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溃疡性结肠炎与非典型溶血尿毒综合征:一种不常见但可能危及生命的并发症。

Ulcerative Colitis and Atypical Hemolytic-Uremic Syndrome: An Unusual But Potentially Life-threatening Complication.

机构信息

Department of Gastroenterology and Nutrition, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

Department of Nephrology, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

出版信息

Inflamm Bowel Dis. 2019 Mar 14;25(4):e27-e28. doi: 10.1093/ibd/izy226.

Abstract

Hemolytic-uremic syndrome (HUS) is defined as the triad of nonimmune hemolytic anemia, thrombocytopenia, and acute renal failure, in which the underlying lesions are mediated by systemic thrombotic microangiopathy (TMA). The atypical HUS (aHUS) can be considered a subtype of HUS that is rare in childhood and has a worse prognosis. Recent findings have established that the TMA in aHUS are consequences of the disregulation of the complement activation, leading to endotelial damage mediated by the complement terminal pathway.1, 2 Likewise, previous research suggests an important role for the deregulation of the alternative complement cascade in the pathogenesis of inflammatory bowel disease (IBD).3, 4 We report the case of a patient with ulcerative colitis (UC) who developed aHUS during a flare-up of her chronic disease. This association is extremely infrequent and had been previously reported in only 1 patient.5.

摘要

溶血尿毒综合征(HUS)定义为非免疫性溶血性贫血、血小板减少和急性肾衰竭三联征,其潜在病变由全身性血栓性微血管病(TMA)介导。非典型 HUS(aHUS)可被视为儿童中罕见且预后较差的 HUS 亚型。最近的研究发现,aHUS 中的 TMA 是补体激活失调的结果,导致补体末端途径介导的内皮损伤。1,2 同样,先前的研究表明,补体替代途径的失调在炎症性肠病(IBD)的发病机制中起着重要作用。3,4 我们报告了一例溃疡性结肠炎(UC)患者在慢性疾病发作期间发生 aHUS 的病例。这种关联非常罕见,之前仅在 1 例患者中报告过。5.

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