Transplantation 1990 Nov;50(5):804-6

Orthotopic liver transplantation for Byler disease

Soubrane O, Gauthier F, DeVictor D, Bernard O, Valayer J, Houssin D, Chapuis Y
Surgical Clinic, Cochin Hospital, Paris, France

Abstract
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