Anaesthesia for adults with XXXX .

@entity1 @entity6 @entity1576 @entity74 @entity354 @entity1577
@entity1577 is an @entity74 which affects one in 2500 live births. It is a multisystem disease and has a variety of presentations. The major clinical features affect the gastrointestinal and respiratory tracts. Severe @entity1576 , @entity6 and @entity354 are common features of concern to anaesthetists. Improved care of young @entity1 has allowed many to survive into adulthood. Lung transplantation has significantly improved the outlook for many @entity1 . At Alfred Hospital, 74 @entity1 with @entity1577 underwent 149 procedures from January 1978 to January 1994, with a mortality of 0.6% (95% CI 0.4%-0.8%). This retrospective cohort study describes the anaesthetic management and perioperative care of these @entity1 . Most of the anaesthetics were for procedures related to @entity1577 but 12% were for unrelated conditions. @entity1577 related procedures include diagnostic, venous access, enteral feeding procedures, treatment of complications of @entity1577 and lung transplantation. Despite extremely poor respiratory function, these @entity1 can be managed with acceptably low postoperative mortality (1%). Pre- and postoperative care must be directed towards optimal clearance of viscous respiratory secretions. Procedures need to be planned so that optimal care can be given by each member of the team caring for @entity1577 @entity1 .
