was observed in 33% of all patients: 25% and 36.6%, respectively. Trapped gas (V T > 2 z ‐score) was present in 18% of all children: 30% and 10%, respectively. Conclusion A gradual decline in lung function is associated with an increase in VI, airway obstruction, pulmonary hyperinflation and development of trapped gas. In children who cannot perform either spirometry or plethysmography, MBNW can deliver a measurement of LCI connecting with VI as well as FRC MBNW to indicate indirectly the increase of hyperinflation.