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Ⅶ因子缺乏症是一种较为少见的遗传性出血性疾病,属常染色体不完全隐性遗传,男女均可发病.1 病历摘要1.1 例1,男,12岁,十年来经常鼻衄,出血量不多,反复发作,三个月前无明原因左膝关节肿胀,活动受限,但无明显疼痛,院外诊为“类风湿性关节炎”,经住院左膝关节腔抽取350ml血性液体,颜色鲜红不凝固,于十天前来我院就诊,家族史中无同病患者.查体:发育正常,营养中等,无贫血,浅表淋巴结不大,心肺无异常,肝脾未触及,左膝关节明显肿胀,浮膑试验阳性,其余关节无异常.实验室检查:红细胞4.30×10~(12)/L,血红蛋白151g/L,白细胞14.3×10~9/L,中性粒细胞0.75,淋巴细胞0.22,单核细胞0.03,肝功能正常,HBsAg阴性.凝血象检查:纤维蛋白原3.5g/L,白陶土部分凝血活酶时间87S/42S(对照),试管法凝血时间14分/12分,血清凝血酶原时间39S/35S,凝血活酶生成试验正常,其余见附表.
Factor VII deficiency is a rare hereditary hemorrhagic disease is an autosomal recessive inheritance, both men and women can be disease .1 medical records 1.1 cases 1, male, 12 years old, ten years of frequent rhinorrhea, bleeding Not much, recurrent, three months ago, unexplained swelling of the left knee, limited mobility, but no obvious pain, out-patient diagnosis of “rheumatoid arthritis,” by the hospital left knee cavity extraction 350ml bloody fluid, color Bright red not solidified in ten days to our hospital for treatment, no family history of patients with the same disease. Physical examination: normal development, moderate nutrition, no anemia, superficial lymph nodes, no abnormal heart and lung, liver and spleen not touched, left knee The joints were obviously swollen, the floating test was positive, and the other joints were normal.Experimental tests showed that the number of erythrocytes was 4.30 × 10 ~ (12) / L, the hemoglobin was 151g / L, the leucocytes was 14.3 × 10 ~ 9 / L, the neutrophils were 0.75, Cells 0.22, monocytes 0.03, normal liver function, HBsAg negative coagulation test: fibrinogen 3.5g / L, kaolin part of the thromboplastin time 87S / 42S (control), test tube method clotting time 14 points / 12 points , Prothrombin time 39S / 35S, thromboplastin production test is normal, the rest see Schedule.