An update on cystic fibrosis and implications for anesthesia.

Cystic fibrosis is a recessive inherited disorder that affects about 30,000 Americans. The majority of infants born with cystic fibrosis now reach adulthood, making surgical procedures performed in this patient group more common. The disease is a heterogeneous disorder caused by widespread dysfunction of exocrine glands and involving salivary, sweat, digestive, and pulmonary secretions. The broad, variable, and sometimes confusing array of clinical manifestations of cystic fibrosis include obstructive pulmonary disease, pancreatic insufficiency, abnormally high sweat electrolyte concentrations, nasal polyps, infertility, and pansinusitis. In this article, the epidemiology, genetics, and pathophysiology of the disease are outlined briefly. Preoperative assessment is reviewed, and guidelines are submitted regarding anesthetic management.