关键词: 先天性Ⅶ缺乏, 围产期, 凝血功能, 治疗策略

Abstract: Objective Analysis the coagulation function of a perinatal pregnant with congenital VII deficiency and the discussion of the treatment strategy. Methods Blood samples from a pregnant with congenital factor VII deficiency at the different time points in peripartum period was collected,plasma clot formation is used to determine the prothrombin time （PT）,activated partial thromboplastin time （APTT）,thrombin time （TT）,Clauss method is used to determine the fibrinogen （Fbg）,partcle-enhance immunoturbidimeric assay is used to determine D-dimer （DD）and antithrombin- III. Results Before the prophylactic substitution treatment in peripartum period,PT was prolonged and Fbg was mild rised,the other index was normal,recombinant activated factor VII （rFVIIa）was given to patients within 1 hour in preoperative,the pregant is characterized by PT shorten and presented high coagulation state. Intraoperative coagulation function was normal and no bleeding complications; postoperation coagulation function became abnormal,characterized by PT extension,higher DD and after 3 days PT was recovered to preoperative levels. Conclusion It may be essential to deteced coagulation function in the perinatal pregnants with congenital factor VII deficiency dynamically,especially the change of PT,such this case of cesarean delivery. Other indexes of coagulation and fibrinolytic may be judge patient's coagulation function effectively.

Key words: Congenital factor Ⅶ deficiency, Peripartum, Coagulation function, Treatment strategy