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Article: Use of the molecular adsorbents recirculating system as a treatment for acute decompensated Wilson disease

TitleUse of the molecular adsorbents recirculating system as a treatment for acute decompensated Wilson disease
Authors
Issue Date2008
PublisherJohn Wiley & Sons, Inc. The Journal's web site is located at http://www3.interscience.wiley.com/cgi-bin/jtoc/106570021
Citation
Liver Transplantation, 2008, v. 14 n. 10, p. 1512-1516 How to Cite?
AbstractAcute decompensated Wilson disease presenting as fulminant liver failure is a life-threatening condition for which liver transplantation is the ultimate treatment. It is listed as a status 1 indication according to the United Network for Organ Sharing classification. A massive amount of copper released during the attack induces hemolytic anemia and acute renal failure. Conventional chelating therapy attempting to remove copper from the patient is not satisfactory because there is inadequate time for these drugs to take action and patients are usually oliguric. The Molecular Adsorbents Recirculating System (MARS) is a form of modified dialysis that removes putative albumin-bound toxins associated with liver failure. It is believed that extracorporeal albumin dialysate absorbs the circulating copper molecules that are trapped in the patient's circulation. We report 2 patients with acute decompensated Wilson disease treated with MARS. In the first case, the patient was started on MARS once conventional treatment failed. A significant amount of copper was removed from her circulatory system, and her condition stabilized afterwards. The treatment gained her extra time, and she was eventually bridged to liver transplantation. In the second case, the patient was started on MARS treatment early in the course of his illness, and his condition soon stabilized after the treatment. He was able to return to his home country for liver transplantation. In both cases, MARS was used as a means of preventing deterioration rather than salvaging devastation. In conclusion, MARS may confer benefits to patients with acute decompensated Wilson disease if it is started early in the course of illness. © 2008 AASLD.
Persistent Identifierhttp://hdl.handle.net/10722/59508
ISSN
2015 Impact Factor: 3.951
2015 SCImago Journal Rankings: 1.763
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorChiu, Aen_HK
dc.contributor.authorTsoi, NSen_HK
dc.contributor.authorFan, STen_HK
dc.date.accessioned2010-05-31T03:51:40Z-
dc.date.available2010-05-31T03:51:40Z-
dc.date.issued2008en_HK
dc.identifier.citationLiver Transplantation, 2008, v. 14 n. 10, p. 1512-1516en_HK
dc.identifier.issn1527-6465en_HK
dc.identifier.urihttp://hdl.handle.net/10722/59508-
dc.description.abstractAcute decompensated Wilson disease presenting as fulminant liver failure is a life-threatening condition for which liver transplantation is the ultimate treatment. It is listed as a status 1 indication according to the United Network for Organ Sharing classification. A massive amount of copper released during the attack induces hemolytic anemia and acute renal failure. Conventional chelating therapy attempting to remove copper from the patient is not satisfactory because there is inadequate time for these drugs to take action and patients are usually oliguric. The Molecular Adsorbents Recirculating System (MARS) is a form of modified dialysis that removes putative albumin-bound toxins associated with liver failure. It is believed that extracorporeal albumin dialysate absorbs the circulating copper molecules that are trapped in the patient's circulation. We report 2 patients with acute decompensated Wilson disease treated with MARS. In the first case, the patient was started on MARS once conventional treatment failed. A significant amount of copper was removed from her circulatory system, and her condition stabilized afterwards. The treatment gained her extra time, and she was eventually bridged to liver transplantation. In the second case, the patient was started on MARS treatment early in the course of his illness, and his condition soon stabilized after the treatment. He was able to return to his home country for liver transplantation. In both cases, MARS was used as a means of preventing deterioration rather than salvaging devastation. In conclusion, MARS may confer benefits to patients with acute decompensated Wilson disease if it is started early in the course of illness. © 2008 AASLD.en_HK
dc.languageengen_HK
dc.publisherJohn Wiley & Sons, Inc. The Journal's web site is located at http://www3.interscience.wiley.com/cgi-bin/jtoc/106570021en_HK
dc.relation.ispartofLiver Transplantationen_HK
dc.rightsLiver Transplantation. Copyright © John Wiley & Sons, Inc.en_HK
dc.titleUse of the molecular adsorbents recirculating system as a treatment for acute decompensated Wilson diseaseen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=1527-6465&volume=14&issue=10&spage=1512&epage=1516&date=2008&atitle=Use+of+the+molecular+adsorbents+recirculating+system+as+a+treatment+for+acute+decompensated+Wilson+diseaseen_HK
dc.identifier.emailFan, ST: stfan@hku.hken_HK
dc.identifier.authorityFan, ST=rp00355en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1002/lt.21553en_HK
dc.identifier.pmid18825711-
dc.identifier.scopuseid_2-s2.0-57849094542en_HK
dc.identifier.hkuros153722en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-57849094542&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume14en_HK
dc.identifier.issue10en_HK
dc.identifier.spage1512en_HK
dc.identifier.epage1516en_HK
dc.identifier.isiWOS:000259800300019-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridChiu, A=35721752900en_HK
dc.identifier.scopusauthoridTsoi, NS=6603693887en_HK
dc.identifier.scopusauthoridFan, ST=7402678224en_HK

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