Source:http://linkedlifedata.com/resource/pubmed/id/18521503
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
6
|
| pubmed:dateCreated |
2008-6-3
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| pubmed:abstractText |
Decreased plasma ADAMTS13 activity (ADAMTS13:AC) results in the accumulation of unusually large von Willebrand factor multimer (UL-VWFM) and the formation of platelet thrombi. It remains controversial whether or not plasma ADAMTS13:AC decreases in patients with liver cirrhosis (LC), and its relationship to clinical features has not been fully investigated. We measured ADAMTS13:AC and its related parameters in plasma in 33 patients with chronic hepatitis (CH) and in 109 patients with LC. ADAMTS13:AC decreased with increasing severity of liver disease (controls means 100%, CH 87%, Child A-LC 79%, Child B-LC 63%, and Child C-LC 31%), and showed severe deficiency (<3% of controls) in five end-stage LC. Activities measured by act-ELISA strongly correlated with those determined by the VWFM assay and ADAMTS13 antigen. Multivariate analysis showed Child-Pugh score and spleen volume independent factors contributing to ADAMTS13:AC. VWFM patterns were normal in 53% of cases, degraded in 31%, and unusually large in 16%. Patients with unusually large VWFM had the lowest ADAMTS13:AC as well as the highest Child-Pugh score, serum creatinine and blood ammonia levels. Plasma inhibitor against ADAMTS13 detected in 83% of patients with severe to moderate ADAMTS13:AC deficiency mostly showed marginal zone between 0.5 and 1.0 BU/ml. The IgG-type autoantibodies specific to plasma derived-ADAMTS13 was detected by Western blot in only five end-stage LC with severe ADAMTS13:AC deficiency. In conclusion, both plasma ADAMTS13 activity and antigen levels decreased with increasing severity of cirrhosis. An imbalance between the decreased ADAMTS13:AC and its increased substrate may reflect the predisposing state for platelet thrombi formation in patients with advanced LC.
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| pubmed:commentsCorrections | |
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/ADAM Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/ADAMTS13 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Autoantibodies,
http://linkedlifedata.com/resource/pubmed/chemical/Cytokines,
http://linkedlifedata.com/resource/pubmed/chemical/von Willebrand Factor
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| pubmed:status |
MEDLINE
|
| pubmed:month |
Jun
|
| pubmed:issn |
0340-6245
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| pubmed:author |
pubmed-author:FujimuraYoshihiroY,
pubmed-author:FukuiHiroshiH,
pubmed-author:IshikawaMasatoshiM,
pubmed-author:IshizashiHiromichiH,
pubmed-author:IsonishiAyamiA,
pubmed-author:KatoSeijiS,
pubmed-author:KurumataniNorioN,
pubmed-author:MatsumotoMasanoriM,
pubmed-author:MatsuyamaTomomiT,
pubmed-author:MoriokaChieC,
pubmed-author:TsujimotoTatsuhiroT,
pubmed-author:UemuraMasahitoM,
pubmed-author:YagitaMasatoM,
pubmed-author:YoshijiHitoshiH
|
| pubmed:issnType |
Print
|
| pubmed:volume |
99
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
1019-29
|
| pubmed:meshHeading |
pubmed-meshheading:18521503-ADAM Proteins,
pubmed-meshheading:18521503-Aged,
pubmed-meshheading:18521503-Autoantibodies,
pubmed-meshheading:18521503-Blotting, Western,
pubmed-meshheading:18521503-Cytokines,
pubmed-meshheading:18521503-Down-Regulation,
pubmed-meshheading:18521503-Enzyme-Linked Immunosorbent Assay,
pubmed-meshheading:18521503-Female,
pubmed-meshheading:18521503-Hepatitis B, Chronic,
pubmed-meshheading:18521503-Hepatitis C, Chronic,
pubmed-meshheading:18521503-Humans,
pubmed-meshheading:18521503-Liver Cirrhosis,
pubmed-meshheading:18521503-Male,
pubmed-meshheading:18521503-Middle Aged,
pubmed-meshheading:18521503-Platelet Count,
pubmed-meshheading:18521503-Purpura, Thrombotic Thrombocytopenic,
pubmed-meshheading:18521503-Severity of Illness Index,
pubmed-meshheading:18521503-Thrombosis,
pubmed-meshheading:18521503-von Willebrand Factor
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| pubmed:year |
2008
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| pubmed:articleTitle |
Comprehensive analysis of ADAMTS13 in patients with liver cirrhosis.
|
| pubmed:affiliation |
Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan. muemura@naramed-u.ac.jp
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| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|