Immunologic aspects of cystic fibrosis. pp. 195-217.

Because of the prominent role of infection in cystic fibrosis, and because 
of the genetic nature of both cystic fibrosis and specific immune 
defects, considerable effort has been expended in determining if there are 
hereditary immune defects in cystic fibrosis patients that render them susceptible 
to infection. Against a congenital immune defect is the fact that 
the infections nearly always are localized to the lung with the 
same two organisms, rather than infections at multiple sites involving multiple 
organisms. Specific immune mechanisms in the B and T cell systems 
are examined. Nonspecific host defense mechanisms include the complement system, phagocytosis, 
pulmonary clearance, the pulmonary barrier to the systemic spread of infection, 
protease inhibitors, and factor XIII-dependent systems. Hypersensitivity states include allergy and 
atopy, allergic aspergillosis, immune complex disease, and hypersensitivity to pancreatic extracts. 
The presence or absence of specific antigen components and the association 
of HL-A antigens are also discussed. 