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Liver transplantation for Wilson's disease

  • Somshekhar Bellary
    Affiliations
    Division of Transplantation Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania and Oklahoma Transplant Institute, Baptist Medical Center of Oklahoma, Oklahoma City, Oklahoma, USA
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  • Tarek Hassanein
    Affiliations
    Division of Transplantation Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania and Oklahoma Transplant Institute, Baptist Medical Center of Oklahoma, Oklahoma City, Oklahoma, USA
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  • David H. Van Thiel
    Correspondence
    Correspondence: David H. Van Thiel, M.d., Oklahoma Transplant Institute, Baptist Medical Center of Oklahoma, 3300 North Expressway, Oklahoma City, Oklahoma 73112, USA
    Affiliations
    Division of Transplantation Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania and Oklahoma Transplant Institute, Baptist Medical Center of Oklahoma, Oklahoma City, Oklahoma, USA
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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.

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      References

        • Frommer DJ
        Defective biliary excretion of copper in Wilson's disease.
        Gut. 1976; 15: 125-129
        • Walshe JM
        Wilson's disease: yesterday, today and tomorrow.
        Mov Disord. 1988; 3: 10-29
        • Walshe JM
        Treatment of Wilson's disease with trientine (trimethylene tetramine) dihydrochloride.
        Lancet. 1982; i: 643-647
        • Hoogenraad TV
        • Van Hattum J
        • Van der Hamer CJA
        Management of Wilson's disease with zinc sulphate.
        J Neurol Sci. 1987; 77: 137-146
        • Walshe JM
        Tetrathimolybdate (MIS4) as an anti-copper agent in man.
        in: Scheinberg IH Walshe JM Orphan diseases and orphan drugs. Manchester University Press, Manchester, England1986: 76-85
        • Schilsky ML
        • Scheinberg IH
        • Sternlieb I
        Prognosis of Wilsonian chronic active hepatitis.
        Gastroenterology. 1991; 100: 762-767
      1. a
        • Olsson R
        • Lindgren A
        • Frizell M
        Early diagnosis is crucial for the prognosis of Wilson's disease.
        Lakartidningen. 1991; 88 (Swe): 229-230
      2. b
        • Olsson R
        • Lindgren A
        • Frizell M
        Early diagnosis is crucial for the prognosis of Wilson's disease.
        Lakartidningen. 1991; 88 (Swe): 232
        • Zilker TR
        • Felgenhauer N
        • Hibler A
        • von Clarmann M
        Indications for liver transplantation in Wilson's disease with a fulminant course.
        Dtsch Med Wochenschr. 1989; 114: 871-875
        • Gottrand F
        • Razemon M
        • Otte JB
        • Vigier JE
        • Farraiux JP
        Indications for liver transplantation in Wilson's disease.
        Arch Fr Pediatr. 1988; 45: 187-188
        • Esquivel CO
        • Marino IR
        • Fioravanti V
        • Van Thiel DH
        Liver transplantation for metabolic diseases of the liver.
        Gastroenterology Clin N Am. 1988; 17: 167-175
        • Polson RJ
        • Rolles K
        • Calne RY
        • Williams R
        • Marsden D
        Reversal of severe neurological manifestations of Wilson's disease following orthotopic liver transplantation.
        Q J Med. 1987; 64: 685-691
        • Rela M
        • Heaton N
        • Mondragon R
        • Vougas V
        • O'Grady J
        • Mieli-Vergani G
        • Mowat A
        • Williams R
        • Tan KC
        Orthotopic liver transplantation for hepatic complications of Wilson's disease.
        Gut. 1992; 33: S54
        • Sternlieb I
        Wilson's disease: indications for liver transplant.
        Hepatology. 1984; 4: 15S-17S
        • DuBois RS
        • Giles G
        • Rodgerson DO
        • Lilly J
        • Martineau G
        • Halgrimson CG
        • Shroter G
        • Starzl TE
        • Sternlieb I
        • Scheinberg IH
        Orthotopic liver transplantation for Wilson's disease.
        Lancet. 1971; i: 505-508
        • Berman DH
        • Leventhal RI
        • Gavaler JS
        • Cadoff Em
        • Van Thiel DH
        Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure.
        Gastroenterology. 1991; 100: 1129-1134
        • Trey C
        • Davidson LS
        The management of fulminant hepatic failure.
        in: Pepper H Schaffner F Progress in Liver Disease. Grune & Stratton, Philadelphia1970: 282-298
        • Retzlaff JA
        • Tauxe WN
        • Kiely JM
        • Stroebel CF
        Erythrocyte volume, plasma volume and lean body mass in adult men and women.
        Blood. 1969; 33: 649-667
        • Dubois EF
        Basal Metabolism in Health and Disease. Lea and Febiger, Philadelphia1936: 125
        • Owen CA
        Measuring radioactivity in the diagnostic laboratory.
        Diagnostic Radioisotopes. 24. Charles C. Thomas, Inc, Springfield Illinois1959
        • Tauxe WN
        • Goldstein NP
        • Randall RN
        • Gross JB
        Copper metabolism in carriers of Wilson's disease: analysis of kinetics of intravenously injected radiocopper as a means of detecting the carrier state.
        in: Bergsma D Wilson's Disease. 45-48. National Foundation - March of Dimes, New York1968: 60-63
        • Zietelli BJ
        • Malatack JJ
        • Gartner Jr, JC
        • Shaw BW
        • Iwatsuki S
        • Starzl TE
        Orthotopic liver transplantation in children with hepatic-based metabolic disease.
        Transplant Proc. 1983; 15: 1284-1287
        • Park RHR
        • McCabe P
        • Fell GS
        • Russell RI
        Wilson's disease in Scotland.
        Gut. 1991; 32: 1541-1545
        • Stremmel W
        • Meyerrose K-W
        • Niederau C
        • Hefter H
        • Kreuzpainter G
        • Strohmeyer G
        Wilson's disease: clinical presentation, treatment and survival.
        Ann Intern Med. 1991; 115: 720-726
        • Beart Jr, RW
        • Putnam CW
        • Porter KA
        • Starzl TE
        Liver transplantation for Wilson's disease.
        Lancet. 1975; i: 176-177
        • Rothfus WE
        • Hirsch WL
        • Malatack J
        • Bergman I
        Improvement of cerebral CT abnormalities following liver transplantation in a patient with Wilson's disease.
        J Comput Assist Tomogr. 1988; 12: 138-140
        • Schilsky ML
        • Scheinberg IH
        • Stonlieb I
        Liver transplantation for Wilson's disease: indications and outcome.
        Hepatology. 1994; 19: 583-587
        • Pennington Jr, JC
        Quality of life following liver transplantation.
        Transplant Proc. 1989; 21: 3514-3516