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NO pathway in CF and non‐CF children
Author(s) -
Wooldridge Jamie L.,
Deutsch Gail H.,
Sontag Marci K.,
Osberg Iris,
Chase Daniel R.,
Silkoff Philip E.,
Wagener Jeffery S.,
Abman Steven H.,
Accurso Frank J.
Publication year - 2004
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.10455
Subject(s) - medicine , cystic fibrosis , airway , bronchoalveolar lavage , exhaled nitric oxide , nitric oxide synthase , immunostaining , respiratory epithelium , inflammation , lung , nitric oxide , immunology , respiratory disease , pathology , bronchoscopy , immunohistochemistry , anesthesia , systemic inflammation
Controversy exists concerning abnormalities of the nitric oxide (NO) pathway in cystic fibrosis (CF) lung disease. Although some studies suggested that NO activity is impaired in CF, changes in NO production in young children have not been studied. We hypothesized that nitric oxide synthase (NOS II) expression is decreased in young children with CF, leading to decreased production of lower airway NO, and that decreased NOS II expression is related to airway inflammation. Accordingly, we measured lower airway exhaled NO, nitrate, and NOS II expression in airway epithelium and macrophages by bronchoscopy, bronchoalveolar lavage (BAL), and bronchial brushing in 13 children with CF, 4 adolescent patients with CF, and 14 disease control children. Lower airway NO and nitrate were not different between CF and disease controls. Immunostaining studies of NOS II expression in airway epithelial cells and macrophages were similar in CF and control patients. Within the CF group, however, expression of NOS II was inversely related to BAL neutrophil counts and IL‐8, two markers of airway inflammation. We conclude that lower airway NO, nitrate levels, and NOS II expression are not different in young children with CF and disease control patients, but that NOS II expression decreases in CF as airway inflammation increases. Pediatr Pulmonol. 2004; 37:338–350. © 2004 Wiely‐Liss, Inc.