伴肺动脉高压的烟雾病中的无名指蛋白213：一篇综述
Ring Finger Protein 213 in Moyamoya Disease With Pulmonary Arterial Hypertension: A Mini-Review.
作者信息
Luo Yuting, Cao Zhixin, Wu Shaoqing, Sun Xunsha
机构信息
Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.
出版信息
Front Neurol. 2022 Mar 24;13:843927. doi: 10.3389/fneur.2022.843927. eCollection 2022.
Moyamoya disease (MMD), most often diagnosed in children and adolescents, is a chronic cerebrovascular disease characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Recently, many investigators show a great interest in MMD with pulmonary arterial hypertension (PAH). is a major susceptibility gene for MMD and also has strong correlations with PAH. Therefore, this review encapsulates current cases of MMD with PAH and discusses MMD with PAH in the aspects of epidemiology, pathology, possible pathogenesis, clinical manifestations, diagnosis, and treatment.
烟雾病（MMD）最常发生于儿童和青少年，是一种慢性脑血管疾病，其特征为颈内动脉末端进行性狭窄以及脑底部异常血管网形成。最近，许多研究者对合并肺动脉高压（PAH）的烟雾病表现出极大兴趣。 是烟雾病的一个主要易感基因，也与肺动脉高压密切相关。因此，本综述总结了目前合并肺动脉高压的烟雾病病例，并从流行病学、病理学、可能的发病机制、临床表现、诊断及治疗等方面对合并肺动脉高压的烟雾病进行了探讨。
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