Korean J Transplant.  2022 Nov;36(Supple 1):S111. 10.4285/ATW2022.F-2306.

Patient selection and outcomes of liver transplantation for adult patients with Wilson disease: a single center experience

• Hwang S
• Kim M
• Kim M
• Ahn CS
• Moon DB
• Ha TY
• Song GW
• Jung DH
• Park GC
• Kang WH

• KMID: 2535627
• DOI: http://doi.org/10.4285/ATW2022.F-2306

Abstract

Background
Liver transplantation (LT) is a definitive cure for Wilson's disease (WD). LT cures the basic defect and is treatment option for liver failure or neurological symptoms of WD. Limited outcomes are reported regarding LT for adult WD patients in Ko-rea.
Methods
Thirty-nine adult patients of our institutional LT cohort from January 2001 to December 2020 were retrospectively ana-lyzed.
Results
The study cohort comprised of 16 males and 23 females, aged 33.9±11.4 years. Four patients had acute liver failure. The model for end-stage liver disease score of other patients were 24.7±10.6. Types of LT were deceased donor LT in 13 and living donor LT in 26. Mean graft-to-recipient ratio in living donor LT recipients were 1.08±0.28%. WD was pathologically con-firmed at explant pathology. Concurrent hepatocellular carcinoma was identified in 3 patients. Posttransplant hospital stay was 43.4±35.4 days. In-hospital mortality was four (10.3%) patients due to septic shock, multi-organ failure and hepatorenal syndrome. Three of these in-hospital mortality cases were deceased LT recipients. During follow-up, only one (2.7%) patient died from hepatocellular carcinoma recurrence at posttransplant 2 years. The overall survival rates were all 89.7% at 1 year, and 87.2% at 5 years, 10 years and 20 years.
Conclusions
The long-term posttransplant outcomes of WD in adult patients were excellent, thus LT is eligibly indicated for WD patients manifesting liver failure.