Elsevier

Journal of Hepatology

Volume 23, Issue 4, October 1995, Pages 373-381
Journal of Hepatology

Liver transplantation for Wilson's disease

https://doi.org/10.1016/0168-8278(95)80194-4Get rights and content

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.

References (27)

  • JM Walshe

    Tetrathimolybdate (MIS4) as an anti-copper agent in man

  • R Olsson et al.

    Early diagnosis is crucial for the prognosis of Wilson's disease

    Lakartidningen

    (1991)
    R Olsson et al.

    Early diagnosis is crucial for the prognosis of Wilson's disease

    Lakartidningen

    (1991)
  • TR Zilker et al.

    Indications for liver transplantation in Wilson's disease with a fulminant course

    Dtsch Med Wochenschr

    (1989)
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