Sarno Laura, Conca Paolo, Capuano Alfredo, Tarantino Giovanni, Russo Domenico, Guida Maurizio
Department of Neurosciences, Reproductive Science and Dentistry, University Federico II, 80131 Naples, Italy.
Department of Clinical Medicine and Surgery, Regional Reference Center for Coagulation Disorders, University Federico II, 80131 Naples, Italy.
J Clin Med. 2022 Nov 25;11(23):6957. doi: 10.3390/jcm11236957.
Atypical Hemolytic Uremic Syndrome is a very rare condition that can be triggered in predisposed patients. It can remain undiagnosed and can result in a life-threatening event or permanent renal failure. We report a case of a 36-year-old pregnant woman who developed atypical hemolytic uremic syndrome postpartum. She underwent an emergency caesarean section due to abruptio placenta, and she developed biochemical alterations suggestive of a thrombotic microangiopathy. Due to worsening of renal function after plasma exchange therapy, we decided to start therapy with eculizumab. Therapy was carried out with a weekly dose of 900 mg IV for five weeks. An improvement of clinical and biochemical parameters was rapidly observed, and her renal function completely recovered. The therapy was continued for six months, with a dose of 1200 mg of eculizumab every two weeks. One year after discontinuation of the therapy, her blood pressure and renal function were still normal. Our case confirms that it is important to promptly identify a pregnancy-related thrombotic microangiopathy and that early therapy can be life-saving for the patient and can preserve renal function, avoiding dialysis.
非典型溶血性尿毒症综合征是一种非常罕见的疾病，可在易感患者中引发。它可能未被诊断出来，并可能导致危及生命的事件或永久性肾衰竭。我们报告一例36岁的产后发生非典型溶血性尿毒症综合征的孕妇病例。她因胎盘早剥接受了急诊剖宫产，并且出现了提示血栓性微血管病的生化改变。由于血浆置换治疗后肾功能恶化，我们决定开始使用依库珠单抗进行治疗。治疗采用每周静脉注射900毫克，共五周。很快观察到临床和生化参数有所改善，她的肾功能完全恢复。治疗持续了六个月，每两周注射1200毫克依库珠单抗。停药一年后，她的血压和肾功能仍然正常。我们的病例证实，及时识别与妊娠相关的血栓性微血管病很重要，早期治疗对患者可能挽救生命，并可保留肾功能，避免透析。
