Skip to main content
Top
Published in: Pediatric Nephrology 6/2008

01-06-2008 | Brief Report

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

Authors: Katherine L. Collins, Eve A. Roberts, Khosrow Adeli, Desmond Bohn, Elizabeth A. Harvey

Published in: Pediatric Nephrology | Issue 6/2008

Login to get access

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 μg/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.
Literature
2.
go back to reference Ferenci P (2004) Review article: diagnosis and current therapy of Wilson’s disease. Aliment Pharmacol Ther 19:157–165CrossRef Ferenci P (2004) Review article: diagnosis and current therapy of Wilson’s disease. Aliment Pharmacol Ther 19:157–165CrossRef
3.
go back to reference Roberts EA, Schilsky ML (2003) A practical guideline on Wilson disease. Hepatology 37:1475–1492CrossRef Roberts EA, Schilsky ML (2003) A practical guideline on Wilson disease. Hepatology 37:1475–1492CrossRef
4.
go back to reference Mitzner S, Klammt S, Stange J, Schmidt R (2006) Albumin regeneration in liver support—comparison of different methods. Ther Apher Dial 10:108–117CrossRef Mitzner S, Klammt S, Stange J, Schmidt R (2006) Albumin regeneration in liver support—comparison of different methods. Ther Apher Dial 10:108–117CrossRef
5.
go back to reference Karvellas CJ, Gibney N, Kutsogiannis D, Wendon J, Bain VG (2007) Bench-to-bedside review: Current evidence for extracorporeal albumin dialysis systems in liver failure. Crit Care 11:215–223CrossRef Karvellas CJ, Gibney N, Kutsogiannis D, Wendon J, Bain VG (2007) Bench-to-bedside review: Current evidence for extracorporeal albumin dialysis systems in liver failure. Crit Care 11:215–223CrossRef
6.
go back to reference Stange J, Hassanein TI, Mehta R, Mitzner SR, Bartlett RH (2002) The molecular adsorbents recycling system as a liver support system based on albumin dialysis: A summary of preclinical investigations, prospective, randomized controlled clinical trial, and clinical experience from 19 centers. Artif Organs 26:103–110CrossRef Stange J, Hassanein TI, Mehta R, Mitzner SR, Bartlett RH (2002) The molecular adsorbents recycling system as a liver support system based on albumin dialysis: A summary of preclinical investigations, prospective, randomized controlled clinical trial, and clinical experience from 19 centers. Artif Organs 26:103–110CrossRef
7.
go back to reference Kreymann B, Seige M, Schweigart U, Kopp K-F, Classen M (1999) 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 31:1080–1085CrossRef Kreymann B, Seige M, Schweigart U, Kopp K-F, Classen M (1999) 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 31:1080–1085CrossRef
8.
go back to reference Mitzner SR, Stange J, Klammt S, Risler T, Erley CM, Bader BD, Berger ED, Lauchart W, Peszynski P, Freytag J, Hickstein H, Loock J, Lohr J-M, Liebe S, Emmrich, J, Korten G, Schmidt R (2000) Improvement of hepatorenal syndrome with extracorporeal albumin dialysis MARS: Results of a prospective, randomized controlled clinical trial. Liver Transpl 6:277–286CrossRef Mitzner SR, Stange J, Klammt S, Risler T, Erley CM, Bader BD, Berger ED, Lauchart W, Peszynski P, Freytag J, Hickstein H, Loock J, Lohr J-M, Liebe S, Emmrich, J, Korten G, Schmidt R (2000) Improvement of hepatorenal syndrome with extracorporeal albumin dialysis MARS: Results of a prospective, randomized controlled clinical trial. Liver Transpl 6:277–286CrossRef
9.
go back to reference Sen S, Mookerjee RP, Davies NA, Williams R, Jalan R (2002) Review article: the molecular adsorbents recirculating system (MARS) in liver failure. Aliment Pharmacol Ther 16:32–38CrossRef Sen S, Mookerjee RP, Davies NA, Williams R, Jalan R (2002) Review article: the molecular adsorbents recirculating system (MARS) in liver failure. Aliment Pharmacol Ther 16:32–38CrossRef
10.
go back to reference Manz T, Ochs A, Bisse E, Strey C, Grotz W (2003) Liver support-a task for nephrologists? Extracorporeal treatment of a patient with fulminant Wilson crisis. Blood Purif 21:232–236CrossRef Manz T, Ochs A, Bisse E, Strey C, Grotz W (2003) Liver support-a task for nephrologists? Extracorporeal treatment of a patient with fulminant Wilson crisis. Blood Purif 21:232–236CrossRef
11.
go back to reference Sen S, Felldin M, Steiner C, Larsson B, Gillett GT, Olausson M, Williams R, Jalan R (2002) Albumin dialysis and molecular adsorbents recirculating system (MARS) for acute Wilson’s disease. Liver Transpl 8:962–967CrossRef Sen S, Felldin M, Steiner C, Larsson B, Gillett GT, Olausson M, Williams R, Jalan R (2002) Albumin dialysis and molecular adsorbents recirculating system (MARS) for acute Wilson’s disease. Liver Transpl 8:962–967CrossRef
12.
go back to reference Sauer IM, Goetz M, Steffen I, Walter G, Kehr DC, Schwartlander R, Hwang YJ, Pascher A, Gerlach JC, Neuhaus P (2004) In vitro comparison of the molecular adsorbent recirculation system (MARS) and Single-pass Albumin Dialysis (SPAD). Hepatology 39:1408–1414CrossRef Sauer IM, Goetz M, Steffen I, Walter G, Kehr DC, Schwartlander R, Hwang YJ, Pascher A, Gerlach JC, Neuhaus P (2004) In vitro comparison of the molecular adsorbent recirculation system (MARS) and Single-pass Albumin Dialysis (SPAD). Hepatology 39:1408–1414CrossRef
13.
go back to reference Askenazi DJ, Goldstein SL, Chang IF, Elenberg E, Feig DI (2004) Management of a severe carbamazepine overdose using albumin-enhanced continuous venovenous hemodialysis. Pediatrics 113:406–409CrossRef Askenazi DJ, Goldstein SL, Chang IF, Elenberg E, Feig DI (2004) Management of a severe carbamazepine overdose using albumin-enhanced continuous venovenous hemodialysis. Pediatrics 113:406–409CrossRef
14.
go back to reference Chawla LS, Georgescu F, Abell B, Seneff MG, Kimmel PL (2005) Modification of continuous venovenous hemodiafiltration with single-pass albumin dialysate allows for removal of serum bilirubin. Am J Kidney Dis 45:E51–E56CrossRef Chawla LS, Georgescu F, Abell B, Seneff MG, Kimmel PL (2005) Modification of continuous venovenous hemodiafiltration with single-pass albumin dialysate allows for removal of serum bilirubin. Am J Kidney Dis 45:E51–E56CrossRef
15.
go back to reference Peszynski P, Klammt S, Mitzner S, Stange J, Schmidt R (2002) Albumin dialysis: single pass vs. recirculation (MARS). Liver 22:40–42CrossRef Peszynski P, Klammt S, Mitzner S, Stange J, Schmidt R (2002) Albumin dialysis: single pass vs. recirculation (MARS). Liver 22:40–42CrossRef
16.
go back to reference Kiss JE, Berman D, Van Thiel D (1998) Effective removal of copper by plasma exchange in fulminant Wilson’s disease. Transfusion 38:327–331CrossRef Kiss JE, Berman D, Van Thiel D (1998) Effective removal of copper by plasma exchange in fulminant Wilson’s disease. Transfusion 38:327–331CrossRef
Metadata
Title
Single pass albumin dialysis (SPAD) in fulminant Wilsonian liver failure: a case report
Authors
Katherine L. Collins
Eve A. Roberts
Khosrow Adeli
Desmond Bohn
Elizabeth A. Harvey
Publication date
01-06-2008
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 6/2008
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-008-0761-x

Other articles of this Issue 6/2008

Pediatric Nephrology 6/2008 Go to the issue