A successful kidney transplantation in case of completely occluded inferior vena cava and iliac veins: a case of inherited antithrombin deficiency

Abstract

Produced in the liver, Antithrombin III, now simply antithrombin (AT), is a vitamin K-independent serine protease inhibitor in the coagulation pathway. It is the most important primary physiologic inhibitor of thrombin in the human body. In addition to thrombin, AT also inhibits other coagulation serine proteases including VIIa, IXa, Xa, XIa, XIIa. The deficiency of AT may be inherited or acquired. The incidence of inherited AT deficiency is about 1:2000-5000 in general population and is the least common of the three main anticoagulant deficiencies (the other two being protein C deficiency and protein S deficiency). Its inheritance is generally in autosomal dominant fashion. The resultant procoagulant state leads to unprovoked recurrent venous thromboses and thromboembolic events such as deep vein thrombosis or pulmonary embolism which generally appear at the post-pubertal period, compared to the very low occurrence in the prepubertal period. The diagnosis is based on both quantitative and qualitative measurement of AT level. The measured AT activity in functional tests in healthy subjects is generally around 80% to 120%, and AT level of less than 70% is considered as being AT deficiency. This evaluation should be made while not on anticoagulation therapy, because heparin decreases AT levels for up to 10 days following its discontinuation and warfarin increases its level. Patients with inherited AT deficiency rarely develops renal failure which may be caused by renal vein thrombosis or glomerular injury associated with fibrin accumulation. In these young patients with end stage renal disease (ESRD), renal transplantation is currently the best therapeutic option to improve the quality of life and to avoid the risk of complications of other renal replacement treatment particularly thrombosis of the hemodialysis access. Kidney transplantation in patients with an occluded iliac veins and inferior vena cava (IVC) is a very challenging surgery to perform. Despite the reported success in few cases for children, kidney transplant surgeries with thrombotic diseases in adults remain very limited in the literature. Herein, we present a successful kidney transplantation by using a polytetrafluoroethylene (PTFE) graft in a young male patient with AT deficiency associated with totally occluded IVC and iliac veins.