摘要
脑静脉窦血栓形成(CVST)是神经内科少见疾病,临床表现不典型,起病隐匿,如同时伴有蛋白S缺乏症则更罕见。该文报道2例家族性病例,其中例1女性患者为家族中的女儿、17岁;例2男性患者为例1的父亲、45岁,例2 CVST发病年龄为30岁。2例临床表现均为头痛伴恶心、呕吐,影像学检查均提示CVST,检测例1的蛋白S为21.3%、例2小于16%,给予抗凝药物联合营养神经等对症支持治疗后临床症状得到缓解,均诊断为蛋白S缺乏症合并CVST。临床上发现无明显诱因出现CVST的年轻患者,应考虑有无高凝血状态,有无先天遗传因素,同时需考虑易栓症可能,及时检测抗凝血酶Ⅲ、蛋白S和蛋白C活性、血小板聚集功能等,这对不明原因CVST的诊治有重要意义。
Cerebral venous sinus thrombosis(CVST)is not a common disease in Department of Neurology,which has atypical clinical manifestations and insidious onset.CVST complicated with protein S deficiency is even more rarely seen.In this article,two family cases of CVST complicated with protein S deficiency were reported.One 17-year-old female patient was the daughter and the other case of 45-yearold male patient was her father.The age of onset of the father was 30 years old.Both two patients presented with headache,nausea and vomitting.Imaging examination prompted the diagnosis of CVST.The protein S in the daughter was 21.3%,and that of the father was less than 16%.Clinical symptoms were relieved after symptomatic and supportive treatment,such as subcutaneous injection and oral anticoagulant drugs combined with nerve nutrition.Both two cases were diagnosed with protein S deficiency complicated with CVST.In clinical practice,it is necessary to consider the possibility of hypercoagulable state,genetic factors and thrombophilia for young CVST patients without known causes.Anti-thrombinⅢ,activities of protein S and protein C and platelet aggregation function should be detected in a timely manner,which is of significance for the diagnosis and treatment of CVST with unknown causes.
作者
徐杰文
Xu Jiewen(Department of Stroke Center,Panyu District Central Hospital,Guangzhou 515041,China)
出处
《新医学》
2020年第11期884-887,共4页
Journal of New Medicine
