先天性アンチトロンビンIII欠乏症を合併した腹部大動脈瘤の1例 [in Japanese] A CASE OF ABDOMINAL AORTIC ANEURYSM WITH CONGENITAL ANTITHROMBIN III DEFICIENCY [in Japanese]
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症例は74歳,男性.姉・姪・甥に先天性アンチトロンビンIII(以下ATIIIと略記)欠乏症を認め,患者は現在までに血栓症を指摘されたことはなかった.初診時,右下肢腫脹を認め,さらに腹部に拍動性腫瘤を触知したため,下肢静脈造影, CT施行し,右下肢深部静脈血栓症,腹部大動脈瘤と診断した.またATIII活性値が36～42%と低値を示した.腹部大動脈瘤に対し人工血管置換術を施行するにあたり,手術前日よりATIII濃縮製剤の投与を行い,術後はATIII活性値を80%に維持した.術中・術後共に血栓症の合併もなく,退院となった.本症を合併した手術において, ATIII濃縮製剤によりATIII活性値を正常値に維持し,周術期に血栓症の予防をすることが重要であると考えられた.
A 74-year-old man was admitted to the hospital because of an abdominal aortic aneurysm. His sister, niece and nephew had been diagnosed as having congenital antithrombin III (ATIII) defeciency, but he had not been diagnosed as having thrombosis. At first appearance, he had the right swollen lower leg, and an abdominal pulsating tumor. His ATIII activity was only 36-42% of the normal value, a venography demonstrated thrombosis of the deep vein of the right leg, and abdominal CT scan demonstrated a huge aortic aneurysm. ATIII concentrates were administered to prevent perioperative thrombosis and a graft replacement of the abdominal aortic aneurysm was performed. Postoperatively ATIII activity was maintained in excess of 80%. No complications of thrombosis occurred as judged by clinical examination. Performing operation in this congenital disorder, it is necessary to maintain the ATIII activity in normal range by administering ATIII concentrates, and to prevent thrombosis.
- Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association)
Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 63(6), 1383-1386, 2002-06-25
Japan Surgical Association