| Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008. | |
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MedLine Citation:
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PMID: 20045995 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Severe deficiency of plasma ADAMTS13 activity (ADAMTS13: AC) is more specific for TTP, but not for HUS. Since 1998, our laboratory has functioned as a nationwide referral center for TMAs by analyzing ADAMTS13. METHODS: Of 1,564 patients tested from 426 hospitals, 919 were positive for TMA. Levels of ADAMTS13: AC and the ADAMTS13 neutralizing autoantibody (ADAMTS13: INH) were determined by chromogenic act-ELISA and/or by classic von Willebrand factor multimer assay. RESULTS: TMA patients consisted of two groups: severe (less than 3% of normal control) and non-severe deficiency of ADAMTS13: AC. Both groups were divided into congenital (n=65) and acquired (n=854) TMA. Of the former, 41 had congenital deficiency of ADAMTS13: AC, while the remaining 24 had disease of unknown etiology. The 854 patients with acquired TMA could be largely grouped into three categories: idiopathic TTP (n=284), idiopathic HUS (n=106), and secondary TMAs (n=464). The secondary TMAs were observed in heterogeneous patient groups and were associated with drugs, connective tissue diseases, malignancies, transplantation, pregnancy, E. coli O157: H7 infection, and other factors. All of the patients with acquired severe ADAMTS13: AC deficiency were positive for ADAMTS13: INH. CONCLUSION: Although TMAs are highly heterogeneous pathological conditions, one-third of TMA patients have severe deficiency of ADAMTS13: AC. Platelet transfusions to such patients are contraindicated. Rapid ADAMTS13: AC assays are therefore prerequisite to appropriately treat TMA patients. |
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Authors:
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Yoshihiro Fujimura; Masanori Matsumoto |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-01-01 |
Journal Detail:
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Title: Internal medicine (Tokyo, Japan) Volume: 49 ISSN: 1349-7235 ISO Abbreviation: Intern. Med. Publication Date: 2010 |
Date Detail:
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Created Date: 2010-01-04 Completed Date: 2010-04-22 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9204241 Medline TA: Intern Med Country: Japan |
Other Details:
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Languages: eng Pagination: 7-15 Citation Subset: IM |
Affiliation:
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Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan. malon@naramed-u.ac.jp |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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ADAM Proteins
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deficiency* Algorithms Hemolytic-Uremic Syndrome / blood, diagnosis* Humans Japan Purpura, Thrombotic Thrombocytopenic / blood, diagnosis* Registries* |
| Chemical | |
Reg. No./Substance:
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EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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