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患者26岁,男性,右小腿郁积性溃疡2年而入院。体检:溃疡旁有静脉曲张,静脉造影:深静脉无血栓形成。皮肤有23个咖啡色斑,3个神经纤维瘤和腋下雀斑,确诊为神经纤维瘤,除前纤维蛋白溶解酶活性降低外,实验室检查无异常。家族调查,都无血栓形成。患者的母亲有1.5cm直径以上的咖啡色斑和前纤维蛋白溶酶缺乏,父亲和兄弟前纤维溶酶均正常。讨论:异常的前纤维蛋白溶酶见于复发性血栓栓塞的病人中,可发生在健康成人和其它疾病中,但
The patient was 26 years old and was admitted to the hospital with a stasis ulcer of the right calf for 2 years. Physical examination: varicose veins next to ulcers, venography: deep veins without thrombosis. The skin had 23 caffeine spots, 3 neurofibromas, and subaxillary freckles. The diagnosis was neurofibromatosis. Except for the decrease of the activity of fibrinolytic enzymes before the test, there was no abnormality in laboratory tests. Family investigations showed no thrombosis. The patient’s mother had brown spots and pro-plasmin deficiency above 1.5 cm in diameter, and the father and brother’s former plasmin were normal. Discussion: Abnormal profibrinolytic enzymes are found in patients with recurrent thromboembolism that may occur in healthy adults and other diseases, but