Association of lower airway inflammation with physiologic findings in young children with cystic fibrosis

• Published in: Pediatric pulmonology Vol. 44; no. 5; pp. 503 - 511
• Main Authors: Peterson-Carmichael, Stacey L., Harris, William T., Goel, Ruchika, Noah, Terry L., Johnson, Robin, Leigh, Margaret W., Davis, Stephanie D.
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.05.2009; Wiley-Liss
• Subjects: Biological and medical sciences; bronchoalveolar lavage; Bronchoalveolar Lavage Fluid - cytology; Bronchoalveolar Lavage Fluid - immunology; Bronchoalveolar Lavage Fluid - microbiology; bronchoscopy; Child, Preschool; Cohort Studies; Colony Count, Microbial; cystic fibrosis; Cystic Fibrosis - immunology; Errors of metabolism; Female; Forced Expiratory Flow Rates - immunology; forced expiratory flows; Functional Residual Capacity - immunology; General aspects; Humans; Infant; infant pulmonary function tests; Inflammation - immunology; Interleukin-8 - analysis; Leukocyte Count; Male; Medical sciences; Metabolic diseases; Metalloproteases - analysis; Miscellaneous hereditary metabolic disorders; Neutrophils - physiology; Plethysmography; Pneumology; raised volume technique; Transforming Growth Factor beta1 - analysis; Human; Bronchoscopy; forced expiratory flows; Pediatrics; Respiratory disease; raised volume technique; Exploration; Metabolic diseases; Cystic fibrosis; Infant; Inflammation; infant pulmonary function tests; Genetic disease; Association; Lung function; Bronchoalveolar lavage; Volume; Digestive diseases; Endoscopy; Technique; Child; Plethysmography; Pancreatic disease; Pneumology
• ISSN: 8755-6863; 1099-0496
• DOI: 10.1002/ppul.21044

Background The relationship between lower airway markers of inflammation and infection with physiologic findings is poorly understood in young children with cystic fibrosis (CF). The goal of this study was to evaluate the association of bronchoalveolar lavage fluid (BALF) markers of infection and inflammation, including mediators linked to airway remodeling, to infant lung function values in young children with CF undergoing clinically indicated bronchoscopy. Methods Plethysmography and the raised volume rapid thoracoabdominal compression (RVRTC) technique were performed in 16 sedated infants and young children with CF prior to bronchoscopy. BALF was collected and analyzed for pathogen density, cell count, % neutrophils, transforming growth factor beta 1 (TGF‐β1), matrix metalloproteinases (MMP), and interleukin‐8 (IL‐8). Results There was a significant direct correlation between functional residual capacity (FRC), the ratio of residual volume to total lung capacity (RV/TLC) and FRC/TLC with % neutrophils (P < 0.05). Forced expiratory flows were inversely correlated to % neutrophils (P < 0.01). Lung function parameters did not differentiate those with and without lower airway infection; however, pathogen density directly correlated with FRC and inversely correlated with flows (P < 0.05). In a subset of the population, MMP‐2 directly correlated with RV/TLC and inversely correlated with flows (P < 0.05) and TGF‐β1 directly correlated with FRC (P < 0.05). Conclusions Results from this study suggest that lower airway inflammation as well as mediators linked to airway remodeling play an active role in pulmonary deterioration in CF infants and young children undergoing clinically indicated bronchoscopy. Pediatr Pulmonol. 2009; 44:503–511. © 2009 Wiley‐Liss, Inc.