Single pass albumin dialysis (SPAD) in fulminant Wilsonian liver failure: a case report
- 1 June 2008
- journal article
- case report
- Published by Springer Nature in Pediatric Nephrology
- Vol. 23 (6) , 1013-1016
- https://doi.org/10.1007/s00467-008-0761-x
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.Keywords
This publication has 17 references indexed in Scilit:
- Bench-to-bedside review: Current evidence for extracorporeal albumin dialysis systems in liver failureCritical Care, 2007
- Albumin Regeneration in Liver Support—Comparison of Different MethodsTherapeutic Apheresis and Dialysis, 2006
- Modification of continuous venovenous hemodiafiltration with single-pass albumin dialysate allows for removal of serum bilirubinAmerican Journal of Kidney Diseases, 2005
- In vitro comparison of the molecular adsorbent recirculation system (MARS) and single-pass albumin dialysis (SPAD)Hepatology, 2004
- Diagnosis and current therapy of Wilson's diseaseAlimentary Pharmacology & Therapeutics, 2004
- Wilson diseaseGastroenterology, 2003
- A Practice Guideline on Wilson DiseaseHepatology, 2003
- Albumin dialysis and molecular adsorbents recirculating system (MARS) for acute Wilson’s diseaseLiver Transplantation, 2002
- Improvement of hepatorenal syndrome with extracorporeal albumin dialysis mars: Results of a prospective, randomized, controlled clinical trialLiver Transplantation, 2000
- Effective removal of copper by plasma exchange in fulminant Wilson's diseaseTransfusion, 1998