Immune Dysfunction in Cystic Fibrosis

摘要: Absence of the cystic fibrosis transmembrane regulator (CFTR) function leads to chronic lung disease characterized by inflammation and persistent infections. The mechanisms for increased susceptibility respiratory tract infections in CF are most likely complex only partially understood. Most attention has been focused on effect defective expression CFTR epithelial cells submucosal gland was mostly thought be related abnormal chloride channel (Welsh MJ, 2011, Ratjen F 2003). However, numerous studies over past years have shown that absence affects immune system dysfunctional responses contribute pathological processes lung. In addition, it become increasingly evident dysfunction alone cannot completely explain pathology other pathways known regulated play a role dysregulation (Mehta A 2008). This chapter reviews both soluble factors milieu modify cell specific alterations cellular components innate adaptive impaired defense disease.