Mahale Ramanath, Rathi Pankaj, Ginegiri Chetan, Aggarwal Rajiv
Department of Pediatrics, Narayana Hrudayalaya Institute of Medical Sciences, Narayana Hrudayalaya Children's Hospital, Anekal Taluk, Bommasandra Indusrial Estate, Bangalore, India.
Indian J Hematol Blood Transfus. 2010 Jun;26(2):68-9. doi: 10.1007/s12288-010-0023-5. Epub 2010 Oct 6.
Factor VII deficiency is a rare congenital coagulopathy. Prolonged prothrombin time with normal partial thromboplastin time indicates factor VII deficiency. For the definitive diagnosis, the specific factor VII level should be investigated. We report a 20 day old, baby girl, born full-term who was admitted with the diagnosis of sepsis. Hematological tests revealed prolonged prothrombin time and a factor VII level of nine percent. After antibiotic therapy and fresh frozen plasma replacement, her clinical status improved but the prothrombin time continued to be prolonged. On the seventh day of discharge the baby died due to sudden intracranial hemorrhage.
凝血因子VII缺乏症是一种罕见的先天性凝血病。凝血酶原时间延长而部分凝血活酶时间正常提示凝血因子VII缺乏症。为明确诊断，应检测特定的凝血因子VII水平。我们报告一例20日龄足月女婴，因脓毒症入院。血液学检查显示凝血酶原时间延长，凝血因子VII水平为9%。经抗生素治疗及输注新鲜冰冻血浆后，她的临床状况有所改善，但凝血酶原时间仍持续延长。出院后第7天，该婴儿因突发颅内出血死亡。
