Abstract: Background: Cystic fibrosis (CF) lung disease is characterized by an infiltration of activated neutrophils. Oxidative radical formation and neutrophil proteases may interact with the components of CF airway secretions. This study examined the soluble factors in CF airway secretions (sol) and determined their effect on the production of superoxide radicals. Methods: Neutrophils were isolated from three normal subjects. Each preparation of neutrophils can be used in several experiments on the same day of preparation. Using a standardized cytochrome c reduction method, the effect of pooled CF sol isolated from nine CF patients, on neutrophil superoxide production was studied. Results: CF sol phase significantly attenuates the production of superoxide by phorbol stimulated control neutrophils in a time and concentration dependent manner. When CF sol is boiled, the inhibitory effect on superoxide was reversed. CF soluble heat labile factors clearly attenuated superoxide production, independent of elastase concentration. We conclude that the presence of heat labile soluble factors in CF sputum, serve to attenuate superoxide production by human neutrophils

Cystic fibrosis (CF) is a genetic disorder resulting in a chloride channel (CFTR) defect characterized by multi-organ damage. The primary cause of morbidity and mortality is end-stage obstructive lung disease. Lung transplantation is a treatment option, but is complicated by the risks of acute rejection, Bronchiolitis Obliterans syndrome (BOS) (graft dysfunction), and serious infection. This study sought to assess survival free from three major complications, namely BOS, acute rejection, and serious infection and also to compare overall survival among school-age CF transplantation recipients to non-CF recipients. We limited consideration to school-age children because they comprise a unique cohort in terms of linear and graft growth, immunity, pharmacokinetics and infection exposure as compared with infants, adolescents and adults. The OPTN national database was searched for period between January 1997 and August 2006 for children between 6 and 10 years of age undergoing lung transplantation. Children with CF were compared with non-CF recipients with regard to occurrence of BOS, infection-related hospitalizations, and acute rejections. Kaplan-Meier analyses were used for statistical comparisons of the two cohorts. There were 50 CF patients and 37 non-CF patients available for analysis from the OPTN database. Up to 5 years post-transplant, there were no statistically significant differences between CF and non-CF patients in overall survival, and survival free from BOS, acute rejections, or serious infections defined as those requiring hospitalization. Despite having an underlying systemic disease based on defective CFTR, CF school-age children receiving a lung transplant do not demonstrate more major complications or lower survival than non-CF children.