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Liver transplantation for Wilson disease
Ahsan Ahmad1, Euriko Torrazza-Perez2, Michael L Schilsky3
1Section of Transplantation and Immunology, Department of Surgery, Yale University School of Medicine, New Haven, CT, USA.
Handbook of Clinical Neurology
|April 24, 2017
Summary
Liver transplantation (LT) offers a curative solution for Wilson disease (WD), restoring liver function and managing complications. While effective for advanced liver disease and cancer, its use for neurological symptoms is debated.
Area of Science:
- Hepatology and Transplant Surgery
- Genetic Liver Diseases
Background:
- Wilson disease (WD) is a rare genetic disorder causing copper accumulation.
- Liver transplantation (LT) is a definitive treatment for end-stage WD.
- Portal hypertension is a common complication managed by LT.
Purpose of the Study:
- To review the role and outcomes of LT in Wilson disease.
- To discuss indications, surgical options, and future directions for WD management.
Main Methods:
- Literature review of studies on liver transplantation for Wilson disease.
- Analysis of indications, surgical techniques, and patient outcomes.
Main Results:
- LT effectively restores liver function and resolves portal hypertension in WD patients.
- Excellent outcomes are reported for LT in WD, with supportive care improving bridge-to-transplant success.
- Hepatocellular carcinoma in WD is an indication for LT when resection is not feasible.
Conclusions:
- Liver transplantation is a life-saving and curative option for Wilson disease.
- LT indications encompass acute liver failure, end-stage disease, and specific cancers.
- Future cell-based therapies may offer alternative or supplementary treatments for WD.