Cystic fibrosis, pulmonary infection and host response

Cystic fibrosis is a multisystem disorder affecting ductal systems lined by epithelial cells. Consequently, the disease affects the lungs, gastrointestinal tract, pancreas, hepatobiliary tree, endocrine and sweat glands and the reproductive system. However, mortality and morbidity are dominated by the relentless progression of pulmonary infection with current median survival only into the third decade of life. The management of pulmonary disease is directed at the two main bacterial pathogens Pseudomonas aeruginosa and Pseudomonas cepacia and the accompanying complex, self-damaging inflammatory host responses to both these organisms. This article will focus on these crucial issues of which greater understanding and improved treatment will improve survival for cystic fibrosis patients.