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

摘要: Thrombotic microangiopathy is the pathological lesion found in hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) as well other conditions. It manifests damaged endothelial cells small vessels with deposits of platelet plugs, occlusion vessels, decreased blood flow and secondary organ insufficiency. HUS characterized by microangiopathic anemia, thrombocytopenia acute renal failure. may be associated gastrointestinal infection Shiga toxin-producing bacteria (enterohemorrhagic Escherichia coli or Shigella dysenteriae) causing a prodrome diarrhea followed some cases HUS. Atypical hereditary recurrent, may, certain cases, dysregulation alternative pathway complement. TTP thrombocytopenia, fever, CNS manifestations. has been deficient activity ADAMTS13, von Willebrand factor cleaving protease. This review will focus on classification, symptoms, pathology, pathogenesis, investigation, management treatment these (Less)