Open AccessORTHOTOPIC LIVER TRANSPLANTATION FOR BYLER DISEASE
Author(s) -
Olivier Soubrane,
F. Gauthier,
Denis Devictor,
Olivier Bernard,
J Valayer,
Didier Houssin,
Y Chapuis
Publication year - 1990
Publication title -
transplantation
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 1.45
H-Index - 204
eISSN - 1534-6080
pISSN - 0041-1337
DOI - 10.1097/00007890-199011000-00012
Subject(s) - medicine , liver transplantation , cholestasis , surgery , transplantation , disease , retrospective cohort study , liver disease , thrombosis , gastroenterology
Byler disease is a rare form of familial intrahepatic cholestasis that is fatal before puberty. This retrospective study reviewed the results of orthotopic liver transplantation in 14 children with Byler disease using 12 whole-liver grafts and 2 reduced-size grafts. One post-operative death occurred after retransplantation for arterial thrombosis. In the other patients, infectious problems and rejection episodes were the most frequent complications during the postoperative period. In the 13 patients alive, graft function, growth, and quality of life were good after an average follow-up of 17 months without any sign of disease recurrence.