Skip to main content

Advertisement

SpringerLink
Log in

d-Penicillamine in Wilson's disease presenting as acute liver failure with hemolysis

  • Case Report
  • Published:
Digestive Diseases and Sciences Aims and scope Submit manuscript

Summary

Wilson's disease in a young woman presenting with an acute course is described. The clinical manifestations were fulminant hepatic failure associated with marked intravascular hemolysis. Immediated-penicillamine and high-dose steroid therapy did not infuence the course of the disease. Necropsy revealed an increased hepatic copper content and cirrhosis with extensive necrosis of the liver.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Wilson SAK: Progressive lenticular degeneration: familial nervous disease associated with cirrhosis of liver. Brain 34:295–509, 1912

    Google Scholar 

  2. Lawrie NR, Carter RA: Acute case of Wilson's disease. Lancet 1:1309–1311, 1958

    PubMed  Google Scholar 

  3. Adler R, Mahnovski V, Heuser ET, Presser DH, Robinson RG: Fulminant hepatitis—a presentation of Wilson's disease. Am J Dis Child 131:870–872, 1977

    PubMed  Google Scholar 

  4. Silverberg M, Gellis SS: The liver in juvenile Wilson's disease. Pediatrics 30:402–413, 1962

    PubMed  Google Scholar 

  5. Danks DM, Stevens BJ: Diagnosis of Wilson's disease in children with liver disease. Lancet 1:22–25, 1969

    PubMed  Google Scholar 

  6. Sternlieb I, Scheinberg H Chronic hepatitis as a first manifestation of Wilson's disease. Ann Intern Med 76:59–64, 1972

    PubMed  Google Scholar 

  7. Chalmers TC, Iber FL, Uzmann LL: Hepatolenticular degeneration (Wilson's disease) as a form of idiopathic cirrhosis. N Engl J Med 256:235–242, 1957

    PubMed  Google Scholar 

  8. McIntyre N, Clink HM, Levi AJ, Cumings JN, Sherlock S: Hemolytic anemia in Wilson's disease. N Engl J Med 276:439–444, 1967

    PubMed  Google Scholar 

  9. Deiss A, Lee GR, Cartwright GE: Hemolytic anemia in Wilson's disease. Ann Intern Med 73:413–418, 1970

    PubMed  Google Scholar 

  10. Scott J, Gollan JL, Samourian S, Sherlock S: Wilson's disease presenting as chronic active hepatitis. Gastroenterology 74:645–651, 1978

    PubMed  Google Scholar 

  11. Robitaille GA, Piscatelli RL, Majeski EJ, Gelehrter TC: Hemolytic anemia in Wilson's disease. J Am Med Assoc 237:2402–2403, 1977

    Google Scholar 

  12. Dobyns WB, Goldstein NP, Gordon H: Clinical spectrum of Wilson's disease (hepaticular degeneration). Mayo Clin Proc 54(1):35–42, 1979

    PubMed  Google Scholar 

  13. Walshe JM: Penicillamine, a new oral therapy for Wilson's disease. Am J Med 21:487–495, 1956

    PubMed  Google Scholar 

  14. Deiss A, Lynch RE, Lee GR, Cartwright GE: Long-term therapy of Wilson's disease. Ann Intern Med 75:57–65, 1971

    PubMed  Google Scholar 

  15. Sherlock S, Scheuer PJ: The presentation and diagnosis of 100 patients with primary biliary cirrhosis. N Engl J Med 289:674–678, 1973

    PubMed  Google Scholar 

  16. Soloway RD, Summerskill WHJ, Bagenstoss AH, Geall MG, Gitnick GL, Elveback LR, Schoenfield LJ: Clinical, biochemical and histological remission of severe chronic active liver disease: A controlled study of treatments and early prognosis. Gastroenterology 63:820–833, 1972

    PubMed  Google Scholar 

  17. Roche-Sicot J, Benhamou JP: Acute intravascular hemolysis and acute liver failure associated as a first manifestation of Wilson's disease. Ann Intern Med 86:301–303, 1977

    PubMed  Google Scholar 

  18. McCullough AJ, Fleming CR, Thistle JL, Baldus WP, Ludwig J, McCall JT, Dickson ER: The diagnosis of Wilson's disease (WD) presenting as fulminant hepatitis (FH). Gastroenterology 79:1037, 1980

    Google Scholar 

  19. Ware AJ, Jones RE, Shorey JW, Combes B: A controlled trial of steroid therapy in massive hepatic necrosis. Am J Gastroenterol 62:130–133, 1974

    PubMed  Google Scholar 

  20. Duve de C, Wattiaux R, Wibo M: Effects of fat-soluble compounds on lysosomesin vitro. Biochem Pharmacol 9:97–116, 1962

    PubMed  Google Scholar 

  21. Cartwright GE, Hodges RE, Gubler CJ, Mahoney JP, Daum K, Wintrobe MM, Bean JB: Studies on copper metabolism. XIII. Hepatolenticular degeneration. J Clin Invest 33:1487–1501, 1954

    PubMed  Google Scholar 

  22. Uzmann LL: Histochemical localization of copper with rubeanic acid. Lab Invest 5:299–305, 1956

    PubMed  Google Scholar 

  23. Fleming CR, Dickson ER, Wahner HW, Hollenhorst RW, McCall JT: Pigmented corneal rings in non-Wilsonian disease. Ann Intern Med 86:285–288, 1977

    PubMed  Google Scholar 

  24. Smallwood RA, Williams HA, Rosenoer VM, Sherlock S: Liver copper levels in liver disease studies using neutron activation analysis. Lancet 2:1310–1313, 1968

    PubMed  Google Scholar 

  25. Conrad ME, Schwartz FD, Young AA: Infectious hepatitis—a generalized disease. A study of renal, gastrointestinal and hematological abnormalities. Am J Med 37:789–801, 1964

    PubMed  Google Scholar 

  26. Jandl JH: The anemia of liver disease: Observations on its mechanism. J Clin Invest 34:390–404, 1955

    PubMed  Google Scholar 

  27. Albiston HE, Bull LB, Dick AT: A preliminary note on the etiology of enzootic jaundice toxaemic jaundice or “yellows” of sheep in Australia. Aust Vet J 16:233–243, 1940

    Google Scholar 

  28. Todd JR, Thompson RH: Studies of chronic copper poisoning II. Biochemical studies on the blood of sheep during the hemolytic crisis. Br Vet J 119:161–173, 1963

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Medicine and Department of Pathology, University of Mainz, Mainz, FRG

    W. Vielhauer MD, V. Eckardt MD, K. H. Holtermüller MD, J. B. Lüth MD, B. Schulte MD, W. Prellwitz MD & W. Sonntag MD

Authors
  1. W. Vielhauer MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. V. Eckardt MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. K. H. Holtermüller MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. J. B. Lüth MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. B. Schulte MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. W. Prellwitz MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. W. Sonntag MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Part of this work has been supported by the Deutsche Forschungsgemeinschaft (Ho 349/8-3), Bonn, Germany

Rights and permissions

Reprints and permissions

About this article

Cite this article

Vielhauer, W., Eckardt, V., Holtermüller, K.H. et al. d-Penicillamine in Wilson's disease presenting as acute liver failure with hemolysis. Digest Dis Sci 27, 1126–1129 (1982). https://doi.org/10.1007/BF01391452

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01391452

Keywords

Search

Navigation