Vernant J C, Buisson G G, Rivierez M, Benyayer P
Nouv Presse Med. 1980 Jan 5;9(1):25-8.
In two children (1 girl and 1 boy aged 6 years) with an SS haemoglobinopathy, a neurological deficit affecting one half of the body led to arteriograms being performed. These revealed stenoses of the major vessels (above all anterior and middle cerebral with development of a collateral circulation giving an appearance suggestive of Moya-moya syndrome). Various aetiologies have been suggested for this syndrome: congenital disease, oral contraceptives, atheroma. Sickle cell disease is a new cause. The mechanism would appear to be fibrosis proliferating secondary to sub-endothelial anoxia caused by sludging in the vasa vasorum.
在两名患有镰状细胞血红蛋白病的儿童（1名6岁女孩和1名6岁男孩）中，影响身体一侧的神经功能缺损导致了动脉造影检查。这些检查显示主要血管狭窄（尤其是大脑前动脉和大脑中动脉，伴有侧支循环形成，外观提示烟雾病综合征）。对于该综合征已提出多种病因：先天性疾病、口服避孕药、动脉粥样硬化。镰状细胞病是一个新的病因。其机制似乎是由于血管滋养管内血液淤滞导致内皮缺氧继发纤维化增生。
