title: Cartilaginous airway wall dimensions and airway resistance in cystic fibrosis lungs.
abstract: It is not clear how airway pathology relates to the severity of airflow obstruction and increased bronchial responsiveness in cystic fibrosis (CF) patients. The aim of this study was to measure the airway dimensions of CF patients and to estimate the importance of these dimensions to airway resistance using a computational model. Airway dimensions were measured in lungs obtained from CF patients who had undergone lung transplantation (n=12), lobectomy (n=1), or autopsy (n=4). These dimensions were compared to those of airways from lobectomy specimens from 72 patients with various degrees of chronic obstructive pulmonary disease (COPD). The airway dimensions of the CF and COPD patients were introduced into a computational model to study their effect on airway resistance. The inner wall and smooth muscle areas of peripheral CF airways were increased 3.3- and 4.3-fold respectively compared to those of COPD airways. The epithelium was 53% greater in height in peripheral CF airways. The sensitivity and maximal plateau resistance of the computed dose/response curves were substantially increased in the CF patients compared to COPD patients. The changes in airway dimensions of cystic fibrosis patients probably contribute to the severe airflow obstruction, and to increased bronchial responsiveness, in these patients.
authors.affiliation: Dept of Pediatrics, Sophia Children's Hospital, Erasmus Medical Centre Rotterdam, The Netherlands.
authors.forename: H A
authors.initials: HA
authors.lastname: Tiddens
authors.forename: L P
authors.initials: LP
authors.lastname: Koopman
authors.forename: R K
authors.initials: RK
authors.lastname: Lambert
authors.forename: W M
authors.initials: WM
authors.lastname: Elliott
authors.forename: W C
authors.initials: WC
authors.lastname: Hop
authors.forename: T W
authors.initials: TW
authors.lastname: van der Mark
authors.forename: W J
authors.initials: WJ
authors.lastname: de Boer
authors.forename: J C
authors.initials: JC
authors.lastname: de Jongste
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000402:Airway Obstruction / Q000473:pathology; D000403:Airway Resistance; D016535:Bronchial Hyperreactivity / Q000503:physiopathology*; D016085:Bronchoconstrictor Agents / Q000494:pharmacology; D002356:Cartilage / Q000473:pathology* / Q000502:physiology; D002648:Child; D046508:Culture Techniques; D003550:Cystic Fibrosis / Q000473:pathology*; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007150:Immunohistochemistry; D008168:Lung / Q000187:drug effects / Q000473:pathology*; D008173:Lung Diseases, Obstructive / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009130:Muscle, Smooth / Q000473:pathology; D012129:Respiratory Function Tests; D012132:Respiratory Muscles / Q000503:physiopathology*; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index
chemical_list: D016085:Bronchoconstrictor Agents