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一名甲型血友病患者并发急性胆囊炎时出现胆囊穿孔及大量腹腔内出血。

Gallbladder perforation and massive intra-abdominal haemorrhage complicating acute cholecystitis in a patient with haemophilia A.

作者信息

Mechera Robert, Graf Lukas, Oertli Daniel, Viehl Carsten T

机构信息

Department of Surgery, University of Basel, Basel, Switzerland.

Department of Haematology, University Hospital Basel, Basel, Switzerland.

出版信息

BMJ Case Rep. 2015 May 2;2015:bcr2014205971. doi: 10.1136/bcr-2014-205971.

Abstract

We present an unusual case of a 32-year-old man with haemophilia A, who sustained massive, haemodynamically significant intra-abdominal bleeding from a perforated gallbladder wall and from the greater omentum as a complication of acute ulcerophlegmonous and haemorrhagic cholecystitis. Recombinant coagulation factor VIII was given and an emergency laparotomy was performed, with open cholecystectomy and haemostasis. Coagulation factor VIII was given for a further 2 weeks postoperatively, and the patient was discharged in good condition. In most published cases of haemorrhagic cholecystitis, the haemoperitoneum arises via transhepatic perforation; in this case, there was a free rupture into the peritoneal cavity. Patients with coagulopathies may have severe haemorrhagic complications and therefore need interdisciplinary management before, during and after surgery. Replacement therapy with factor concentrates should be initiated at once, and early surgery for gallbladder disease should be considered.

摘要

我们报告了一例罕见病例,一名32岁的甲型血友病男性患者,因急性溃疡出血性胆囊炎并发胆囊壁穿孔和大网膜大量血流动力学显著的腹腔内出血。给予重组凝血因子VIII并进行急诊剖腹手术,行开放式胆囊切除术和止血。术后继续给予凝血因子VIII 2周,患者出院时情况良好。在大多数已发表的出血性胆囊炎病例中,血腹是通过肝穿破形成的;而在本病例中,是胆囊直接破裂进入腹腔。凝血功能障碍患者可能会出现严重的出血并发症,因此在手术前、手术中和手术后都需要多学科管理。应立即开始使用凝血因子浓缩物进行替代治疗,并应考虑对胆囊疾病进行早期手术。

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