Kohrman M H, Huttenlocher P R
Department of Pediatrics, University of Chicago, Pritzker School of Medicine, Illinois.
Pediatr Neurol. 1986 May-Jun;2(3):154-8. doi: 10.1016/0887-8994(86)90007-x.
Takayasu disease is an arteritis of unknown etiology involving the aorta and its major branches. Untreated, 75% of patients die within two years. A 6-month-old black female presented with a right focal seizure, a flaccid right hemiparesis, decreased pulses in the right arm, a large left frontoparietal hypodense area on computed tomography, and an elevated erythrocyte sedimentation rate. Cerebral angiography demonstrated irregular dilatation of both carotid arteries and narrowing of the left middle cerebral artery. Aneurysm of the right sinus of Valsalva, dilatation of the aortic root, narrowing of the origins of both carotid arteries, and beading of the descending aorta were demonstrated by cardiac angiography. The patient was treated with prednisone (2 mg/kg/day) and azathioprine (1 mg/kg/day). There was gradual return of the erythrocyte sedimentation rate to normal over the ensuing 3-10 months, resolution of the hemiparesis, and acquisition of normal developmental milestones. Digital subtraction angiography revealed improvement in the appearance of the descending aorta and of the common carotid arteries with the disappearance of the arterial wall irregularities. Early diagnosis and vigorous immunosuppression may improve outcome in this rare and often fatal vascular disease in childhood.
高安氏病是一种病因不明的动脉炎，累及主动脉及其主要分支。若不治疗，75%的患者会在两年内死亡。一名6个月大的黑人女性出现右侧局灶性癫痫发作、右侧弛缓性偏瘫、右臂脉搏减弱、计算机断层扫描显示左侧额顶叶有大片低密度区以及红细胞沉降率升高。脑血管造影显示双侧颈动脉不规则扩张，左侧大脑中动脉狭窄。心脏血管造影显示主动脉瓣窦右动脉瘤、主动脉根部扩张、双侧颈动脉起始处狭窄以及降主动脉串珠样改变。该患者接受了泼尼松（2毫克/千克/天）和硫唑嘌呤（1毫克/千克/天）治疗。在随后的3至10个月里，红细胞沉降率逐渐恢复正常，偏瘫症状消失，并且达到了正常的发育里程碑。数字减影血管造影显示降主动脉和颈总动脉的外观有所改善，动脉壁不规则现象消失。早期诊断和积极的免疫抑制治疗可能会改善这种儿童期罕见且往往致命的血管疾病的预后。
