선천성 제 V 혈액 응고인자 결핍증 임산부의 정상 질식 분만 1 예

Abstract

Congenital factor Ⅴ deficiency is a rare coagulation disorder, which is genetically autosomal recessive. In 1947, Owren first described a case of parahemophilia. During childhood, ecchymosis (bruising), epistaxis, oral hemorrhage, soft-tissue hemorrhage, and postpartum hemorrhage are noted in half of the patients. Because factor Ⅴ is involved in common pathway in the coagulation scheme, prothrombin time (PT) and partial thromboplastin time (PTT) are prolonged. Definite diagnosis can be made with a specific factor Ⅴ assay. Fresh frozen plasma would appear to be the treatment of choice. For the first time in Korea, we experienced a case of vaginal delivery in a patient with factorⅤ deficiency. She delivered a male baby weighing 3120gm with median episiotomy. Six units of fresh frozen plasma were transfused during delivery. After additional transfusions of fresh frozen plasma, she was discharged on fourth postpartum day without hemorrhage or hematoma.