title: 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.
authors.affiliation: Department of Paediatrics, The Hospital for Sick Children and the University of Toronto, Toronto, Canada.
authors.forename: Katherine L
authors.initials: KL
authors.lastname: Collins
authors.forename: Eve A
authors.initials: EA
authors.lastname: Roberts
authors.forename: Khosrow
authors.initials: K
authors.lastname: Adeli
authors.forename: Desmond
authors.initials: D
authors.lastname: Bohn
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Harvey
mesh_terms: D000293:Adolescent; D002614:Chelating Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003300:Copper / Q000097:blood*; D005260:Female; D006440:Hemofiltration; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000378:metabolism / Q000628:therapy*; D006801:Humans; D017114:Liver Failure, Acute / Q000209:etiology / Q000378:metabolism / Q000628:therapy*; D016031:Liver Transplantation; D010956:Plasmapheresis; D011485:Protein Binding; D006435:Renal Dialysis / Q000379:methods*; D012709:Serum Albumin / Q000378:metabolism*; D016896:Treatment Outcome
chemical_list: D002614:Chelating Agents; D012709:Serum Albumin; D003300:Copper