Hanna Ramy M, Hasnain Huma, Abdelnour Lama, Yanny Beshoy, Burwick Richard M
1 Division of Nephrology, Department of Medicine, UCLA David Geffen School of Medicine, Los Angeles, CA, United States.
2 Department of Medicine, Division of Digestive Diseases, Hepatology UCLA David Geffen School of Medicine, Los Angeles, CA, United States.
J Int Med Res. 2019 Aug;47(8):4027-4032. doi: 10.1177/0300060519864808. Epub 2019 Jul 31.
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.
非典型溶血性尿毒症综合征（aHUS）是一种由多种触发因素引起的超罕见疾病，所有这些因素都会产生微血管病性溶血和血栓性微血管病这一共同的终末表型。我们在此描述一名63岁女性，她患有持续性蛋白丢失性肠病且因低白蛋白血症频繁出现漏出液。该患者接受了依库珠单抗治疗，血液学方面完全缓解，肾脏方面部分缓解。蛋白丢失性肠病是一种炎症性疾病，与补体激活增加有关，补体激活增加可在CD55表达缺失的患者中引发aHUS。本例患者的估计肾小球滤过率升高，但处于IV至V期慢性肾脏病。一年后，她仍未进行透析，估计肾小球滤过率稳定。我们在此报告了一种不寻常的补体激活触发因素，该因素进而在该患者中引发了aHUS。
