title: Orthotopic liver transplantation in patients with cystic fibrosis.
abstract: An 11-year-old boy who had cystic fibrosis underwent an orthotopic liver transplantation. His immediate postoperative course was not unusually complicated when compared with other liver transplant recipients. Transplantation did not correct abnormalities in the sweat test or the respiratory disease. Cholestasis due to obstruction of the recipient duct with tenacious bile was cleared by instilling N-acetylcysteine into the duct. On the 48th day after the transplantation, he died of an intraventricular and intracerebral hemorrhage caused by an Aspergillus brain abscess. We conclude that certain patients with cystic fibrosis may be appropriate candidates for liver transplantation, but their pre- and post-operative management may need to differ from other liver transplant recipients.
authors.affiliation: Department of Pediatrics, University of California, Davis Medical Center, Sacramento 95817.
authors.forename: K L
authors.initials: KL
authors.lastname: Cox
authors.forename: R E
authors.initials: RE
authors.lastname: Ward
authors.forename: T L
authors.initials: TL
authors.lastname: Furgiuele
authors.forename: R A
authors.initials: RA
authors.lastname: Cannon
authors.forename: K D
authors.initials: KD
authors.lastname: Sanders
authors.forename: G
authors.initials: G
authors.lastname: Kurland
mesh_terms: D001228:Aspergillosis / Q000209:etiology; D002648:Child; D002780:Cholestasis, Intrahepatic / Q000209:etiology; D003550:Cystic Fibrosis / Q000150:complications*; D005234:Fatty Liver / Q000209:etiology; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D011183:Postoperative Complications; D011552:Pseudomonas Infections / Q000209:etiology