Abstract:
BACKGROUND:Wilson's disease (WD) can present in a fulminant form with hepatocellular dysfunction, hemolysis and multiorgan failure (Wilson's crisis). We present a previously healthy young woman with severe WD whose WD severity score was 13. A score >11 indicates a poor chance of survival and liver transplantation will usually be recommended. METHODS:Penicillamine and acetylcysteine were initially administered, but the patient deteriorated further, and extracorporeal liver support with the Prometheus FPSA (fractionated plasma separation and adsorption) system was initiated. The patient was treated 6 h daily during 3 consecutive days. RESULTS:Severe hemolysis was reduced to low-grade hemolysis, with no further need for transfusions. The mental state improved and after 4 months practically all biochemical markers were normalized. CONCLUSIONS:This is the first report of FPSA albumin dialysis of a patient with Wilson's crisis and the first report in which a patient with a WD score >11 survived without transplantation.
journal_name
Blood Purifjournal_title
Blood purificationauthors
Aagaard NK,Thomsen KL,Holland-Fischer P,Jørgensen SP,Ott Pdoi
10.1159/000218090subject
Has Abstractpub_date
2009-01-01 00:00:00pages
102-7issue
2eissn
0253-5068issn
1421-9735pii
000218090journal_volume
28pub_type
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pub_type: 杂志文章,评审
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pub_type: 临床试验,杂志文章,多中心研究
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pub_type: 杂志文章,多中心研究
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journal_title:Blood purification
pub_type: 临床试验,杂志文章
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pub_type: 临床试验,杂志文章
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journal_title:Blood purification
pub_type: 杂志文章,评审
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pub_type: 临床试验,杂志文章
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journal_title:Blood purification
pub_type: 杂志文章,评审
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pub_type: 杂志文章,meta分析,评审
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pub_type: 杂志文章,多中心研究
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pub_type: 杂志文章
doi:10.1159/000113009
更新日期:2007-01-01 00:00:00
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journal_title:Blood purification
pub_type: 临床试验,杂志文章
doi:10.1159/000070696
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journal_title:Blood purification
pub_type:
doi:10.1159/000170132
更新日期:1993-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1159/000322622
更新日期:2011-01-01 00:00:00
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journal_title:Blood purification
pub_type: 杂志文章
doi:10.1159/000170015
更新日期:1991-01-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2010-01-01 00:00:00
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pub_type: 杂志文章,随机对照试验
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pub_type: 杂志文章
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更新日期:1998-01-01 00:00:00
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pub_type: 杂志文章
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更新日期:2013-01-01 00:00:00
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pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:1988-01-01 00:00:00
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journal_title:Blood purification
pub_type: 杂志文章
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更新日期:2019-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1159/000169604
更新日期:1989-01-01 00:00:00
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pub_type: 杂志文章,多中心研究
doi:10.1159/000441270
更新日期:2016-01-01 00:00:00
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pub_type: 杂志文章
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更新日期:2020-01-01 00:00:00
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