Department of Clinical Medicine, Nephrology Unit, Sapienza University of Rome, Rome, Italy.
Semin Thromb Hemost. 2013 Jul;39(5):469-76. doi: 10.1055/s-0033-1343887. Epub 2013 Apr 27.
Nephrotic syndrome (NS) is characterized by heavy proteinuria, edema, hypoalbuminemia, and hyperlipidemia and the most frequent causes are glomerular diseases. An uncommon presentation is iatrogenic NS, an adverse effect of some drugs administration. In the clinical course of NS, a typical feature is dysregulated coagulation state, promoted by the breakdown of permselectivity barrier of the glomerular capillary wall, resulting in the leakage of high-molecular-mass proteins, at least the size of albumin. This hypercoagulable condition is supported by several factors, such as abnormalities in platelet activation and an imbalance between anticoagulation/antithrombosis and procoagulant/prothrombotic mechanisms. Thus, NS and the risk of developing thromboses are strictly related. Thrombotic events affect the venous system rather than arterial vessels with different features and frequencies. Deep venous system of the lower extremities and renal veins are the most frequent source of pulmonary embolism, the most dangerous NS complication. Prophylactic anticoagulation and thrombosis treatment are not clearly established because large randomized trials and guidelines are lacking. The management of NS and the decision of when and how to anticoagulate the patient represent a teamwork challenge for physicians.
肾病综合征（NS）的特征是大量蛋白尿、水肿、低白蛋白血症和高脂血症，最常见的病因是肾小球疾病。不常见的表现是医源性 NS，这是某些药物治疗的不良反应。在 NS 的临床病程中，一个典型的特征是凝血状态失调，这是由肾小球毛细血管壁的选择性屏障破裂引起的，导致高分子质量蛋白漏出，至少漏出白蛋白的大小。这种高凝状态由多种因素支持，如血小板激活异常以及抗凝/抗血栓和促凝/血栓形成机制之间的失衡。因此，NS 和发生血栓的风险密切相关。血栓事件影响静脉系统而不是动脉血管，具有不同的特征和频率。下肢深静脉系统和肾静脉是最常见的肺栓塞来源，也是 NS 最危险的并发症。由于缺乏大型随机试验和指南，预防性抗凝和血栓治疗尚未明确确立。NS 的管理以及决定何时以及如何给患者抗凝是医生团队面临的挑战。
