Cheong H I, Han H W, Park H W, Ha I S, Han K S, Lee H S, Kim S J, Choi Y
Departments of Pediatrics, General Surgery, Clinical Pathology and Pathology, Seoul National University Children's Hospital, Seoul and Department of Pediatrics, Seoul City Boramae Hospital, Seoul, Korea.
Nephrol Dial Transplant. 2000 Jan;15(1):78-81. doi: 10.1093/ndt/15.1.78.
We analysed risk factors to predict the recurrence of nephrotic syndrome and the therapeutic efficacy of plasmapheresis combined with oral cyclophosphamide (PE+CPM) in early recurrent nephrotic syndrome after transplantation in children with focal segmental glomerulosclerosis (FSGS).
Medical records after 1990 of 16 children with biopsy-proven idiopathic FSGS and renal transplantation before the age of 18 years were reviewed.
Early recurrence of nephrotic syndrome developed in six cases (37. 5%). While early kidney graft biopsies, performed within the first week after the onset of recurrence, revealed diffuse effacement of foot process only, late biopsies contained segmentally sclerosed glomeruli as well. Among several possible risk factors, the mean duration from onset of original nephrotic syndrome to development of end-stage renal disease was shorter in the recurrent group (P=0.045) and the percentage of globally sclerosed glomeruli was higher in the non-recurrent group (P=0.001). PE+CPM therapy resulted in complete remission of nephrotic syndrome if it was started early and if there was no evidence of accompanying acute rejection.
These results support more liberal use of living-related donors for renal transplantation of children with FSGS and ESRD, considering the shortage of cadaveric donors in our society and relatively good efficacy of the early and intensive PE+CPM therapy for early recurrent nephrotic syndrome.
我们分析了预测肾病综合征复发的危险因素，以及血浆置换联合口服环磷酰胺（PE+CPM）治疗儿童局灶节段性肾小球硬化（FSGS）移植后早期复发性肾病综合征的疗效。
回顾了1990年后16例经活检证实为特发性FSGS且18岁前接受肾移植儿童的病历。
6例（37.5%）发生肾病综合征早期复发。复发后第一周内进行的早期肾移植活检仅显示足突弥漫性消失，而晚期活检还包含节段性硬化的肾小球。在几个可能的危险因素中，复发组从原发性肾病综合征发病到终末期肾病发展的平均时间较短（P=0.045），非复发组全球硬化肾小球的百分比更高（P=0.001）。如果早期开始且无伴随急性排斥反应的证据，PE+CPM治疗可使肾病综合征完全缓解。
考虑到我们社会中尸体供体短缺以及早期强化PE+CPM治疗对早期复发性肾病综合征的疗效相对较好，这些结果支持更广泛地使用亲属活体供体为FSGS和终末期肾病儿童进行肾移植。
