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Effect of molecular adsorbents recirculating system treatment in children with acute liver failure caused by Wilson
Najla Rustom1, Muriel Bost, Fleur Cour-Andlauer
1*Department of Pediatric Gastroenterology, Hepatology and Nutrition, Hôpital Femme Mère Enfant, Hospices Civils de Lyon †Laboratory of Trace Element Analysis, Biochemistry and Molecular Biology, Hôpital Edouard Herriot ‡Pediatric Intensive Care Unit, Hôpital Femme Mère Enfant, Hospices Civils de Lyon §Division of Digestive Surgery and Liver Transplantation Unit, Edouard Herriot Hospital, Lyon, France.
Insights
Molecular Adsorbents and Recirculating System (MARS) therapy effectively removes copper and stabilizes children with acute liver failure from Wilson disease, bridging them to liver transplantation.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Nephrology
Background:
- Fulminant Wilson disease (WD) presents a critical challenge with a poor prognosis.
- Liver support systems are crucial for bridging patients to liver transplantation.
- Albumin dialysis using MARS is a potential life-saving intervention for acute liver failure (ALF) in children with WD.
Purpose of the Study:
- To evaluate the efficacy and safety of albumin dialysis with the Molecular Adsorbents and Recirculating System (MARS) in pediatric patients with ALF secondary to acute WD.
- To assess MARS's ability to remove copper and improve clinical and biological parameters in these patients.
Main Methods:
- Retrospective chart review of children admitted for ALF due to acute WD treated with MARS.
- Monitoring of blood and circuit copper levels during MARS sessions.
- Clinical and biological assessments before and after MARS treatment.
Main Results:
- Four children (median age 12.3 years) with severe ALF, acute renal failure, hemolysis, and cholestasis underwent 14 MARS sessions (median 7.5 hours).
- MARS successfully removed copper, reducing serum copper levels by a mean of 28%.
- Significant improvements were observed in neurological status, Fisher index, ammonia, bilirubin, and creatinine levels (>25% reduction).
Conclusions:
- MARS therapy is effective in removing copper and stabilizing children with ALF secondary to WD.
- This liver support system serves as a vital bridge to liver transplantation (LT) in pediatric WD patients.
- MARS facilitates successful liver transplantation with good outcomes and no long-term disability.
Objectives:
Because fulminant Wilson disease (WD) has an extremely poor prognosis, the use of liver support that can bridge patients to liver transplantation is lifesaving. We report the experience of albumin dialysis in acute liver failure (ALF) caused by WD in children.
Methods:
Chart review of children admitted for ALF secondary to acute WD and treated by the molecular adsorbents and recirculating system. Measures of copper level in blood and within the circuit during molecular adsorbents recirculating system (MARS) sessions were performed. Clinical and biological assessments after MARS session were reported.
Results:
Four children, with a median age of 12.3 years, were treated from 2004 to 2009 for a severe ALF associated with acute renal failure, haemolysis, and severe cholestasis. All of the children had a new Wilson index >12. A total of 14 MARS sessions were performed, for a median duration of 7.5 hours. Tolerance was good, except for 1 child who experienced haemorrhage because of vascular injury following insertion of the dialysis catheter. A neurological improvement or stabilisation was noted in all of the children along with an improvement in the Fisher index and ammonia level after MARS treatment. MARS was able to remove copper, to decrease the serum copper level of 28% in mean, and to decrease the bilirubin and creatinin levels >25%. All of the children were subsequently underwent liver transplants with a good outcome without disability.
Conclusions:
MARS is able to remove copper and to stabilise children with ALF secondary to WD, allowing bridging to LT.
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