Goonasekera C D, Koziell A B, Hulton S A, Dillon M J
Institute of Child Health and Great Ormond Street Children's Hospital NHS Trust, London, UK.
Pediatr Nephrol. 1998 May;12(4):284-9. doi: 10.1007/s004670050454.
Over the last 10 years, eight children have received vincristine for the treatment of steroid- and cyclophosphamide-resistant nephrotic syndrome at Great Ormond Street Hospital for Children, London. We present our experience of these eight cases and put forward a case for reassessing the effectiveness of vincristine in this disorder. In our series, two children treated with vincristine achieved complete remission with preserved renal function, including relapses in one. Both had primary steroid- and cyclophosphamide-resistant focal segmental glomerulo sclerosis (FSGS). Of the other cases, four also had primary FSGS, one familial FSGS and one mesangioproliferative glomerulonephritis. We discuss in general the pros and cons of vincristine therapy in nephrotic syndrome versus the cytotoxic agents that are currently used and the differences in clinical features among the responders and non-responders in this small group. In addition, we explore why this may have occurred and summarise the literature over the last 25 years, where vincristine appeared to have been beneficial, especially in secondary forms of nephrotic syndrome associated with malignancy. We conclude that vincristine therapy warrants re-examination as it could be a valuable alternative therapeutic agent in some cases of FSGS with relatively minor side effects.
在过去10年里，伦敦大奥蒙德街儿童医院有8名儿童接受长春新碱治疗类固醇和环磷酰胺耐药的肾病综合征。我们介绍这8例病例的经验，并提出重新评估长春新碱对这种疾病有效性的理由。在我们的系列病例中，两名接受长春新碱治疗的儿童实现了完全缓解且肾功能得以保留，其中一名出现复发。两人均患有原发性类固醇和环磷酰胺耐药的局灶节段性肾小球硬化（FSGS）。在其他病例中，4例也患有原发性FSGS，1例为家族性FSGS，1例为系膜增生性肾小球肾炎。我们总体讨论了长春新碱治疗肾病综合征相对于目前使用的细胞毒性药物的利弊，以及这一小群有反应者和无反应者临床特征的差异。此外，我们探究了为何会出现这种情况，并总结了过去25年的文献，其中长春新碱似乎是有益的，尤其是在与恶性肿瘤相关的继发性肾病综合征中。我们得出结论，长春新碱治疗值得重新审视，因为在某些FSGS病例中它可能是一种有价值的替代治疗药物，且副作用相对较小。
