[非典型溶血尿毒综合征中的眼部血栓性微血管病（一项临床病例研究）]
[Ocular thrombotic microangiopathy in atypical hemolytic-uremic syndrome (a clinical case study)].
作者信息
Smirnova T V, Sheludchenko V M, Kozlovskaya N L, Kazaryan E E, Andzhelova D V, Sherstneva L V, Velieva I A, Kuchieva A M, Akaeva M I
机构信息
Research Institute of Eye Diseases, 11A Rossolimo St., Moscow, Russian Federation, 119021.
I.M. Sechenov First Moscow State Medical University, 8-2 Trubetskaya St., Moscow, Russian Federation, 119991.
出版信息
Vestn Oftalmol. 2018;134(5. Vyp. 2):215-226. doi: 10.17116/oftalma2018134051215.
The article presents a clinical observation of ocular thrombotic microangiopathy in atypical hemolytic-uremic syndrome - a rare genetic disease characterized by systemic thrombosis caused by uncontrolled activation of alternative complement pathway. A typical manifestation of this ocular lesion in this disease is bilateral Purtscher-like retinopathy. Timely diagnostics of atypical hemolytic-uremic syndrome, including ophthalmologic examination, determines the early start of a highly effective pathogenetic therapy with complement inhibitor eculizumab.
本文介绍了非典型溶血尿毒综合征中眼部血栓性微血管病的临床观察——这是一种罕见的遗传性疾病，其特征是替代补体途径不受控制的激活导致全身血栓形成。该疾病中这种眼部病变的典型表现是双侧类Purtscher样视网膜病变。非典型溶血尿毒综合征的及时诊断，包括眼科检查，决定了使用补体抑制剂依库珠单抗进行高效病因治疗的早期开始。
Zotero 插件