Cystic fibrosis: an inherited susceptibility to bacterial respiratory infections

Cystic fibrosis is a severe monogenic disorder of ion transport in exocrine glands. The basic defect predisposes to chronic bacterial airway infection s with Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginos a and Burkholderia cepacia, The Pseudomonas infections in cystic fibrosis a re a paradigm of how versatile environmental bacteria can conquer, adapt an d persist in an atypical habitat and successfully evade defence mechanisms and chemotherapy in a susceptible host. Regular chemotherapy with aerosol a nd systemic antipseudomonal drugs has improved the course and prognosis of the disease, and research for effective vaccines is on the way.