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

Abstract

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 sessions and 4 plasma exchange sessions were performed. Bilirubin levels and hepatic encephalopathy improved under MARS therapy. A total of 75 mg copper was removed until serum copper levels were within the normal range. Copper elimination was measured in 15 MARS treatments, which removed a total of 12.9 mg copper. Four plasma exchange sessions, with a total exchange of 11 liters of plasma, removed 12 mg copper. Urinary copper elimination with penicillamine was 50 mg. Conclusion: MARS was an effective method to stabilize a patient with Wilson crisis, contributed to copper elimination and gained time for liver transplantation. The risk of high-urgency transplantation could be avoided. Liver support was easy in the hands of nephrologists familiar with extracorporeal therapy.

A 15-Year-Old Girl with Severe Hemolytic Wilson’s Crisis Recovered without Transplantation after Extracorporeal Circulation with the Prometheus® System

“…The MARS system [13,15] or a modification [13,14] has been applied earlier on patients with fulminant WD. The duration of the treatment ranged from some hours to well over 100 h. The patients improved or stabilized [13][14][15] , but eventually they were all transplanted.…”

“…pheresis [4][5][6][7][8][9][10][11][12] or the MARS system (molecular adsorbent recirculating system) [13][14][15] . In most of the reported cases [4,5,7,[9][10][11][13][14][15] , the patients were eventually transplanted even though the extracorporeal liver support system chosen stabilized the patient and thus provided a bridge to transplantation.…”

“…In most of the reported cases [4,5,7,[9][10][11][13][14][15] , the patients were eventually transplanted even though the extracorporeal liver support system chosen stabilized the patient and thus provided a bridge to transplantation. The mechanism was believed to be removal of copper from plasma, though there seems to be substantial variation in the fraction of the total excretion that can be contributed to the albumin dialysis relative to urinary excretion [4][5][6][7][8][9][10][11][12][13][14][15] . The Prometheus FPSA (fractionated plasma separation and adsorption) system is a recently developed, somewhat different albumin dialysis technique [16][17][18] .…”

Wilson’s disease: A review of what we have learned

“…The specific prognostic index revised Wilson prognostic index represents a valid tool for assessing these critically ill patients [121,122] . As bridging therapies to liver transplantation, or as an alternative altogether in regions were liver transplantation is not possible, strategies such as rapid plasma exchange [124,125] via any method such as plasmapheresis [126] , hemofiltration [127,128] , albumin dyalisis [129] , or exchange transfusion, may be successfully used to lower circulating copper levels, renal protection from coppermediated tubular damage, and reduce hemolysis [130] . Albeit some degree of improvement has been reported, the need for liver transplantation has not been obviated in numerous cases, although successful treatment without transplantation has been reported [131] .…”

Multiorgan Dysfunction: the State-of-the-Art and Current Approaches to Its Treatment Using Low Flow-Rate Membrane Technologies

“…На основании данных, полученных в результате мульти центровых исследований, и анализов клинических случаев [34,[79][80][81] исследователи определяют следующие показания к применению МАРС терапии:…”