Thrombotic thrombocytopenic purpura: incidence of congenital form of disease in north Moravia (region Moravia-Silesia)
Language English Country Czech Republic Media print
Document type Journal Article
PubMed
19655700
Knihovny.cz E-resources
- MeSH
- Adult MeSH
- Hemolytic-Uremic Syndrome epidemiology genetics MeSH
- Incidence MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Purpura, Thrombotic Thrombocytopenic congenital diagnosis epidemiology MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- Czech Republic epidemiology MeSH
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.
