Baum Michelle A
Nephrology Division, Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
Pediatr Transplant. 2004 Aug;8(4):329-33. doi: 10.1111/j.1399-3046.2004.00181.x.
Focal segmental glomerulosclerosis (FSGS) is the primary diagnosis resulting in end-stage renal disease in approximately 12% of children receiving renal transplantation. Recurrent FSGS after transplantation is unpredictable and clear risk factors have not been identified. Post-transplantation, the incidence of acute tubular necrosis requiring dialysis is higher in children with FSGS compared with other diagnoses and may represent immediate severe recurrence. Graft survival is decreased in children with FSGS compared with other primary diagnoses, and the impact is greatest in recipients of living donor transplants. Graft loss caused by recurrent FSGS is significantly higher in living donor transplants compared with cadaveric donor transplants in children. Compared with adults, the impact of FSGS on graft survival appears to be greatest in children. White recipient race is associated with a higher risk of graft loss from recurrent FSGS. Efforts to elucidate the mechanisms of recurrent FSGS and to understand risk factors based on genetics, potential circulating cytokines and permeability factors, age and race must move forward before we can significantly impact outcomes in renal transplantation for FSGS.
局灶节段性肾小球硬化(FSGS)是约12%接受肾移植的儿童终末期肾病的主要诊断病因。移植后复发性FSGS不可预测,且尚未明确其确切的危险因素。移植后,FSGS患儿发生需要透析的急性肾小管坏死的发生率高于其他诊断情况,这可能代表着即刻的严重复发。与其他原发性诊断相比,FSGS患儿的移植肾存活率降低,且对活体供肾移植受者的影响最大。在儿童中,活体供肾移植因复发性FSGS导致的移植肾丢失显著高于尸体供肾移植。与成人相比,FSGS对移植肾存活的影响在儿童中似乎最大。白人受者发生复发性FSGS导致移植肾丢失的风险更高。在我们能够显著改善FSGS肾移植的预后之前,必须推进对复发性FSGS机制的阐明,并基于遗传学、潜在的循环细胞因子和通透性因子、年龄和种族来了解危险因素。