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Liver Transplantation for Wilson Disease

  • Tamir Miloh
  • Michael L. Schilsky
Chapter
Part of the Clinical Gastroenterology book series (CG)

Abstract

Liver transplantation (LT) for patients with Wilson disease (WD) is lifesaving for WD patients with acute liver failure (ALF) and liver failure from chronic end-stage liver disease or for patients with non-resectable liver cancer (hepatocellular carcinoma). LT restores liver function and resolves portal hypertension. LT in WD is currently not indicated for solely neurologic or psychiatric WD without liver failure. LT carries the risk of morbidity and mortality of the transplant surgery and requires lifelong immunosuppression. Outcomes for LT in WD are overall superior to most other indications. Patients with WD and ALF are listed as status 1a, the highest priority on the wait list, and patients with chronic disease are listed with a calculated model of end-stage liver disease score that uses several biochemical variables and is based on age (pediatric PELD or adult MELD with sodium adjustment). Options for donor organs are deceased (cadaveric) orthotopic (replacing the recipients’ liver), living liver donation, and auxiliary or heterotopic LT (where a new graft is placed without removing the patients’ liver). Hepatocyte or stem cell transplantation is under investigation.

Keywords

Wilson disease Liver transplant Copper Liver failure Cirrhosis 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Pediatric Hepatology and Liver Transplant MedicineTexas Children’s HospitalHoustonUSA
  2. 2.Baylor College of MedicineHoustonUSA
  3. 3.Division of Digestive Diseases and Transplant and Immunology, Departments of Medicine and SurgeryYale UniversityNew HavenUSA

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