Antithrombin is a potent inhibitor of the coagulant effect of thrombin.In the latter half of 20(th) century, many families have been described in which an autosomaly dominantinherited antithrombin deficiency has caused severe venous thromboembolic disease in successive generations.The important complication is severe venoocclusive disease by deep venous thrombus. Some inherited antithrombindeficient patients developed renal failure because of fibrin deposition in the kidney glomeruli or renalvein thrombus, and therefore the need for replacement therapy for end stage renal disease (ESRD). Althoughan inherited antithrombin deficiency with renal failure is rare, prevention against renal failure insuch patients, and their renal replacement therapy for ESRD are important. Proteinuria decreases plasmaantithrombin level leading to more severe hyper-coagulation state. Therefore early in renal disease,it may be prudent for adaptation of anti-coagulation therapy even if recurrent thrombosis has not occurred.All replacement therapy (hemodialysis, transplantation or peritoneal dialysis) for ESRD are availablefor such thrombophilic disorders. Anticoagulation agents working without aggravation of antithrombineffects (Argatroban, Nafamostat mesilate etc.) are useful for hemodialysis. The renal allograft recipientswith thrombophilia seem to be at risk of developing an acute rejection or other vascular event. Peritonealdialysis is potentially a good adaptation for such thrombophilic disorders. However which therapy hasthe best mortality and morbidity outcomes is not clear. Physicians and Surgeons must pay attention tothe coagulation state and thrombophilia in ESRD patients, give strong consideration for adequate anti-coagulationtherapy and review the best renal replacement modality for each patient.

Keywords: Antithrombin III Deficiency - genetics, Antithrombins - metabolism, Kidney Failure, Chronic - therapy, Kidney Transplantation, Renal Dialysis, Venous Thrombosis - therapy