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Research Article Open Access
Abdominal compartment syndrome (ACS) is a life-threatening condition which can occur in patients with hemophilia although they have trivial traumas. Hemostatic control for bleeding episodes in hemophilia patients with inhibitors is difficult particularly when the availability of bypassing agents, recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (APCC), is constrained. Plasma exchange with continuous infusion of factor concentrate has been reported as a life-saving intervention in these patients. We reported a teenager with severe hemophilia A and a high-titer inhibitor who underwent two surgeries for ACS which developed after a minor trauma. Computerized tomography angiogram (CTA) of abdomen revealed a large pelvic hematoma and a bleeding from the sigmoidal artery. He underwent an abdominal angiography followed by the first surgery to relieve the ACS, and the second surgery for abdominal closure. The patients received plasma exchange with cryo-removed plasma peri-operatively. High-dose factor VIII (FVIII) concentrate (100 U/kg) was started after plasma exchange followed by continuous infusion at the rate 14 units/kg/hour for 7 days. rFVIIa and APCC concomitant with tranexamic acid were used for breakthrough bleeding. He received six times of plasma exchange, three doses of rFVIIa and five doses of APCC. Bleeding was successfully stopped and the titers of inhibitor decreased from the maximum of 4,400 BU to 3,680 BU. Plasma exchange and continuous FVIII infusion can be considered as an option for life-threatening hemorrhage in hemophilia patients with high-titer inhibitors in the countries where an access to bypassing agents is limited.
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Author(s): Rungrote Natesirinilkul, Pimlak Charoenkwan, Sanit Ruangrongrat, Kaweesak Chittawatanarat, Ampaiwan Chuansumrit, Rungnapa Jutavijittum, Nipapan Leetrakool and Ornkamon Wongtagun
Abnormal Behavior,Abnormal Psychology,Behavioural Psychology