ACRP  >> Vol. 3 No. 1 (February 2015)

    Neonatal Congenital Absence of Clotting Factor VII: A Case Report

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赵 伟,孙志群,张立明,尹延娥,刘 欣:潍坊市人民医院新生儿科,山东 潍坊

新生儿先天性凝血因子VII缺乏新生儿出血症Newborn Coagulation Factor VII Hemorrhagic Disease of Newborn


目的:探讨新生儿先天性凝血因子VII缺乏的原因和治疗方法。方法:对我科发现的1例新生儿先天性凝血因子VII缺乏病例进行临床资料的回顾性分析。结果:患儿因无明显原因及诱因发现皮肤出血点,转入新生儿重症监护室,经送检凝血因子VII:C 0.5%,提示凝血因子VII缺乏症,送检其父母、姐姐的凝血因子VII C分别为:28.5%、31.0%、和25.2%,提示与遗传因素有关。经反复输注凝血酶原复合物、新鲜冰冻血浆等治疗后,康复出院。结论:在临床工作中,新生儿存在凝血功能异常,治疗效果不满意,应及时完善凝血因子检查,以避免漏诊、误诊,延误病情。

Objective: To investigate the causes and treatment of congenital absence of clotting factor VII. Me-thod: The clinical data of one infant with the disease of congenital absence of clotting factor VII in our department were retrospectively analyzed. Result: The infant was transferred to neonatal ICU because of the skin bleeding for no apparent reason. The determination of coagulation factor VII was C 0.5%, revealing the coagulation factor VII deficiency. The coagulation factor VII C of the in-fant’s parents and sister was respectively 28.5%, 31.0% and 25.2%, indicating the relationship with genetic factors. After repeated infusion of fresh frozen plasma and prothrombin complex, the infant recovered and was discharged from hospital. Conclusion: In clinical work, coagulant function abnormality of neonatal repeatedly, we should check the clotting factor as soon as possible in order to avoid missed diagnosis, misdiagnosis and delay treatment.

赵伟, 孙志群, 张立明, 尹延娥, 刘欣. 新生儿先天性凝血因子VII缺乏一例报道[J]. 亚洲儿科病例研究, 2015, 3(1): 1-3.