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Padua型第七凝血因子缺失之一病例報告
Factor VII Padua Defect-A Case Report
摘要
本文報告一位23歲經心臟超音波法及心導管檢查證實心室中隔缺損之女性病人合併血凝異常患者。其凝血酶原時間（prothrombin time）延長，但部分凝血活酶時間（activated partial thromboplastin time）Stypven凝血時間正常。使用兔腦的凝血活酶（thromboplastin）測定，則凝血酶原時間約為正常人的74%；但如用牛腦凝血活酶測定，則可達100%。估計其第七凝血因子量，前者為48%，後者為100%，其餘之凝血因子均為正常。病人之母親及母之兄姐亦呈現凝血酶原時間延長，測其第七凝血因子約為25～80%之間，臨床均無異常出血症狀，綜觀其結果本病例應為Padua型第七凝血因子缺失之異種胚子，且其遺傳形式，可能屬體隱性遺傳，治療此種病人可採用新鮮冷凍血漿輸注。
並列摘要
A case of hereditary factor VII Padua defect is presented. The proposed was a 23 years old female patient diagnosed as having Ventricular Septal Defect. Previously there was no bleeding tendency, however the prothrombin time was prolonged. The activated partial thromboplastin time and Stypven clotting time were normal. The prothrombin time (74%) was prolonged when employing rabbit brain thromboplastin, but it was normal (100%) when employing ox brain thromboplastin. The prolonged prothrombin time could be corrected by the addition of normal serum. Factor VII level was 48% using rabbit brain as a substrate and was 100% when using ox brain. Other coagulation factors were normal. Patient's mother and maternal relatives also showed mild prolonged prothrombin time with factor VII level of 25-80%, highly suggesting autosomal recessive inheritance with a heterozygous state.