| Thrombotic thrombocytopenic purpura: incidence of congenital form of disease in north Moravia (region Moravia-Silesia). | |
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MedLine Citation:
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PMID: 19655700 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Thrombotic thrombocytopenic purpura (TTP) was first described by Eli Moschcowitz in 1924. The pathophysiology of this disease is related to unusual, large multimers of von Willebrand factor in microcirculation, that are insufficiently cleaved by ADAMTS13 protease (a disintegrin-like and metalloprotease with thrombospondin type 1motif,13). Congenital TTP/Upshaw-Schulman syndrome is less frequent than acquired one TTP/HUS (haemolytic-ureamic syndrome). Short characteristic of patients with inherited form of TTP is reported as well as their clinical and laboratory features and management of treatment. |
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Authors:
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Z Cermáková; R Hrdliková; T Suláková; M Korístka; P Kovárová; I Hrachovinová |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Prague medical report Volume: 110 ISSN: 1214-6994 ISO Abbreviation: Prague Med Rep Publication Date: 2009 |
Date Detail:
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Created Date: 2009-08-06 Completed Date: 2009-09-02 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101227436 Medline TA: Prague Med Rep Country: Czech Republic |
Other Details:
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Languages: eng Pagination: 239-44 Citation Subset: IM |
Affiliation:
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Blood Center, Faculty Hospital Ostrava, 17. listopadu 1790, 708 52 Ostrava, Czech Republic. zuzana.cermakova@fnspo.cz |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Czech Republic / epidemiology Female Hemolytic-Uremic Syndrome / epidemiology, genetics Humans Incidence Male Purpura, Thrombotic Thrombocytopenic / congenital*, diagnosis, epidemiology Young Adult |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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