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Liver Transplantation for Wilson's Disease in Non-adult Patients: A Systematic Review
Z Garoufalia1, A Prodromidou1, N Machairas2
12nd Department of Propaedeutic Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
Introduction:
Wilson's disease (WD) is a rare autosomal recessive disorder transmitted through a gene located on chromosome 13. Liver transplantation (LT) provides a therapeutic option for patients with WD presenting fulminant liver failure or drug resistance. LT in patients with WD has a twofold aim: to save the patient's life when the disorder has progressed to hepatic (or other organ) failure and to cure the underlying metabolic defect. The aim of our study was to investigate the indications, aspects and post-operative outcomes in pediatric patients (< 18 years old) with WD who underwent LT.
Methods:
A meticulous search of the literature since 1971 was performed. A retrospective analysis of all the studies, presenting cases of LT in children due to WD, was conducted. Studies that did not report patients' characteristics, transplantation indications, post-operative outcomes, and complications, as well as those with small study populations (< 10 patients), were excluded.
Results:
Six studies were included in the present review, which involved 290 children. The main indications for LT included chronic liver failure and fulminant liver failure. The average 1-year survival rate was 91.9%, while the average 5-year survival rate was 88.2%. Retransplantation was performed in 16 patients due to transplant rejection. In general, patients transplanted for WD displayed an excellent quality of life after LT.
Conclusion:
LT is a safe and efficient procedure in selected pediatric patients with WD, demonstrating excellent long-term outcomes and quality of life.
