부산대학교 도서관

Aim: Cystic fibrosis (CF) is an autosomal recessive disorder. Although it is considered as an epithelial disease due to impaired chloride transport, its pathogenesis remains unclear. CF is classified as a syndrome with congenital defects of phagocyte in recent human inborn errors of immunity phenotypic classifications. Neutrophils are the most effective cells in the eradication of bacterial infections such as Pseudomonas aeruginosa. The aim of the present study was to investigate the phagocyte functions in pseudomonas colonized CF patients. Materials and Methods: A total number of 26 pseudomonas colonized CF patients and 21 healthy controls (gender and age matched) were included in this study. Absolute neutrophil counts (ANC), immunoglobulin values (Ig), the Migratest to evaluate chemotaxis in neutrophils and monocytes, CD11A/CD18/CD15 S (b2 integrin) adhesion molecules, and the Phagoburst test for intracellular bacterial killing were analyzed by flow cytometer. Results: ANC, CD15S expression on neutrophils and IgG, IgA and IgM levels were higher in the CF patients than the control group (p<0.01). The neutrophils oxidative burst activity and the chemotactic ability of the CF patients did not differ from those of the controls. Patients with allergic bronchopulmonary aspergillosis and those with a mutation of 2183AA>G had significantly lower chemotaxis indexes than the others (p=0.01, p=0.01 respectively). Conclusion: Our results from a small group of patients does not support impaired functions such as migration and phagocytosis of neutrophils in patients with CF. Further studies involving more CF patients are needed to make a definitive interpretation. [ABSTRACT FROM AUTHOR]