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目的通过回顾分析遗传性纤维蛋白原缺乏合并妊娠的病例,同时结合文献探讨遗传性纤维蛋白原缺乏合并妊娠的处理。方法回顾分析某院近10年内接诊排除继发性纤维蛋白原降低的疾病,明确为遗传性纤维蛋白原缺乏合并妊娠4例患者的资料。结果 4例患者均为常规查体时发现血纤维蛋白原水平低于正常,在妊娠期间均无出血倾向,分娩前血纤维蛋白原水平均低于120 mg/d L,同时伴凝血酶时间(TT)延长,4例患者妊娠期间均未发生流产、胎盘早剥,分娩期未发生产后出血及DIC.其中有3例患者在分娩前使用纤维蛋白原替代治疗。结论遗传性纤维蛋白原缺乏对于妊娠期最常见的并发症为自然流产、胎盘早剥及产后出血。妊娠期应严密观察患者有无出血倾向,有无流产及胎盘早剥的临床表现,定期监测凝血功能,注意纤维蛋白原和其他凝血指标变化,必要时输入纤维蛋白原或冷沉淀物进行替代治疗。分娩前后维持血纤维蛋白原≥120mg/d L,必要时使用替代治疗以减少产后出血和DIC的风险。
Objective To review the cases of hereditary fibrinogen lacking in pregnancy combined with literature review and discuss the treatment of hereditary fibrinogen lacking in pregnancy. Methods A retrospective analysis of a hospital in the past 10 years, admissions to exclude secondary fibrinogen reduction of the disease, clear genetic hereditary fibrinogen lack of merger in 4 patients with pregnancy information. Results All the 4 patients were found to have normal fibrinogen level below normal. There was no bleeding tendency during pregnancy. The levels of fibrinogen before delivery were both lower than 120 mg / d L, with thrombin time (TT ) Prolonged, 4 cases of pregnancy did not occur during pregnancy miscarriage, placental abruption, childbirth and no postpartum hemorrhage and DIC occurred in 3 patients before delivery of fibrinogen replacement therapy. Conclusions The most common complication of hereditary fibrinogen during pregnancy is spontaneous abortion, placental abruption and postpartum hemorrhage. Pregnancy should be closely observed in patients with or without bleeding tendency, with or without abortion and clinical manifestations of placental abruption, regular monitoring of coagulation, pay attention to changes in fibrinogen and other coagulation parameters, if necessary, enter fibrinogen or cryopreserved replacement therapy . Pre and postpartum maintenance fibrinogen ≥ 120mg / d L, if necessary, the use of alternative treatment to reduce the risk of postpartum hemorrhage and DIC.