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P497 DIRECT SEQUENCING OF PROTEIN-C AND -S GENES IN PATIENTS WITH BUDD–CHIARI SYNDROME (BCS), PORTAL-VEIN THROMBOSIS (PVT) AND OBLITERATIVEPORTAL-VENOPATHY (OPV) AND SUSPICION OF INHERITED PROTEIN-C OR -S DEFICIENCY A. Plessier, L. Elkrief, P.E. Rautou, E. Deraucourt, O. Goria, K. Zekrini, L. Boudaoud, D. Valla, M. Alhenc Gelas. Department of Hepatology, Hopital Beaujon, AP-HP, University Paris-Diderot, INSERM U 773, Department of Hematology, Haemostasis and Biology, AP-HP, Hopital Beaujon, INSERM U 773 & Universite Paris-VII, Clichy, Department of Gastroenterology, Hepatology, CHU Rouen, Rouen, Hopital Beaujon, AP-HP, University Paris-Diderot, INSERM U 773, Service d’Hepatologie, Clichy, Department of Hematology, Haemostasis and Biology, AP-HP, Hopital Europeen Georges Pompidou, INSERM & Universite Paris, Paris, France E-mail: aurelie.plessier@bjn.aphp.fr Background and Aims: Protein C (PC) and S (PS) deficiencies identified in up to 30% of vascular liver disease patients, are possible risk factors for such diseases, but may alternatively be a mere consequence of liver dysfunction. To address this point, we analyzed PROC or PROS1 genes in these patients with PC or PS deficiency suspicion. Methods: Out of 150 PVT, 74 BCS, 30 OPV, currently included in the dedicated database for vascular disease, inherited PC or PS deficiency [isolated deficiency (protein C clotting activity |
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