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Hemolytiskt uremiskt syndrom och trombotisk trombocytopen purpura. Nya rön om EHEC, komplementmutationer och ADAMTS13.

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Název: Hemolytiskt uremiskt syndrom och trombotisk trombocytopen purpura. Nya rön om EHEC, komplementmutationer och ADAMTS13.
Autoři: Karpman, Diana
Zdroj: Läkartidningen; 105(15), pp 1096-1101 (2008) ; ISSN: 0023-7205
Informace o vydavateli: Swedish Medical Association
Rok vydání: 2008
Sbírka: Lund University Publications (LUP)
Témata: Pediatrics, ADAM Proteins: blood, ADAM Proteins: genetics, ADAM Proteins: immunology, Biological Markers: blood, Complement System Proteins: genetics, Enterohemorrhagic Escherichia coli: pathogenicity, Hemolytic-Uremic Syndrome: diagnosis, Hemolytic-Uremic Syndrome: etiology, Hemolytic-Uremic Syndrome: therapy, Kidney Cortex: pathology, Purpura, Thrombotic Thrombocytopenic: diagnosis, Thrombotic Thrombocytopenic: etiology, ADAM Proteins: deficiency, Thrombotic Thrombocytopenic: therapy, Thrombosis: diagnosis, Thrombosis: etiology
Popis: Thrombotic microangiopathy is the pathological lesion found in hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) as well as other conditions. It manifests as damaged endothelial cells in small vessels with deposits of platelet plugs, occlusion of vessels, decreased blood flow and secondary organ insufficiency. HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It may be associated with gastrointestinal infection with Shiga toxin-producing bacteria (enterohemorrhagic Escherichia coli or Shigella dysenteriae) causing a prodrome of diarrhea followed in some cases by HUS. Atypical HUS may be hereditary and recurrent, and may, in certain cases, be associated with dysregulation of the alternative pathway of complement. TTP is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, renal and CNS manifestations. It has been associated with deficient activity of ADAMTS13, the von Willebrand factor cleaving protease. This review will focus on the classification, symptoms, pathology, pathogenesis, investigation, management and treatment of these conditions.
Druh dokumentu: article in journal/newspaper
Jazyk: Swedish
Relation: https://lup.lub.lu.se/record/1168721; pmid:18561752; scopus:42649137892
Dostupnost: https://lup.lub.lu.se/record/1168721
Přístupové číslo: edsbas.4F9F09D3
Databáze: BASE
Popis
Abstrakt:Thrombotic microangiopathy is the pathological lesion found in hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) as well as other conditions. It manifests as damaged endothelial cells in small vessels with deposits of platelet plugs, occlusion of vessels, decreased blood flow and secondary organ insufficiency. HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It may be associated with gastrointestinal infection with Shiga toxin-producing bacteria (enterohemorrhagic Escherichia coli or Shigella dysenteriae) causing a prodrome of diarrhea followed in some cases by HUS. Atypical HUS may be hereditary and recurrent, and may, in certain cases, be associated with dysregulation of the alternative pathway of complement. TTP is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, renal and CNS manifestations. It has been associated with deficient activity of ADAMTS13, the von Willebrand factor cleaving protease. This review will focus on the classification, symptoms, pathology, pathogenesis, investigation, management and treatment of these conditions.