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Effect of Molecular Adsorbents Recirculating System Treatment in Children With Acute Liver Failure Caused by Wilson Disease

Rustom, Najla*; Bost, Muriel; Cour-Andlauer, Fleur; Lachaux, Alain*; Brunet, Anne-Sophie*; Boillot, Olivier§; Bordet, Fabienne; Valla, Frederic; Richard, Nathalie; Javouhey, Etienne

Journal of Pediatric Gastroenterology and Nutrition: February 2014 - Volume 58 - Issue 2 - p 160–164
doi: 10.1097/MPG.0b013e3182a853a3
Original Articles: Hepatology and Nutrition

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.

*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.

Address correspondence and reprint requests to Dr Najla Rustom, Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Hôpital Femme Mère Enfant, Groupement Hospitalier Est, 59 Boulevard Pinel, 69500 Bron, France (e-mail:

Received 6 August, 2013

Accepted 6 August, 2013

The authors report no conflicts of interest.

© 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,