[光学相干断层扫描血管造影在非典型溶血尿毒综合征视网膜微血管病变诊断中的应用（病例报告）]
[Optical coherence tomography angiography in the diagnosis of retinal microangiopathy in atypical hemolytic-uremic syndrome (a case report)].
作者信息
Smirnova T V, Kozlovskaya N L, Sheludchenko V M, Budzinskaya M V
机构信息
Research Institute of Eye Diseases, Moscow, Russia.
Peoples' Friendship University of Russia, Moscow, Russia.
出版信息
Vestn Oftalmol. 2020;136(4. Vyp. 2):226-234. doi: 10.17116/oftalma2020136042226.
The article describes a clinical case of acute macular neuroretinopathy and «chronic» paracentral acute middle maculopathy in a patient with atypical hemolytic-uremic syndrome - an orphan disease characterized by systemic thrombosis in the vessels of the microcirculatory bed due to chronic uncontrolled activation of the alternative complement pathway. Optical coherence tomography angiography data confirm the ischemic nature of the disease with localization in the deep vascular plexus of the retina in acute macular neuroretinopathy and in the superficial vascular plexus of the retina in «chronic» paracentral acute middle maculopathy. The use of modern diagnostic methods, including optical coherence tomography angiography, can help improve detection of the pathology and expand its understanding in severe microangiopathic syndromes, which include atypical hemolytic-uremic syndrome.
本文描述了一名非典型溶血尿毒综合征患者的急性黄斑神经视网膜病变和“慢性”中心旁急性黄斑中部病变的临床病例。非典型溶血尿毒综合征是一种罕见病，其特征是由于替代补体途径的慢性失控激活，导致微循环床血管发生全身性血栓形成。光学相干断层扫描血管造影数据证实了该疾病的缺血性质，急性黄斑神经视网膜病变定位于视网膜深层血管丛，“慢性”中心旁急性黄斑中部病变定位于视网膜浅层血管丛。使用包括光学相干断层扫描血管造影在内的现代诊断方法，有助于提高对该病理的检测，并扩大对包括非典型溶血尿毒综合征在内的严重微血管病综合征的认识。
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