Mentese Ilay Berke, Tugcu Murat, Nazli Ismail, Filinte Deniz, Asicioglu Ebru, Arikan Hakki, Tuglular Serhan, Velioglu Arzu
Department of Nephrology.
Department of Internal Medicine, and.
Clin Nephrol Case Stud. 2021 Mar 17;11:50-54. doi: 10.5414/CNCS110617. eCollection 2023.
Kidney function may be impaired during pregnancy due to various reasons, and the physiological changes of pregnancy may unmask or worsen pre-existing kidney disease. Herein, we report a pregnant patient presenting with nephrotic-range proteinuria. She later developed acute kidney injury and pre-eclampsia. However, hemolytic anemia and thrombocytopenia persisted after delivery, and she was diagnosed with atypical hemolytic uremic syndrome (aHUS). Although hematological abnormalities resolved with eculizumab treatment, her renal functions did not improve. Kidney biopsy showed crescentic glomerulonephritis without thrombotic microangiopathy features. Concurrently, she was evaluated for hearing impairment, and a diagnosis of Alport syndrome was confirmed with genetic testing. Kidney function may worsen in patients with Alport syndrome during pregnancy. However, crescentic glomerulonephritis (GN) is a rare finding in Alport disease. Pauci-immune crescentic GN has been shown to be related to dysregulated activation of the alternative complement pathway, which is also the underlying pathophysiological mechanism in aHUS.
由于各种原因，孕期肾功能可能受损，而孕期的生理变化可能会使已有的肾脏疾病暴露或加重。在此，我们报告一例出现肾病范围蛋白尿的孕妇。她随后发展为急性肾损伤和先兆子痫。然而，产后溶血性贫血和血小板减少持续存在，她被诊断为非典型溶血性尿毒症综合征（aHUS）。尽管使用依库珠单抗治疗后血液学异常得到缓解，但她的肾功能并未改善。肾活检显示为新月体性肾小球肾炎，无血栓性微血管病特征。同时，对她进行了听力障碍评估，基因检测确诊为Alport综合征。Alport综合征患者在孕期肾功能可能恶化。然而，新月体性肾小球肾炎（GN）在Alport病中是一种罕见的表现。寡免疫性新月体性GN已被证明与替代补体途径的失调激活有关，这也是aHUS的潜在病理生理机制。
