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Protein C deficiency and portal thrombosis in liver transplantation in children
P L Harper1, P F Edgar, R J Luddington
1Department of Haematology, Addenbrooke's Hospital, Cambridge.
Insights
Pediatric liver transplant recipients experienced protein C deficiency and hypercoagulability, increasing portal thrombosis risk. Protein C and antithrombin replacement is crucial for preventing thrombosis post-transplant.
Area of Science:
- Pediatric Hepatology
- Transplantation Immunology
- Hemostasis and Thrombosis
Background:
- Liver transplantation is a life-saving procedure for children with end-stage liver disease.
- Post-transplant complications, including thrombosis, remain a significant concern, particularly in pediatric recipients.
- Understanding coagulation factor dynamics is essential for optimizing perioperative management.
Purpose of the Study:
- To investigate plasma protein C and antithrombin levels in pediatric liver transplant patients.
- To assess the relationship between coagulation factor deficiencies and the incidence of portal vessel thrombosis.
- To evaluate the efficacy of current anticoagulant strategies in preventing post-transplant thrombosis.
Main Methods:
- Prospective monitoring of plasma protein C and antithrombin concentrations in 14 pediatric liver transplant recipients.
- Comparison with a control group of 14 adult liver transplant recipients.
- Correlation analysis between coagulation factor levels, plasminogen activator inhibitor, and the occurrence of portal vessel thrombosis.
Main Results:
- Children exhibited persistent protein C deficiency and a less pronounced antithrombin deficiency in the early postoperative period.
- A hypercoagulable state was observed in pediatric patients, exacerbated by increased plasminogen activator inhibitor.
- The peak incidence of portal vessel thrombosis (4-10 days post-transplant) coincided with these coagulation abnormalities.
- Antithrombin replacement and heparin failed to prevent portal thrombosis in two pediatric cases.
Conclusions:
- Pediatric liver transplant recipients are at high risk for thrombosis due to protein C and antithrombin deficiencies.
- Effective prevention of portal thrombosis requires targeted replacement therapy.
- Protein C replacement combined with appropriate antithrombin supplementation, not exceeding normal plasma activity, is recommended for successful thrombosis prevention.
Abstract:
Changes in plasma protein C and antithrombin concentrations after liver transplantation were monitored in fourteen children and a control group of fourteen adults. In the children, there was a persistent deficiency in the plasma concentration of protein C and a less pronounced deficiency in antithrombin during the early postoperative period, causing a hypercoaguable state. A concomitant rise in plasminogen activator inhibitor further increased the risk of thrombosis by inhibiting fibrinolysis. These changes coincided with the peak incidence of portal vessel thrombosis (4-10 days). Replacement of plasma antithrombin, together with heparin, did not prevent portal thrombosis in two of the children. It is concluded that successful prevention will require protein C replacement together with antithrombin supplements up to, but not exceeding, normal plasma activity.