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首页> 外文期刊>Journal of International Medical Research >Atypical hemolytic uremic syndrome in a patient with protein-losing enteropathy
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Atypical hemolytic uremic syndrome in a patient with protein-losing enteropathy

机译:蛋白质丢失性肠病患者的非典型溶血性尿毒症综合征

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Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease induced by many triggers, all of which produce a common end phenotype of microangiopathic hemolysis and thrombotic microangiopathy. We herein describe a 63-year-old woman with ongoing protein-losing enteropathy and frequent transudates caused by hypoalbuminemia. The patient was treated with eculizumab with a full hematologic and partial renal response. Protein-losing enteropathy is an inflammatory condition that has been linked with increased complement activation, which can trigger aHUS in patients with loss of CD55 expression. The patient in the present case had an increased estimated glomerular filtration rate but stage IV to V chronic kidney disease. One year later, she remains off dialysis with a stable estimated glomerular filtration rate. We herein report an unusual trigger of complement activation that in turn triggered aHUS in this patient.
机译:非典型溶血性尿毒症综合征(aHUS)是一种由多种触发因素诱发的超罕见疾病,所有触发因素都会产生微血管病性溶血和血栓性微血管病的常见终末表型。我们在此描述了一名63岁的女性,患有持续性的蛋白质丢失性肠病和由低白蛋白血症引起的频繁渗出液。该患者接受依库丽单抗治疗,具有完全的血液学和部分肾脏反应。蛋白质丢失性肠病是一种炎症性疾病,与补体激活增加有关,可在CD55表达丧失的患者中触发aHUS。在本例中,该患者的肾小球滤过率估计升高,但处于IV至V期慢性肾脏疾病。一年后,她仍处于透析状态,估计肾小球滤过率稳定。我们在此报告了补体激活的异常触发,进而触发了该患者的aHUS。

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