Document Detail

Orthotopic liver transplantation for Wilson's disease: a single-center experience.
MedLine Citation:
PMID:  11602847     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Wilson's disease is an inherited disorder of copper metabolism characterized by reduced biliary copper excretion, which results in copper accumulation in tissues with liver injury and failure. Orthotopic liver transplantation (OLT) can be lifesaving for patients with Wilson's disease who present with fulminant liver failure and for patients unresponsive to medical therapy. The aim of this study is to review our experience with OLT for patients with Wilson's disease. METHODS: Between 1988 and 2000, 21 OLTs were performed in 17 patients with Wilson's disease. Patient demographics, pre-OLT laboratory data, operative data, and early and late postoperative complications were reviewed retrospectively. One-year patient and graft survival was calculated. RESULTS: Eleven patients had fulminant Wilson's disease; in six patients the presentation was chronic. Mean patient age at presentation was 28 years (range 4-51 years); mean follow-up was 5.27 years (range 0.4-11.4 years). Neurologic features of Wilson's disease were not prominent preoperatively and did not develop post-OLT except in one patient who developed acute neuropsychiatric illness and seizure. Renal failure, present in 45% of patients with fulminant Wilson's disease, resolved post-OLT with supportive care. One-year patient and graft survivals were 87.5% and 62.5%, respectively. Fifteen survivors have remained well with normal liver function and no disease recurrence. CONCLUSION: Liver transplantation for hepatic complications of Wilson's disease cures and corrects the underlying metabolic defect and leads to long-term survival in patients who present with either acute or chronic liver disease. Acute renal failure develops frequently in patients with fulminant Wilsonian hepatitis and typically resolves postoperatively.
S Emre; E O Atillasoy; S Ozdemir; M Schilsky; C V Rathna Varma; S N Thung; I Sternlieb; S R Guy; P A Sheiner; M E Schwartz; C M Miller
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Transplantation     Volume:  72     ISSN:  0041-1337     ISO Abbreviation:  Transplantation     Publication Date:  2001 Oct 
Date Detail:
Created Date:  2001-10-16     Completed Date:  2001-12-04     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0132144     Medline TA:  Transplantation     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1232-6     Citation Subset:  IM    
The Recanati-Miller Transplant Institute, Department of Medicine, The Mount Sinai School of Medicine, New York, NY, USA.
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MeSH Terms
Child, Preschool
Chronic Disease
Graft Survival
Hepatolenticular Degeneration / pathology,  physiopathology,  surgery*
Liver / pathology
Liver Transplantation*
Middle Aged
Postoperative Complications
Retrospective Studies
Severity of Illness Index
Survival Analysis

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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