Schachter A D, Harmon W E
Division of Nephrology, Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Pediatr Transplant. 2001 Dec;5(6):406-9. doi: 10.1034/j.1399-3046.2001.t01-2-00024.x.
Recurrence of nephrotic syndrome (NS) after transplantation (Tx) occurs in 20-50% of renal transplant recipients, with a median time to recurrence of 14 days and a 50% rate of graft loss. We performed a retrospective analysis of 22 pediatric patients with NS who received renal transplants at the Children's Hospital, Boston, between 1982 and 1999. During the first 14 days following Tx, 13 (59%) patients developed clinical recurrent nephrotic syndrome (RNS). RNS developed in 50% of living donor recipients and in 70% of cadaveric donor recipients (p= non-significant). Seven of the 13 patients with RNS were treated with plasmapheresis, while six received standard immunosuppressive induction therapy only. Two of the seven treated patients and one of the six untreated patients lost their grafts to RNS, yielding a total RNS graft loss rate of 23%. However, patients with RNS who achieved remission had significantly higher cumulative graft survival at 5 yr than did RNS patients who did not achieve remission (p< 0.001). Overall cumulative graft survival at 5 yr was not significantly different between the two groups: 67% in those with non-recurrent nephrotic syndrome (NRNS) vs. 64% in those with RNS, p= non-significant. We conclude that successful reversal of early RNS improves long-term graft survival in pediatric RNS. Multi-center studies are sorely needed to develop novel, less toxic therapies for native and recurrent NS.
肾移植(Tx)后肾病综合征(NS)复发见于20% - 50%的肾移植受者,复发的中位时间为14天,移植肾丢失率为50%。我们对1982年至1999年间在波士顿儿童医院接受肾移植的22例NS患儿进行了回顾性分析。在肾移植后的前14天内,13例(59%)患者出现临床复发性肾病综合征(RNS)。活体供肾受者中50%发生RNS,尸体供肾受者中70%发生RNS(p值无统计学意义)。13例RNS患者中有7例接受了血浆置换治疗,而6例仅接受了标准免疫抑制诱导治疗。7例接受治疗的患者中有2例、6例未接受治疗的患者中有1例因RNS导致移植肾丢失,RNS导致的移植肾总丢失率为23%。然而,缓解的RNS患者5年时的累积移植肾存活率显著高于未缓解的RNS患者(p<0.001)。两组患者5年时总体累积移植肾存活率无显著差异:非复发性肾病综合征(NRNS)患者为67%,RNS患者为64%,p值无统计学意义。我们得出结论,早期RNS的成功逆转可提高小儿RNS患者的长期移植肾存活率。迫切需要开展多中心研究,以开发针对原发性和复发性NS的新型、低毒性治疗方法。