Mehls O, Andrassy K, Koderisch J, Herzog U, Ritz E
J Pediatr. 1987 Jun;110(6):862-7. doi: 10.1016/s0022-3476(87)80397-9.
Eleven of 204 children with nephrotic syndrome had thrombotic complications: arterial thrombosis in five, venous thrombosis in four, and pulmonary embolism in two. Fifty-one episodes of thromboembolism were recognized in 116 adult patients with nephrotic syndrome. Despite the lower incidence, thromboembolic complications tended to be more severe in children. In vitro indices of hemostasis and clinical evidence of thromboembolic complications were compared in children and adults. Antithrombin III concentrations and activities were abnormal in seven of 10 children, but in only two of 32 adults. In both groups, alpha 2-macroglobulin was elevated, but more markedly so in children. No evidence for circulating granulocyte-derived proteases (elastase/antielastase complexes) was noted in either group. Protein C was significantly elevated in children with nephrotic syndrome, but was normal in adults. Children also differed from adults with nephrotic syndrome in laboratory evidence of subthreshold disseminated intravascular coagulation (i.e., elevated soluble fibrinogen monomeric complexes and fibrin degradation products) and indicators of in vivo platelet activation (elevated beta-thromboglobulin). The more severe coagulation abnormalities in children may be linked to the more pronounced hypoalbuminemia.
204例肾病综合征患儿中有11例发生血栓并发症:5例为动脉血栓形成,4例为静脉血栓形成,2例为肺栓塞。116例成年肾病综合征患者共发生51次血栓栓塞事件。尽管儿童发病率较低,但血栓栓塞并发症往往更为严重。对儿童和成人的体外止血指标及血栓栓塞并发症的临床证据进行了比较。10例儿童中有7例抗凝血酶III浓度和活性异常,而32例成人中只有2例异常。两组α2-巨球蛋白均升高,但儿童更为明显。两组均未发现循环粒细胞衍生蛋白酶(弹性蛋白酶/抗弹性蛋白酶复合物)的证据。肾病综合征患儿蛋白C显著升高,而成人正常。儿童与成年肾病综合征患者在亚阈值弥散性血管内凝血的实验室证据(即可溶性纤维蛋白原单体复合物和纤维蛋白降解产物升高)及体内血小板活化指标(β-血小板球蛋白升高)方面也存在差异。儿童更严重的凝血异常可能与更明显的低白蛋白血症有关。