Long-Term Results of Liver Transplantation in Patients 60 Years of Age and Older (original) (raw)

This retrospective analysis aims to evaluate results of hepatic based metabolic disorders as an indication for liver transplantation in a single center experience with regard to survival, metabolic cure and improvement of quality of life. Patients and methods: From 1991-2003, 363 patients have been transplanted in our center out of which 66 (17,8%) suffered from metabolic disorders: BylerЈs disease (PFIC2, n ϭ 17), MDR3-defect (PFIC3, n ϭ 11), Crigler-Najjar syndrome (CRNA, n ϭ 9), hyperoxaluria type 1 (OXAL. n ϭ 8), a1-AT deficiency (A1ATD, n ϭ 6), WilsonЈs disease (WЈd, n ϭ 4), neonatal hemochromatosis (NHC, n ϭ 4), tyrosinemia type 1 (Ty I n ϭ 3), urea cycle defects (UCD) :OTC-deficiency (OTC-d, n ϭ 2) Citrullinemia (n ϭ 1)., glycogen storage disease type 4 (GSD IV, n ϭ 1) and cystic fibrosis (CF, n ϭ 1). The diagnosis was based on classical clinical, biochemical and in some cases on molecular biological findings. The indication was fulminant liver failure (n ϭ 10), chronic liver failure (n ϭ 38), failure of a second organ (n ϭ 4) and preemptive (n ϭ 14). Transplantation technique was full size organ (n ϭ 15), reduced size (n ϭ 7), split-liver (n ϭ 30) and LR-LTX (n ϭ 14). Survival was calculated using the Kaplan-Meyer method. Results: The survival of the individual disorders is shown in table 1: The overall ten year survival of these patients was 80% compared to 78% of the non-metabolic indications. The bad survival in WЈd and NHC is explained by the presentation as fulminant liver failure. Relaps of the metabolic disease was observed in 1 patient after LRLTX in PFIC2. Metabolic cure was achieved in patients with PFIC3, CRNA, a1-ATD, NHC, UCD and GSD IV. In some patients the metabolic cure was only partial but still there was a good quality of life CF: diabetes m., PFIC 2: diarrhea(n ϭ 1), WЈd (n ϭ 1) and Ty I (n ϭ 1: tubulopathy). In hyperoxaluria I, preemptive LTX prevented kidney transplantation in 3 out of 4 patients. The post-op course in patients with HCC (incidental finding after hepatectomy n ϭ 1, detected before LTX (n ϭ 1) was uneventful in both children. As far as quality of life is concerned, there was a dramatic improvement in patients with PFIC2 and 3 with regard to itching, no need of further phototherapy in patients with CRNA or dietary restrictions in UCD. In LRLTX there was no morbidity related to liver metabolism in the donors and no impaired metabolism in the recipients. Conclusion: Liver transplantation in hepatic based metabolic disorders is very effective. Survival rates are comparable to nonmetabolic indications. Even LRLTX may be considered since our data show phenotypic cure in all and metabolic cure in 95% of patients. Quality of life improved significantly in all indications.