BACKGROUND: @entity1577 is no longer a @entity221 of childhood and mean survival is now over 30 years. Adult @entity1 with atypical @entity1577 are increasingly being diagnosed. In Israel, all @entity1 are still followed in pediatric centers. OBJECTIVES: To describe our experience with adult @entity1577 , stressing the importance of adult-related health and psychosocial issues. METHODS: Twenty-five @entity1577 @entity1 aged 20-50 years, constituting 44% of the 57 @entity1 followed at our center, were analyzed for pulmonary and extrapulmonary features and management. RESULTS: Nineteen were diagnosed as @entity1 and 6 as adults. Nineteen were @entity3210 and 6 were pancreatic-sufficient, including 5 diagnosed as adults. Pulmonary status was usually stable, with forced expiratory volume in 1 second 66.3 +/- 21% (mean +/- SD) and no difference between @entity3210 @entity1 . The latter had more hemoptysis, @entity2782 , @entity409 and @entity6 . Two @entity1 died of @entity5 and two of advanced @entity358 . A majority received continuous inhaled and oral antibiotics, bronchodilators, Dnase, physiotherapy and periodic home intravenous antibiotics. Psychosocial functioning was excellent: 60% were employed, 36% were married and 40% had @entity1 (none with @entity1577 ). @entity1 diagnosed as adults had mild @entity66 or isolated severe @entity358 . CONCLUSIONS: @entity1577 adults generally have a good quality of life. Advances in understanding the @entity1577 and a plethora of new treatment modalities bode well for the future. @entity1 must be maintained in optimal condition to reap the benefits, and there is an urgent necessity for adult physicians to develop expertise in @entity1577 .

XXXX in adults: a changing scene.

@entity1 :: ('9606', 'Species') :: ['children', 'Patients', 'patients'] @entity2782 :: ('MESH:D011552', 'Disease') :: ['Pseudomonas infection'] @entity66 :: ('MESH:D004194', 'Disease') :: ['multisystem disease'] @entity6 :: ('MESH:D003920', 'Disease') :: ['diabetes'] @entity5 :: ('MESH:D009369', 'Disease') :: ['malignancy'] @entity409 :: ('MESH:D008107', 'Disease') :: ['intestinal obstruction, liver disease'] @entity3210 :: ('MESH:D010188', 'Disease') :: ['pancreatic-sufficient and insufficient', 'pancreatic-insufficient'] @entity358 :: ('MESH:D008171', 'Disease') :: ['lung disease'] @entity221 :: ('MESH:D005334', 'Disease') :: ['terminal illness'] @entity1577 :: ('MESH:D003550', 'Disease') :: ['Cystic fibrosis', 'CF', 'CF defect']
@entity1577 :: (MESH:D003550,Disease) :: ['Cystic fibrosis']
