Mütiş Alan Aydan, Dinçer Mevlüt Tamer, Kaykioğlu Ata, Türk Bahar, Nuhoğlu Kantarci Eda, Seyahi Nurhan, Trabulus Sinan, Eşkazan Ahmet Emre
Division of Nephrology, Department of Internal Medicine, Istanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Istanbul, Turkey.
Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Turkey.
Nephron. 2025;149(9):539-544. doi: 10.1159/000546526. Epub 2025 May 23.
Atypical hemolytic uremic syndrome (aHUS) is a rare, complement-mediated disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Eculizumab, a monoclonal antibody that inhibits complement component C5, is a cornerstone therapy for aHUS but increases the risk of infections, particularly from encapsulated organisms. Surgical procedures can also raise infection risks or exacerbate thrombotic microangiopathy. However, data on the perioperative management of patients with aHUS, particularly those receiving eculizumab, remain limited.
A 73-year-old male with a history of prostate cancer presented with acute kidney injury, thrombocytopenia, and hemolysis, leading to a diagnosis of aHUS. He was treated with eculizumab, which improved kidney function and eliminated the need for dialysis. Four months later, he developed abdominal pain and was found to have gallstones. The patient subsequently underwent a laparoscopic cholecystectomy while continuing maintenance eculizumab therapy. The procedure was performed without any complications, and the patient was discharged in stable condition.
This case report details the successful perioperative management of a patient with aHUS on maintenance eculizumab undergoing cholecystectomy, highlighting the importance of careful management, including continued complement inhibition and infection prevention strategies. Our report emphasizes the need for individualized perioperative care to minimize risks in aHUS patients requiring surgery.
非典型溶血性尿毒症综合征(aHUS)是一种罕见的、由补体介导的疾病,其特征为微血管病性溶血性贫血、血小板减少和急性肾损伤。依库珠单抗是一种抑制补体成分C5的单克隆抗体,是aHUS的基石疗法,但会增加感染风险,尤其是来自包膜菌的感染风险。外科手术也会增加感染风险或加重血栓性微血管病。然而,关于aHUS患者围手术期管理的数据,尤其是接受依库珠单抗治疗的患者的数据仍然有限。
一名73岁有前列腺癌病史的男性出现急性肾损伤、血小板减少和溶血,最终诊断为aHUS。他接受了依库珠单抗治疗,肾功能得到改善,不再需要透析。四个月后,他出现腹痛,被发现患有胆结石。该患者随后在继续维持依库珠单抗治疗的同时接受了腹腔镜胆囊切除术。手术过程中没有出现任何并发症,患者出院时情况稳定。
本病例报告详细介绍了一名接受维持性依库珠单抗治疗的aHUS患者在进行胆囊切除术时围手术期管理的成功经验,强调了包括持续补体抑制和感染预防策略在内的谨慎管理的重要性。我们的报告强调了需要个体化的围手术期护理,以将需要手术的aHUS患者的风险降至最低。