Abstract
Wilson disease (WD) is an uncommon indication for liver transplantation. It carries outstanding long-term patient and graft survival rates for both children and adults with either acute liver failure (ALF) [notably, the distinctive presentation denoted as classic Wilsonian ALF (ALF-WD)] or end-stage cirrhosis. Once patients present with ALF-WD, it is unlikely that they will recover without a transplant, so they are prioritized on transplant waiting lists. There is evidence that timely and ongoing medical therapy can stabilize the chronic liver dysfunction associated with WD, and it should always be attempted prior to transplantation if time allows. It remains controversial as to the extent of reversibility of neuropsychiatric complications, but consensus opinion is that liver transplantation should not be pursued for neuropsychiatric disease in the absence of liver disease; severe neurological disease may not be reversible with transplantation and appears associated with poorer postoperative outcomes. Long-term posttransplant outcomes in WD are otherwise excellent, in part, because the patients are younger and do not have medical comorbidities. Copper metabolism normalizes posttransplantation, even when a live donor transplant is performed from a first-degree relative who carries one abnormal ATP7B allele.
Original language | English |
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Title of host publication | Clinical and Translational Perspectives on WILSON DISEASE |
Publisher | Elsevier |
Pages | 391-398 |
Number of pages | 8 |
ISBN (Electronic) | 9780128105320 |
ISBN (Print) | 9780128105337 |
DOIs | |
State | Published - 1 Jan 2018 |
Externally published | Yes |
Keywords
- Acute liver failure
- Cirrhosis
- MELD score
- Neuropsychiatric
- PELD score
- Portal hypertension
- Transplantation