La mucoviscidose et la réanimation adulte

Cystic fibrosis (CF) is a common life-shortening genetic disorder among Caucasians leading to respiratory, pancreatic, and gastro-intestinal disorders. Although the disease remains incurable, advances in CF chronic disease management resulted in increasing the median survival age. In 2003 almost 40% of CF patients were older than 18, corresponding to an adult population with CF of 2,200 individuals in France. As severity of CF pulmonary disease usually increases with age, adults are a group at higher risk for acute complications that are more likely to be life threatening and to request ICU hospitalization. This review provides a summary of the pathophysiology, clinical epidemiology and treatment of severe complications such as pulmonary exacerbations, hemoptysis, distal intestinal obstruction syndrome and severe dehydration.