Clin Transplant 1998 Jun;12(3):228-32

Orthotopic liver transplantation for hepatic associated metabolic disorders

Pratschke J, Steinmuller T, Bechstein WO, Neuhaus R, Tullius SG, Jonas S,
Schumacher G, Luck W, Becker M, Neuhaus P
Department of Surgery, Rudolf-Virchow Clinic, Humboldt University, Berlin,
Germany.
Abstract
Hepatic associated metabolic disorders represent 5% of the indications for
orthotopic liver transplantation (OLTX) according to the European Liver
Transplant Registry. We studied the outcome of this group at our institution
after OLTX and combined liver/kidney transplantation. Between September 1988 and
January 1997, 837 OLTXs were performed in 735 patients. Patient survival and
graft function at 1 yr were 91.3 and 86%, respectively. Thirty-nine OLTXs were
performed in 38 patients (15 female/23 male, median age +/- SD: 35 +/- 14 yr,
range 4-60 yr) due to liver associated metabolic disorders (4.7%). Indications
included Wilson's disease (n = 14), alpha-1-anti-trypsin-deficiency (n = 7),
hemochromatosis (n = 4), erythropoetic protoporphyria (n = 4), cystic fibrosis
(n = 2), Crigler-Najjar syndrome type I (n = 1), glycogenosis type I (n = 1),
ornithine-transcarbomylase-deficiency (n = 1). In addition 4 patients suffering
from primary hyperoxaluria type I received combined liver/kidney grafts.
Survival rate the 1 yr after OLTX and combined OLTX/NTX was 91.8%. Twenty
patients received cyclosporin A (55%) and 17 patients tacrolimus (45%) as
primary immunosuppression. The mean follow-up was 28.6 months (range 4-73
months). Two patients with hemochromatosis died 1 and 3 months after OLTX,
respectively, from Aspergillus sepsis followed by multiorgan-failure. One
patient died of malignant lymphoma 5 months after transplantation. One patient
required retransplantation 2 months after OLTX following arterial thrombosis and
ischemic type biliary lesion. One year after OLTX, all patients demonstrated
good graft function, liver grafts (ALT 17.9 +/- 13.6 IU/L, bilirubin 0.8 +/-
0.3.mg/dl, thromboplastin time 94 +/- 15%), and combined liver/kidney grafts
(creatinine 2.4 +/- 1.4 mg/dl). OLTX, respectively combined OLTX/NTX, represent
a successful therapy for hepatic associated metabolic disorders. Survival rates
and graft function are similar to those in liver graft recipients for
established indications at our institution. OLTX seems to be an excellent
treatment for hepatic based therapy resistant neurological disorders