Journal of Okayama Medical Association
Published by Okayama Medical Association

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胆嚢十二指腸瘻に対し合併症なく手術を行えた発作性夜間血色素尿症の1例

加藤 卓也 広島市立広島市民病院 外科
松川 啓義 広島市立広島市民病院 外科
塩崎 滋弘 広島市立広島市民病院 外科
藤 智和 広島市立広島市民病院 外科
藤原 康宏 広島市立広島市民病院 外科
二宮 基樹 広島市立広島市民病院 外科
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抄録
 In cases of paroxysmal nocturnal hemoglobinuria (PNH), attention must be paid to potential complications such as thrombosis and hemolysis due to perioperative stress and infection from complement activation. Here we present the case of a 61-year-old Japanese woman with PNH. We made the diagnosis of PNH when she was 28 years old, and we administered repeated steroid medication and erythrocyte transfusion. The patient's cholecystocholedocholithiasis with a cholecystoduodenal fistula was diagnosed based on a survey of the right hypochondriac pain. We performed endoscopic nasobiliary drainage (ENBD) for the prophylaxis of perioperative infection, plus a cholecystectomy and fistulectomy. There were no complications, including hemolysis attack, infection, thrombosis with irrigation erythrocyte transfusion, steroid cover, or the need for heparin administration during the perioperative period. The reduction of the complement activation is necessary in the perioperative management of PNH patients. The prevention of the development of acidosis and hypoxemia, the selection of washed red blood cells, steroid use, appropriate infection measures and thrombosis prophylaxis are all important for the prevention of complications.
キーワード
発作性夜間血色素尿症(PNH)(paroxysmal nocturnal hemoglobinuria (PNH))
胆嚢十二指腸瘻(cholecystoduodenal fistula)
溶血発作(hemolysis)
備考
症例報告 (Case Reports)
ISSN
0030-1558
NCID
AN00032489
DOI
NAID