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  [in Japanese] Cholelithiasis with a cholecystoduodenal fistula complicated with paroxysmal nocturnal hemoglobinuria  [in Japanese]

Access this Article

Author(s)

Abstract

  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.

  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.

Journal

  • Okayama Igakkai Zasshi (Journal of Okayama Medical Association)

    Okayama Igakkai Zasshi (Journal of Okayama Medical Association) 127(1), 35-39, 2015

    Okayama Medical Association

Codes

  • NII Article ID (NAID)
    130005068351
  • NII NACSIS-CAT ID (NCID)
    AN00032489
  • Text Lang
    JPN
  • Article Type
    journal article
  • Journal Type
    大学紀要
  • ISSN
    0030-1558
  • NDL Article ID
    026375270
  • NDL Call No.
    Z19-269
  • Data Source
    NDL  IR  J-STAGE 
Page Top