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近已证明,遗传性C蛋白缺乏发生静脉血栓形成和栓塞的危险性很大。该病大部份发生在青年人,病前有浅静脉血栓性静脉炎、深静脉血栓或肺栓塞,但无明确病因。本文分析了20个家族中53例遗传性C蛋白缺乏症,42例有静脉血栓形成病史,其中在3个家族中有3例出血性脑梗塞,均为女性,发病年龄分别为27岁、34岁和38岁,发病前或后均有下肢静脉血栓形成和肺栓塞。1例有服避孕药史,在应用香豆素治疗深静脉血栓的早期阶段发生脑梗塞,但无引起出血性梗塞的危险因素;另2例为自发性脑梗塞。3例均无动脉疾患、高血压或动脉源性栓塞。共同的临床表现为头痛、肢体瘫痪、意识障碍。1例动脉造影显示上矢状窦额部血栓形成,相应的皮层静脉亦不充盈,而CT扫描第四脑室出血;另1例CT扫
It has recently been shown that hereditary protein C is at high risk of developing venous thrombosis and embolism. Most of the disease occurs in young people, pre-sick superficial vein thrombophlebitis, deep vein thrombosis or pulmonary embolism, but no clear cause. In this study, we analyzed 53 cases of inherited C protein deficiency in 20 families and 42 cases of history of venous thrombosis. Among them, 3 cases had hemorrhagic cerebral infarction in 3 families, all of whom were women with onset ages of 27 and 34 Year-old and 38 years old, both before and after the onset of venous thrombosis and pulmonary embolism. 1 case of oral contraceptives history, the application of coumarin in the early stages of deep venous thrombosis occurred in cerebral infarction, but no risk factors for hemorrhagic infarction; the other two cases of spontaneous cerebral infarction. All 3 had no arterial disease, hypertension or arterial embolism. Common clinical manifestations of headache, limb paralysis, disturbance of consciousness. One case of arteriography showed thrombosis in the superior sagittal sinus and the corresponding cortical veins were not filled, while CT scan of the fourth ventricle hemorrhage. Another CT scan