Thromboembolic complications in childhood nephrotic syndrome: a clinical profile

Abstract

Background: Thromboembolism is a rare life-threatening complication of childhood nephrotic syndrome.

Methods: We present the clinical profile and outcome of 34 children with 35 events of thromboembolic complications with nephrotic syndrome.

Results: Cerebral venous thrombosis (CVT) was the commonest complication seen in 11 (31.4 %) children followed by pulmonary thromboembolism and deep venous thrombosis in 9 (25.7 %) and 6 (16.6 %) children, respectively. Arterial thrombosis resulting in central nervous system infarcts was observed in 7 (20 %) children and 2 children had thrombosis of the peripheral arteries. Episodes were equal in steroid-resistant nephrotic syndrome and steroid-dependent nephrotic syndrome groups. Most of the thromboembolic complications occurred with relapse but 11.4 % of children developed intracranial thrombosis during remission. The most sensitive symptom of CVT was persistent headache while unexplained respiratory distress and hypoxemia pointed towards pulmonary thromboembolism. Hypoalbuminemia was seen in 82.8 % of children, while concurrent infection was seen in 31.4 %. Coexistence of genetic prothrombotic condition was identified and merits evaluation. Early heparin therapy followed by oral anticoagulants resulted in complete recovery in 91.1 % of children. Death occurred in 3 (8.5 %) children and autopsy revealed pulmonary thromboembolism in 2 children.

Conclusion: Venous and arterial thrombotic complications can occur in children with nephrotic syndrome. A high index of suspicion is required as the clinical features may be subtle. Neuroimaging and angiographic techniques help in confirming diagnosis. Early aggressive heparin therapy followed by oral anticoagulants is necessary for a favorable outcome.