Genetic diagnosis of TMA: TTP and aHUS
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چکیده
منابع مشابه
Biomarkers of terminal complement activation confirm the diagnosis of aHUS and differentiate aHUS from TTP.
Atypical hemolytic uremic syndrome (aHUS) is characterized by dysregulated complement activity, the development of a thrombotic microangiopathy (TMA), and widespread end organ injury. aHUS remains a clinical diagnosis without an objective laboratory test to confirm the diagnosis. We performed a retrospective analysis of 103 patients enrolled in the Ohio State University TTP/aHUS Registry presen...
متن کاملCOMPLEMENTing the diagnosis of aHUS.
In this issue of Blood, Noris et al describe a functional complement assay that can be used to diagnose atypical hemolytic-uremic syndrome (aHUS). This assay might also be useful in identifying aHUS patients in remission and in detecting asymptomatic carriers of complement gene mutations. Besides being used for diagnostic purposes, this assay potentially can be used to monitor anticomplement th...
متن کاملAtypical hemolytic uremic syndrome (aHUS): making the diagnosis.
Atypical hemolytic uremic syndrome (aHUS) is a major thrombotic microangiopathy (TMA). A TMA is recognized by the laboratory signs of microangiopathic hemolysis, as indicated by schistocytes, elevated lactate dehydrogenase, low haptoglobin, and low hemoglobin, plus thrombocytopenia and accompanying signs and symptoms of organ system involvement. aHUS results from chronic, uncontrolled activity ...
متن کاملAtypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis.
Atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA), is a rare, life-threatening, systemic disease. When unrecognized or inappropriately treated, aHUS has a high degree of morbidity and mortality. aHUS results from chronic, uncontrolled activity of the alternative complement pathway, which activates platelets and damages the endothelium. Two-thirds of aHUS cases are as...
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ژورنال
عنوان ژورنال: Japanese Journal of Thrombosis and Hemostasis
سال: 2020
ISSN: 0915-7441,1880-8808
DOI: 10.2491/jjsth.31.17