Malekzadeh M H, Heuser E T, Ettenger R B, Pennisi A J, Uittenbogaart C H, Warshaw B L, Fine R N
J Pediatr. 1979 Aug;95(2):249-54. doi: 10.1016/s0022-3476(79)80660-5.
Eighteen patients with corticosteroid-resistant nephrotic syndrome developed end-stage renal disease and received one or more renal allografts. The lesion of focal segmental glomerulosclerosis and/or of focal glomerular obsolescence was demonstrable in the native kidneys of each patient. Following transplantation, nephrosis developed in three recipients. Two recipients developed nephrosis at two weeks and nine months posttransplant in association with rejection; the lesion of FGS was present in association with chronic rejection. Only one recipient developed recurrence of nephrosis and FGS unrelated to rejection. This was manifested by immediate onset of nephrosis in two successive allografts and histologic evidence of the lesion of FGS. The immediate recurrence in successive allografts suggests a circulating factor responsible for the renal lesion in this patient and indicates a separate etiology for a small number of patients with corticosteroid-resistant nephrosis and FGS.
18例对皮质类固醇耐药的肾病综合征患者发展为终末期肾病,并接受了一个或多个同种异体肾移植。在每位患者的原肾中均证实存在局灶节段性肾小球硬化和/或局灶性肾小球荒废病变。移植后,3名受者发生了肾病。2名受者分别在移植后2周和9个月因排斥反应发生肾病;局灶节段性肾小球硬化病变与慢性排斥反应相关。只有1名受者发生了与排斥反应无关的肾病和局灶节段性肾小球硬化复发。这表现为连续两次同种异体移植均立即出现肾病,并有局灶节段性肾小球硬化病变的组织学证据。连续同种异体移植的立即复发提示存在一种循环因子导致该患者的肾脏病变,并表明少数对皮质类固醇耐药的肾病和局灶节段性肾小球硬化患者存在独立的病因。