%0 Case Reports
%T Budd-Chiari syndrome associated with congenital afibrinogenaemia reversed after orthotopic liver transplant.
%A Nikam VG
%A Dhakre VW
%A Motwani K
%A Chattopadhyay S
%J BMJ Case Rep
%V 17
%N 7
%D 2024 Jul 3
%M 38960420
暂无%R 10.1136/bcr-2023-259078
%X A woman in her mid-20s, a known case of congenital afibrinogenaemia, presented with abdominal pain and distension. She was diagnosed with decompensated liver cirrhosis due to Budd-Chiari syndrome. She underwent deceased donor liver transplantation. Preoperatively, her serum fibrinogen level was undetectable and prothrombin time and international normalised ratio (INR) were unrecordable. Intraoperatively, she was given thromboelastography-guided human fibrinogen concentrate. Postoperatively, her fibrinogen, prothrombin time and INR normalised rapidly. This report summarises the rare occurrence of a complication of hypercoagulability (Budd-Chiari syndrome) in the setting of congenital hypocoagulability (congenital afibrinogenaemia). In this report, we discuss the simultaneous management of these two clinical problems and the curative role of liver transplantation.