Antithrombin III (AT III) deficiency, a coagulation disorder in nephrotic syndrome, which is characterized by increased trombocyte reactivation and loss of urinary protein, particularly leads to increased incidence of thromboembolism. Although arterial thromboembolism is rarely associated with nephrotic syndrome in children, it is one of the leading causes of increased morbidity and mortality. In this article, we present an 18-month-old boy who was admitted to the Emergency Unit in our hospital with acute onset of purple veins under the skin and pain and lack of motion in the left lower limb and diagnosed with AT III deficiency secondary to nephrotic syndrome. The clinical examination revealed motor dysfunction and necrotic tissue changes in the left lower limb. Acute arterial thromboembolism was diagnosed. Clinical prognosis was improved following medical and surgical treatments without the need for amputation of the limb. In this report, the administration of intraarterial tissue plasminogen activator during surgical thromboembolectomy in children, dose and side effects of the therapy and other treatment of choices were discussed.

Keywords : Antithrombin III deficiency; arterial occlusive disease; nephrotic syndrome; thromboemboli