Anaesthesia and cystic fibrosis.

Cystic fibrosis occurs in 1 in 2000 children and the majority now reach adulthood. The disease is a complex multisystem disorder which is likely to challenge anaesthetists with increasing frequency. In this review the presentation and genetics of the disease are briefly described, followed by a detailed account of the pathophysiology relevant to anaesthesia. The pre-operative assessment and conduct of anaesthesia are discussed and some suggestions made regarding management.