Relationship between the deficiency of protein C, protein S and antithrombin III with extra-hepatic portal hypertension in children

Abstract

Objective To explore the relationship between the deficiency of protein C, protein S and antithrombin Ⅲ and extra-hepatic portal hypertension and evaluate the effects of Rex shunt on protein C, protein S and antithrombin Ⅲ. Methods From December 2013 to January 2015, 23 children (9 boys and 14 girls, age: 2.1-17.5 years, mean age: 6.5 years) with portal cavernous lesions were recruited. The procedures included Rex shunt (n=18), laparoscopic splenic artery ligation (n=2), recurrent portal hypertension with Rex shunt (n=2) and Warren shunt (n=1). Blood samples were collected preoperatively in all children, postoperatively in 17 children undergoing Rex shunt and 1 child undergoing Warren shunt and 1 child undergoing laparoscopic splenic artery ligation. And blood samples of two children with recurrent portal hypertension were also withdrawn. Blood samples of 18 normal children were used as controls. The concentrations of protein C, protein S and antithrombin Ⅲ and the activity of antithrombin Ⅲ were detected by enzyme-linked immunosorbent assay (ELISA) kit. Results The preoperative concentrations of protein C and protein S and the activity of antithrombin Ⅲ were significantly lower than those of control group (P=0.000, P=0.001 & P=0.000). No difference existed between the preoperative concentration of antithrombin Ⅲ and control group (P=0.057). The concentrations of protein C and protein S and the activity of antithrombin Ⅲ after Rex shunt were significantly higher than those prior to Rex shunt (P=0.000, P=0.001 & P=0.000). No difference existed between the preoperative concentration of antithrombin Ⅲ and that after Rex shunt (P=0.959). The concentration of protein C after Rex shunt was significantly higher than that of control group (P=0.043). No difference existed between the concentrations of protein S and antithrombin Ⅲ and the activity of antithrombin Ⅲ after Rex shunt and control group (P=0.398, P=0.134 & P=0.612). Conclusions The deficiency of PC, PS and AT Ⅲ is significant in patients with extra-hepatic portal hypertension. Rex shunt is beneficial for improvements of protein C, protein S and antithrombin Ⅲ due to restoration of intra-hepatic portal vein perfusion. Key words: Child; Hypertension, portal; Antithrombin Ⅲ