Suri Deepti, Ahluwalia Jasmina, Saxena Akshay K, Sodhi Kushaljit S, Singh Paramjeet, Mittal Bhagwant R, Das Reena, Rawat Amit, Singh Surjit
Division of Allergy Immunology and Nephrology, Department of Pediatrics, Advanced Pediatric Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India,
Clin Exp Nephrol. 2014 Oct;18(5):803-13. doi: 10.1007/s10157-013-0917-2. Epub 2013 Dec 18.
Thromboembolism is a rare life-threatening complication of childhood nephrotic syndrome.
We present the clinical profile and outcome of 34 children with 35 events of thromboembolic complications with nephrotic syndrome.
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.
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.
血栓栓塞是儿童肾病综合征罕见但危及生命的并发症。
我们呈现了34例患有35次肾病综合征血栓栓塞并发症事件的儿童的临床资料及预后情况。
脑静脉血栓形成(CVT)是最常见的并发症,11例(31.4%)儿童出现该并发症,其次是肺血栓栓塞和深静脉血栓形成,分别有9例(25.7%)和6例(16.6%)儿童出现。7例(20%)儿童出现导致中枢神经系统梗死的动脉血栓形成,2例儿童出现外周动脉血栓形成。在激素抵抗型肾病综合征和激素依赖型肾病综合征组中,血栓栓塞事件的发生率相同。大多数血栓栓塞并发症发生在复发时,但11.4%的儿童在缓解期出现颅内血栓形成。CVT最敏感的症状是持续性头痛,而不明原因的呼吸窘迫和低氧血症提示肺血栓栓塞。82.8%的儿童出现低白蛋白血症,31.4%的儿童并发感染。发现了遗传性血栓前状态的共存情况,值得评估。早期肝素治疗后口服抗凝剂,91.1%的儿童完全康复。3例(8.5%)儿童死亡,尸检显示2例儿童有肺血栓栓塞。
肾病综合征患儿可发生静脉和动脉血栓并发症。由于临床特征可能不明显,需要高度怀疑。神经影像学和血管造影技术有助于确诊。早期积极的肝素治疗后口服抗凝剂对于获得良好预后是必要的。