Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Japan.
Intern Med. 2021 Mar 15;60(6):917-922. doi: 10.2169/internalmedicine.5716-20. Epub 2020 Oct 21.
Patients with atypical hemolytic uremic syndrome (aHUS) associated with a C3 p.Ile1157Thr mutation show a relatively high renal survival and low mortality rates, but renal histopathological findings after recurrence have been rarely reported. A 30-year-old man with a C3 p.Ile1157Thr mutation experienced a third recurrence of thrombotic microangiopathies with neurological and gastrointestinal disorders. A renal biopsy performed during the recovery phase of acute kidney injury revealed collapsed glomeruli and arteriolar vacuolization. Approximately 10% of glomeruli were globally sclerotic, despite the absence of arterio-/arteriolo-sclerosis. These findings suggest substantial progression of irreversible injuries in multiple organs, including kidneys, which occurs in aHUS patients with repeated thrombotic microangiopathies.
携 C3 p.Ile1157Thr 突变的非典型溶血尿毒综合征（aHUS）患者的肾脏存活率相对较高，死亡率较低，但复发后的肾脏组织病理学发现却很少有报道。一位 30 岁的男性携 C3 p.Ile1157Thr 突变，经历了第三次血栓性微血管病伴神经和胃肠道紊乱的复发。在急性肾损伤的恢复期进行的肾脏活检显示肾小球塌陷和小动脉空泡化。尽管没有动脉/小动脉硬化，约 10%的肾小球呈全球性硬化。这些发现提示包括肾脏在内的多个器官的不可逆损伤实质性进展，这发生在反复发生血栓性微血管病的 aHUS 患者中。
