Single pass albumin dialysis (SPAD) in fulminant Wilsonian liver failure: a case report

Abstract

Since fulminant Wilsonian liver failure has an extremely poor prognosis, the use of a liver support system that can bridge patients to liver transplant is life saving. We report here the case of a 17-year-old female who presented with fulminant Wilsonian liver failure and intravascular hemolysis. With the subsequent development of encephalopathy and oliguria, single pass albumin dialysis (SPAD) was initiated for 5 days to augment copper removal. Continuous venovenous hemodialysis (CVVHD) was performed using the PRISMA machine, with a blood flow of 100 ml/min and a dialysate flow of 2 L/h for 8 h, then 1 L/h. A 5% albumin dialysate was made by exchanging 1 L of 25% albumin for 1 L of Hemosol BO in a 5-L bag. Single pass albumin dialysis resulted in reductions in serum copper (154 to 59 microg/dL), conjugated bilirubin (37 to 23 mg/dL), lactate dehydrogenase (1305 to 729 units/L), and creatinine (1.1 to 0.9 mg/dL) as well as reduced blood transfusion requirements. Cessation of SPAD was followed by three plasmapheresis treatments for further copper removal. We conclude 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.

Single Pass Albumin Dialysis and Plasma Exchange for Copper Toxicity in Acute Wilson Disease

“…Many members of staff within intensive care units require little re-training, as they are already familiar with the setup for continuous venovenous hemodiafiltration. Several case reports have reported the efficacy of using SPAD as supportive treatment to successfully bridge patients with fulminant Wilsonian liver failure to liver transplantation [4, 9]. …”

“…Various methods reported to bridge to transplantation include single pass albumin dialysis (SPAD), plasma exchange (PLEX), and molecular adsorbent recirculating systems (MARS), but no consensus exists on the preferred treatment [4, 6, 9-14]. We report the effective use of SPAD alone and in combination with PLEX as a bridge to liver transplantation in 2 patients with fulminant WD.…”

“…Patients with fulminant WD present with liver failure, hepatic encephalopathy, and hemolytic anemia, the last a result of the direct toxicity of copper released from necrotic hepatocytes [4]. Therapy includes chelation with agents such as D-penicillamine and trientine to reduce serum copper levels [5], but this is often limited by the presence of acute kidney injury, which prevents adequate urinary copper removal by chelation.…”

Pharmaceutically Important Pre- and Posttranslational Modifications on Human Serum Albumin

“…Albumin-bound toxins are also important in the pathophysiology of systemic inflammatory response syndrome, and poisoning. Due to its intrinsic ability to bind molecules, albumin has been used in blood purification techniques such as single-pass albumin dialysis, 81,82) the molecular adsorbent recirculating system, 83,84) and the Prometheus systems. 85,86) The molecular adsorbent recirculating system of albumin dialysis is a high-flux dialysis setting using HSA as a molecular adsorbent to the dialysate with subsequent recirculation of the dialysate over sorbents.…”

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] .…”