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- W3029498159 abstract "Thrombotic microangiopathy(TMA) is a group of clinical and pathological syndromes, characterized mainly by hemolytic anemia, thrombocytopenia, and multiple organ dysfunctions caused by a variety of factors.Classic types of TMA include hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura(TTP). HUS is a critically ill glomerular disease.Depending on the pathogenesis, HUS is currently divided into 2 categories, namely typical and atypical HUS.Atypical HUS is at a rapid onset and is a type of dangerous disease, which tends to recur easily, and has high mortality in its acute phase.TTP is a rare but fatal entity of TMA.The pathophysiology of the disease is based on a severe functional deficiency of a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13(ADAMTS-13), the specific von Willebrand factor(VWF)-cleavage protease.This deficiency may be either acquired or congenital.The activity of plasma ADAMTS-13<10%is the only definitive method for diagnosing TTP.This article reports the recent advances on the diagnosis and treatment of severe TMA in children.Key words: Thrombotic microangiopathy; Hemolytic uremic syndrome; Thrombotic thrombocytopenic purpura; Diagnosis; Treatment; Child" @default.
- W3029498159 created "2020-06-05" @default.
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- W3029498159 date "2019-09-20" @default.
- W3029498159 modified "2023-10-03" @default.
- W3029498159 title "Diagnosis and treatment of severe thrombotic microangiopathy in children" @default.
- W3029498159 doi "https://doi.org/10.3760/cma.j.issn.2095-428x.2019.18.004" @default.
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