Effect of molecular adsorbents recirculating system treatment in children with acute liver failure caused by Wilson disease.
OBJECTIVE
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.
Najla
N
Rustom
Muriel
M
Bost
Fleur
F
Cour-Andlauer
Alain
A
Lachaux
Anne-Sophie
AS
Brunet
Olivier
O
Boillot
Fabienne
F
Bordet
Frederic
F
Valla
Nathalie
N
Richard
Etienne
E
Javouhey
D058186:Acute Kidney Injury / Q000097:blood / Q000209:etiology / Q000628:therapy; D000293:Adolescent; D000327:Adsorption; D000418:Albumins / Q000378:metabolism; D000641:Ammonia / Q000097:blood; D001663:Bilirubin / Q000097:blood; D002648:Child; D003300:Copper / Q000097:blood*; D003404:Creatinine / Q000097:blood; D005260:Female; D006501:Hepatic Encephalopathy / Q000209:etiology / Q000628:therapy*; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000628:therapy*; D006801:Humans; D008099:Liver / Q000473:pathology / Q000601:surgery; D017114:Liver Failure, Acute / Q000097:blood / Q000209:etiology / Q000601:surgery / Q000628:therapy*; D008111:Liver Function Tests; D016031:Liver Transplantation; D008297:Male; D006435:Renal Dialysis / Q000379:methods; D012189:Retrospective Studies
D000418:Albumins; D000641:Ammonia; D003300:Copper; D003404:Creatinine; D001663:Bilirubin