2014JSN-JPS非典型溶血性尿毒癥綜合征的診斷標準
發(fā)布時間:2017-09-28 14:34:41    瀏覽量: 995
關鍵字:ADAMTS13,補體替代途徑,非典型溶血尿毒綜合征,配合失調,血栓病微血管病變,診斷標準,alternative complement pathway,atypical hemolytic urem
簡介:

Atypical hemolytic uremic syndrome (aHUS) is rare and comprises the triad of microangiopathic hemolytic anemia,thrombocytopenia, and acute kidney injury. Recently, abnormalities in the mechanisms underlying complement regu-lation have been focused upon as causes of aHUS. The prognosis for patients who present with aHUS is very poor, with the first aHUS attack being associated with a mortality rate of approximately 25%, and with approximately 50% of cases resulting in end-stage renal disease requiring dialysis. If treatment is delayed, there is a high risk of this syndrome progressing to renal failure. Therefore, we have developed diagnostic criteria for aHUS to enable its early diagnosis and to facilitate the timely initiation of appropriate treatment. We hope these diagnostic criteria will be disseminated to as many clinicians as possible and that they will be used widely.


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