Orthotopic Liver Transplantation for Byler's Disease

In this study we analyzed the features of 12 patients who underwent liver transplantation for progressive familial intrahepatic cholestasis (Byler's disease [BD]) in view of the technical features of the OLTx, incidence and type of complications, need for retransplantation, as well as patient a...

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Bibliographic Details
Published in:Transplantation proceedings 2005-03, Vol.37 (2), p.1149-1150
Main Authors: Torri, E., Lucianetti, A., Pinelli, D., Corno, V., Guizzetti, M., Maldini, G., Zambelli, M., Bertani, A., Melzi, M.L., Alberti, D., Doffria, E., Giovanelli, M., Torre, G., Spada, M., Gridelli, B., Colledan, M.
Format: Article
Language:English
Subjects:
Adolescent
Biological and medical sciences
Child
Child, Preschool
Female
Follow-Up Studies
Fundamental and applied biological sciences. Psychology
Fundamental immunology
Gastroenterology. Liver. Pancreas. Abdomen
Humans
Infant
Liver Cirrhosis, Biliary - surgery
Liver Transplantation
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical sciences
Other diseases. Semiology
Retrospective Studies
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Tissue, organ and graft immunology
Treatment Outcome
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Summary:In this study we analyzed the features of 12 patients who underwent liver transplantation for progressive familial intrahepatic cholestasis (Byler's disease [BD]) in view of the technical features of the OLTx, incidence and type of complications, need for retransplantation, as well as patient and graft survivals. BD was the indication in 12 patients of median age 1.32 years and median weight 10 kg. Median follow-up was 670 days. Major surgical complications requiring reintervention occurred in three patients. No thrombosis of the hepatic artery was observed. Infections with positive blood cultures were diagnosed in four patients. One patient had a biliary anastomotic stenosis successfully treated by percutaneous techniques. Four patients had episodes of acute rejection treated with steroids. Two patients were retransplanted, both of whom died in the early postoperative period due to hepatic vein thrombosis and venoenteric fistula. The actuarial patient and graft survival was 83% at 1 year and 83% at 5 years. Split-liver grafts represent an excellent organ supply for these patients, achieving good results with no mortality on the waiting list.
ISSN:0041-1345
1873-2623
DOI:10.1016/j.transproceed.2005.01.041