难治性肾病综合征的治疗及其进展

The Development and Treatment of Refractory Nephrotic Syndrome

难治性肾病综合征是原发性肾病综合征中频繁复发、激素依赖和耐药病例的总称。其病理类型多数为系膜毛细血管性肾炎、膜性肾病、局灶节段性硬化性肾炎，由于该病本身存在血液高凝状态、脂质代谢紊乱及免疫力低下，激素使用耐药，且多数已加用免疫抑制剂但疗效不理想，同时免疫抑制剂又使免疫力进一步降低且易引起骨髓抑制及肝肾功能损害，属于目前临床治疗最为棘手和预后差的病种之一。

Refractory nephrotic syndrome is a frequent and relapse, steroid-dependent and drug-resistant cases collectively in primary nephrotic syndrome. The majority of its pathological type mesangial capillary glomerulanephritis, membranous nephropathy, focal segmental sclerosing glomerulanephritis, because of RNS exist hypercoagulability of blood,lipid metabolic disorders and immunocompromised, the use of hormone-resistant medicine,and the majority have been added but the efficacy of immunosuppressive agents is not satisfaetory,immunosupprossive agents at the same time makes a further reduction in immunity and easy to cause bone marrow suppression and liver and kidney dysfunction, which is currently the most difficult and the clinical treatment of disease and poor prognosis.