Lilova M I, Velkovski I G, Topalov I B
University Children's Hospital, Clinic of Pediatric Nephrology, Sofia, Bulgaria.
Pediatr Nephrol. 2000 Nov;15(1-2):74-8. doi: 10.1007/s004679900253.
Over a period of 22 years, 447 children with nephrotic syndrome (NS) have been retrospectively studied for clinically apparent thromboembolic complications (TEC). The incidence of TEC is 2% (9/447); 16 clinically apparent TEC were registered in 9 children. The incidence of TEC was 1.5% among patients with steroid-sensitive NS and 3.8% among those with steroid-resistant NS. TEC were predominantly venous (81% venous vs. 19% arterial). The most commonly affected vessels were deep leg veins, followed by inferior vena cava (IVC). Rare locations of TEC were also observe--superior vena cava (SVC), mesenteric artery, IVC, and hepatic veins with the development of Budd-Chiari syndrome. Depending on the location of the TEC, imaging techniques used were: X-ray, computed tomography, and Doppler sonography. The major iatrogenic risk factor was furosemide, administered to 7 of the 9 children with TEC. In some patients additional predisposing factors were infections, dehydration, trauma, venepuncture, and immobilization. Treatment with heparin was followed by oral anticoagulation. Fibrinolytic therapy was effective in 3 of 4 patients. No new TEC occurred under oral anticoagulant prophylaxis. The outcome was a full recovery in 6 patients and a partial recovery in 1 patient. Two children died--1 with SVC thrombosis and the other with recurrent TEC affecting cerebral vessels. Although rare, TEC are among the most serious life-threatening complications in children with NS and require intensive care.
在22年的时间里，对447例肾病综合征（NS）患儿进行了回顾性研究，以观察临床上明显的血栓栓塞并发症（TEC）。TEC的发生率为2%（9/447）；9名患儿共出现16例明显的TEC。激素敏感型NS患者中TEC的发生率为1.5%，激素抵抗型NS患者中为3.8%。TEC主要为静脉血栓（静脉血栓占81%，动脉血栓占19%）。最常受累的血管是下肢深静脉，其次是下腔静脉（IVC）。也观察到TEC的罕见部位——上腔静脉（SVC）、肠系膜动脉、IVC和肝静脉，并伴有布加综合征的发生。根据TEC的部位，使用的成像技术有：X线、计算机断层扫描和多普勒超声检查。主要的医源性危险因素是速尿，9例TEC患儿中有7例使用过。在一些患者中，其他诱发因素包括感染、脱水、创伤、静脉穿刺和制动。肝素治疗后采用口服抗凝治疗。4例患者中有3例接受纤维蛋白溶解治疗有效。口服抗凝预防期间未出现新的TEC。6例患者完全康复，1例部分康复。2名患儿死亡——1例死于SVC血栓形成，另1例死于复发性TEC累及脑血管。尽管罕见，但TEC是NS患儿最严重的危及生命的并发症之一，需要重症监护。
