Abstract:

Factor Ⅺ deficiency is a rare autosomal inherited hemorrhagic disease with a prevalence of about one in a million. Heterozygous carriers often have no obvious bleeding tendency, and homozygous or compound heterozygous carriers often have severe factor Ⅺ deficiency. Factor Ⅺ deficiency in pregnancy is particularly rare and it has rarely been reported in China. Two cases of severe factor Ⅺ deficiency in pregnancy were reported, including one case of factor Ⅺ compound heterozygous mutation. Two patients were diagnosed with severe coagulation factor Ⅺ deficiency at the time of prenatal examination. After admission, a multidisciplinary consultation was conducted, evaluate 2 cases of patients without vaginal delivery contraindications, suggest by vaginal birth and anti-fibrinolytic drugs were used after labor. Healthy live infants were delivered vaginally under close monitoring. After delivery, tranexamic acid antifibrinolytic and strong uterine contraction agents were used to strengthen uterine contraction treatment. After delivery, both lower limbs were treated with air pressure to prevent thrombosis. After 4 days of hospitalization, the mother and child were discharged without complications. By reviewing the medical history clinical diagnosis and treatment process of the case and reviewing the relevant literature, the aim is to increase the clinicians understanding of the disease and accumulate more clinical diagnosis and treatment experience.

Key words: Coagulation protein disorders, Factor Ⅺ, Blood coagulation disorders, inherited, Perinatal management, Case reports