Liver Support – A Task for Nephrologists? Extracorporeal Treatment of a Patient with Fulminant Wilson Crisis

@article{Manz2003LiverS,
  title={Liver Support – A Task for Nephrologists? Extracorporeal Treatment of a Patient with Fulminant Wilson Crisis},
  author={Tanja Manz and Andreas Ochs and Emmanuel Biss{\'e} and Christoph W. Strey and Wolfgang Grotz},
  journal={Blood Purification},
  year={2003},
  volume={21},
  pages={232 - 236}
}
Background: Patients with Wilson’s disease may present with cirrhosis, acute hepatitis or fulminant hepatic failure. Without urgent orthotopic liver transplantation, a fulminant Wilson crisis has a mortality of 100%. We report on an 18-year-old female patient with fulminant hepatic failure due to Wilson crisis. Methods: The molecular adsorbent recirculating system (MARS) was used to eliminate albumin-bound toxins and to bridge waiting until an organ became available. Results: A total of 18 MARS… 
Single pass albumin dialysis (SPAD) in fulminant Wilsonian liver failure: a case report
TLDR
It is concluded that SPAD is potentially an effective treatment in fulminant Wilson disease with hemolysis but that it should be used in combination with chelation to optimize the removal of copper.
Effect of Molecular Adsorbents Recirculating System Treatment in Children With Acute Liver Failure Caused by Wilson Disease
TLDR
MARS is able to remove copper and to stabilise children with ALF secondary to WD, allowing bridging to LT.
Use of the molecular adsorbents recirculating system as a treatment for acute decompensated wilson disease
  • A. Chiu, N. Tsoi, S. Fan
  • Medicine
    Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • 2008
TLDR
In both cases, MARS was used as a means of preventing deterioration rather than salvaging devastation, and may confer benefits to patients with acute decompensated Wilson disease if it is started early in the course of illness.
Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis
TLDR
The case of a young woman who presented with classic acute Wilsonian hepatitis complicated by liver and renal failure and a severe hemolysis related to massive nonceruloplasmin bound copper accumulation requiring repeated blood transfusions is reported here.
Liver transplantation for Wilson disease.
TLDR
Living related liver transplantation represents an alternative to deceased donor LT with excellent long-term survival, without disease recurrence, in patients with Wilson disease (WD).
Fulminant Wilson Disease in Children: Recovery After Plasma Exchange Without Transplantation
TLDR
Multiple case reports and case series demonstrate transplant free survival after plasma exchange and subsequent chelation therapy, despite a NWI ≥11, which is a therapeutic option in children and young adults presenting with fulminant WD.
A 15-Year-Old Girl with Severe Hemolytic Wilson’s Crisis Recovered without Transplantation after Extracorporeal Circulation with the Prometheus® System
TLDR
This is the first report of FPSA albumin dialysis of a patient with Wilson’s crisis and the first reported in which a patients with a WD score >11 survived without transplantation.
Plasmapheresis for hemolytic crisis and impending acute liver failure in Wilson disease
TLDR
A patient presenting with severe hemolysis and impending acute liver failure that made a rapid recovery with prompt initiation of plasmapheresis and chelation therapy and Rapid copper removal by plasmAPheresis alleviated hemolytics and liver injury.
Liver support for fulminant hepatic failure: Is it time to use the Molecular Adsorbents Recycling System in children?*
  • P. Tissières, J. Sasbón, D. Devictor
  • Medicine
    Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
  • 2005
TLDR
Although promising preliminary results suggest that MARS may have a significant position in the therapeutic arsenal for FHF, no sufficient data exist to justify its use in children, and the indications of this expensive technique in children with FHF are limited.
The role of the Molecular Adsorbents Recirculating System (MARS) in the management of liver failure
TLDR
The Molecular Adsorbents Recirculating System (MARS) is an extracorporeal liver support device based on dialysis across an albumin-impregnated membrane, using 20% albumin as dialysate and charcoal and anion exchange resin columns in the circuit help cleanse and regenerate the dialysates.
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