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Canadian Journal of Gastroenterology
Volume 20, Issue 7, Pages 475-478
http://dx.doi.org/10.1155/2006/539345
Original Article

Liver Transplantation for Cirrhosis in Cystic Fibrosis

T Lamireau,1 S Martin,1 M Lallier,2 JE Marcotte,3 and F Alvarez1

1Division of Pediatric Gastroenterology, Hôpital Sainte-Justine, Montreal, Quebec, Canada
2Division of Pulmonology, Hôpital Sainte-Justine, Montreal, Quebec, Canada
3Division of Surgery, Hôpital Sainte-Justine, Montreal, Quebec, Canada

Received 24 January 2006; Accepted 24 January 2006

Copyright © 2006 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

BACKGROUND: Liver disease is the third most common cause of death in children with cystic fibrosis (CF). Liver transplantation is an effective treatment in children with hepatic failure.

AIMS: The objective of the present study was to review the indications and postoperative course of hepatic transplantation in a cystic fibrosis population.

PATIENTS: Five children with CF, at a mean age of 16.5 years, underwent liver transplantation.

RESULTS: All patients showed cirrhosis, portal hypertension and hepatic failure. The main postoperative complication was ascites refractory to treatment in two patients. No significant deterioration of the pulmonary function was noted. Two patients died, one of Hodgkin lymphoma and the other of progressive pulmonary failure.

CONCLUSION: Liver transplantation was indicated in children with CF when hepatic failure and/or severe portal hypertension was present with well-preserved pulmonary function.