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- W2150140043 abstract "Abstract The thrombotic microangiopathies are a group of conditions characterized by microvascular bed thrombosis, haemolytic anaemia and thrombocytopenia. Renal involvement, due to occlusion of glomerular capillaries and renal arterioles by platelet-fibrin thrombi, is a common though not universal finding. Haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are examples of thrombotic microangiopathies that illustrate learning points for the diagnosis, pathogenesis, management and prognosis for this group of conditions. There have been significant advances in understanding the separate aetiologies of these conditions in the last 10 years, which will increasingly influence treatment. HUS has prominent renal involvement and is classified into two major subtypes. Diarrhoeal HUS (D+ HUS) is associated with a preceding diarrhoeal illness, commonly caused by Escherichia coli O157:H7. Atypical HUS (aHUS) is associated with mutations in proteins of the alternative pathway of complement, particularly complement factor H. TTP has prominent neurological involvement and is caused by an inherited or acquired deficiency of the von Willebrand factor-cleaving metalloprotease, ADAMTS13. Rapid instigation of plasma exchange is the mainstay of treatment for TTP and aHUS and has significantly improved survival. Genetic testing is available in the UK to guide counselling and management decisions in those with a positive family history." @default.
- W2150140043 created "2016-06-24" @default.
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- W2150140043 date "2011-08-01" @default.
- W2150140043 modified "2023-09-27" @default.
- W2150140043 title "Thrombotic microangiopathies and the kidney" @default.
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- W2150140043 doi "https://doi.org/10.1016/j.mpmed.2011.05.011" @default.
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