1: Pediatrics 1987 Oct;80(4):571-4

Orthotopic liver transplantation in patients with cystic fibrosis

Cox KL, Ward RE, Furgiuele TL, Cannon RA, Sanders KD, Kurland G
Department of Pediatrics, University of California, Davis Medical Center,
Sacramento 95817

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