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Abstract
Background/Aims: As has been the case with other metabolic diseases of the liver in the last decade,
orthotopic liver transplantation has been applied to the treatment of Wilson's disease
with increasing frequency. The experience at the University of Pittsburg with orthotopic
liver transplantation for Wilson's disease is reported.
Methods: Between February 1981 and December 1991, 51 orthotopic liver transplants were performed
on 39 patients (16 pediatric, 23 adults) with Wilson's disease. Twenty-two patients
were transplanted because of a presentation co-existent with fulminant hepatic failure.
Seventeen presented with chronic advanced liver disease with (n=9) or without (n=8)
associated neurologic dysfunction.
Results: The rate of primary graft survival (n-39) was 73% and patient survival was 79.4%.
No patient mortality occurred beyond 3 weeks post-orthotopic liver transplantation.
Survival was better for those with a chronic advanced liver disease presentation (90%)
than it was for those with a fulminant hepatic failure (73%) presentation, but the
difference was not statistically significant.
Conclusions: 1) Currently, orthotopic liver transplantation is the treatment of choice for Wilson's
disease presenting as fulminant hepatic hepatic failure; 2) orthotopic liver transplantation
should be considered for patients with Wilson's disease with advanced, chronic liver
disease for whom no other therapy is possible; 3) orthotopic liver transplantation
only partially corrects the underlying metabolic defect of patients with Wilson's
disease and converts the copper kinetics from that characteristic of an individual
affected with a homozygous disease to that of an individual who is an obligate heterozygote,
thereby effecting a phenotypic cure.
Keywords
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Article info
Publication history
Accepted:
March 2,
1995
Received in revised form:
February 21,
1995
Received:
December 7,
1994
Identification
Copyright
© 1995 Published by Elsevier Inc.
