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CC BY 4.0 · Journal of Child Science 2021; 11(01): e145-e147
DOI: 10.1055/s-0041-1731079
Case Report

Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis

Nida Mirza 
1   Department of Paediatrics, Sri Aurobindo Institute of Medical Science, Indore, Madhya Pradesh, India
,
Ravi Bharadwaj
2   Department of Paediatric Gastroenterology, Indraprastha Apollo Hospital, New Delhi, India
,
Smita Malhotra
2   Department of Paediatric Gastroenterology, Indraprastha Apollo Hospital, New Delhi, India
,
Anupam Sibal
2   Department of Paediatric Gastroenterology, Indraprastha Apollo Hospital, New Delhi, India
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Abstract

Wilson disease (WD) is a disorder of copper metabolism resulting in accumulation of copper in vital organs of the human body, predominantly in the liver and the brain. Acute liver failure in WD has a bad prognosis, especially with a score ≥11 in the revised WD prognostic index; emergency liver transplantation is considered the only life-saving option in this scenario. Here, we reported a girl patient with WD-induced liver failure and poor prognostic score who was rescued by plasmapheresis. She also manifested severe Coombs negative hemolytic anemia and acute kidney injury. This case report highlights the utility of an adjunctive modality besides liver transplantation for the management of fulminant liver failure caused by WD.

Keywords

acute liver failure - Wilson disease - plasmapheresis


Publication History

Received: 25 March 2021

Accepted: 24 April 2021

Article published online:
22 June 2021

© 2021. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Roberts EA, Schilsky ML. American Association for Study of Liver Diseases (AASLD). Diagnosis and treatment of Wilson disease: an update. Hepatology 2008; 47 (06) 2089-2111
    CrossrefPubMedGoogle Scholar
  • 2 Sternlieb I. Perspectives on Wilson's disease. Hepatology 1990; 12 (05) 1234-1239
    CrossrefPubMedGoogle Scholar
  • 3 Kreymann B, Seige M, Schweigart U, Kopp KF, Classen M. Albumin dialysis: effective removal of copper in a patient with fulminant Wilson disease and successful bridging to liver transplantation: a new possibility for the elimination of protein-bound toxins. J Hepatol 1999; 31 (06) 1080-1085
    CrossrefPubMedGoogle Scholar
  • 4 Dhawan A, Taylor RM, Cheeseman P, De Silva P, Katsiyiannakis L, Mieli-Vergani G. Wilson's disease in children: 37-year experience and revised King's score for liver transplantation. Liver Transpl 2005; 11 (04) 441-448
    CrossrefPubMedGoogle Scholar
  • 5 Kiss JE, Berman D, Van Thiel D. Effective removal of copper by plasma exchange in fulminant Wilson's disease. Transfusion 1998; 38 (04) 327-331
    CrossrefPubMedGoogle Scholar
  • 6 Reynolds HV, Talekar CR, Bellapart J, Leggett BA, Boots RJ. Copper removal strategies for Wilson's disease crisis in the ICU. Anaesth Intensive Care 2014; 42 (02) 253-257
    PubMedGoogle Scholar
  • 7 Larsen FS, Schmidt LE, Bernsmeier C. et al. High-volume plasma exchange in patients with acute liver failure: an open randomised controlled trial. J Hepatol 2016; 64 (01) 69-78
    CrossrefPubMedGoogle Scholar
  • 8 Hilal T, Morehead RS. Fulminant Wilson's disease managed with plasmapheresis as a bridge to liver transplant. Case Rep Med 2014; 2014: 672985 DOI: 10.1155/2014/672985.
    CrossrefPubMedGoogle Scholar
  • 9 Verma N, Pai G, Hari P, Lodha R. Plasma exchange for hemolytic crisis and acute liver failure in Wilson disease. Indian J Pediatr 2014; 81 (05) 498-500
    CrossrefPubMedGoogle Scholar
  • 10 Zhang Y, Li L, Zhang X, Xu W, Guo Q, Zhou J. Plasmapheresis combined with continuous plasma filtration adsorption rescues severe acute liver failure in Wilson's disease before liver transplantation. Blood Purif 2019; 47 (1-3): 120-125
    CrossrefPubMedGoogle Scholar
  • 11 Damsgaard J, Larsen FS, Ytting H. Reversal of acute liver failure due to Wilson disease by a regimen of high-volume plasma exchange and penicillamine. Hepatology 2019; 69 (04) 1835-1837
    CrossrefPubMedGoogle Scholar
  • 12 Tian Y, Gong GZ, Yang X, Peng F. Diagnosis and management of fulminant Wilson's disease: a single center's experience. World J Pediatr 2016; 12 (02) 209-214
    CrossrefPubMedGoogle Scholar