Giris ve Amaç: Etyolojisinde tromboz olan ve/veya dogal seyrinde trombotik komplikasyonlarla sik karsilasilan gastrointestinal hastaliklarda (portal ven trombozu, Budd-Chiari sendromu, karaciger sirozu, inflamatuar barsak hastaligi ve gastrointestinal malignite) aktive protein C direnci sikligi ile protein C, protein S ve antitrombin eksikliklerini arastirmak üzere bu prospektif çalisma planlandi. Gereç ve Yöntem: Hastalardan fonksiyonel yöntemlerle protein C, protein S, antitrombin aktiviteleri ve koagülasyon faktörü V?den yoksun test plazmasi kullanilarak modifiye yöntemle aktive protein C direnci arastirildi. Bulgular: Protein C eksikligi siroz, malign ve sekonder portal ven trombozu gruplarinda diger gruplardan daha sikti (p<0.05). Primer portal ven trombozu ve Budd-Chiari sendromundaki protein C eksikligi ise kontrolden daha fazlaydi (p<0.05). Protein S eksikligi Budd-Chiari sendromunda diger tüm gruplardan istatistiksel önemde fazlaydi. Inflamatuar barsak hastaligi, malign ve her iki portal ven trombozu grubunda protein S eksikligi orani siroz ve kontrolden yüksekdi (p<0.05). Antitrombin eksikligi sirozda diger tüm gruplardan daha fazlaydi. Aktive protein C direnci varligi orani tüm hasta gruplarinda kontrolden istatistiksel önemde fazlalik gösteriyordu (p<0.05). Budd-Chiari sendromunda aktive protein C direnci bulunma orani ise diger tüm gruplardan fazlaydi (p<0.05). Ayrica sirozlu ve maligniteli hastalarda tromboz varligiyla aktive protein C direnci bulunmasi korelasyon gösteriyordu (p<0.05). Trombofilik faktörlerden iki veya daha fazlasinin birarada bulunmasi tromboz riskinde belirgin artisa neden olmaktaydi. Sonuç: Protein C, protein S ve antitrombin eksikliklerinin ve özellikle aktive protein C direnci varliginin; Budd-Chiari sendromu ve portal ven trombozu etyolojisinde rol oynayabilecegi, sirozlu, inflamatuar barsak hastalikli ve maligniteli hastalarda tromboz gelisme riskini arttirdigi saptandi.
Background and Aims: We aimed to determine the frequency of activated protein C resistance and deficiencies of protein C, protein S and antithrombin in different gastrointestinal diseases (portal vein thrombosis, Budd-Chiari syndrome, liver cirrhosis, inflammatory bowel disease and gastrointestinal malignancies), which related with thrombosis etiologically and/or pathogenetically. Materials and Methods: Protein C, protein S and antithrombin activities were measured by functional methods. Activated protein C resistance was detected by the modified method using factor V-deficient plasma. Results: Protein C deficiency in the cirrhosis, malignancy and secondary portal vein thrombosis groups was significantly higher than in the other groups (p<0.05). Protein C deficiency in primary portal vein thrombosis and Budd-Chiari syndrome was higher than in the healthy controls (p<0.05). Protein S deficiency in Budd-Chiari syndrome was higher than in all other groups (p<0.05). In addition, protein S deficiency in the inflammatory bowel disease, malignancy and both portal vein thrombosis groups was higher than in the cirrhosis and control groups (p<0.05). Antithrombin deficiency in the cirrhosis group was significantly higher than in all other groups (p<0.05). The presence of activated protein C resistance was significantly higher in all of the disease groups compared to the control group (p<0.05). Furthermore, the presence of activated protein C resistance in patients with Budd-Chiari syndrome was significantly higher than in all the other groups (p<0.05). The presence of activated protein C resistance was correlated with the presence of thrombosis in patients with cirrhosis and malignancy. Co-occurrences of two or more abnormalities among these four studied factors significantly increased the risk of thrombosis. Conclusions: Deficiencies in protein C, protein S and antithrombin and especially the presence of activated protein C resistance are important factors playing a role in the pathogenesis of Budd-Chiari syndrome and portal vein thrombosis, and they increased the risk of thrombosis in the cirrhosis, malignancy and inflammatory bowel disease groups.