Trachtman Rebecca, Sran Simranjeet S, Trachtman Howard
Division of Pediatric Nephrology, NYU Langone Medical Center, CTSI, Room #733 227 E 30th Street, New York, NY, 10016, USA.
Pediatr Nephrol. 2015 Oct;30(10):1793-802. doi: 10.1007/s00467-015-3062-1. Epub 2015 Feb 19.
Focal segmental glomerulosclerosis (FSGS) is an important cause of glomerular disease in children and adolescents and nearly 50 % of affected patients will progress to end-stage kidney disease over a 5 to 10-year period. Unfortunately, there is no established treatment for disease in the native kidney. Moreover, up to 55 % of patients develop recurrent disease after receiving a kidney transplant, with a substantially higher risk in patients who have already experienced recurrent disease in a prior transplant. A number of clinical and laboratory factors have been identified as risk factors for this complication. In addition, new investigations into podocyte biology and circulating permeability factors have shed light on the cause of recurrent the disease. While a number of novel therapeutic agents have been applied in the management of this problem, there still is no proven treatment. In this review, we summarize recent advances in the epidemiology, pathophysiology, and treatment of recurrent FSGS in pediatric patients who have received a kidney transplant.
局灶节段性肾小球硬化(FSGS)是儿童和青少年肾小球疾病的重要病因,近50%的受累患者将在5至10年内进展为终末期肾病。不幸的是,目前尚无针对原发性肾脏疾病的确立治疗方法。此外,高达55%的患者在接受肾移植后会出现疾病复发,既往移植中已出现复发疾病的患者风险更高。一些临床和实验室因素已被确定为这种并发症的危险因素。此外,对足细胞生物学和循环通透性因子的新研究揭示了疾病复发的原因。虽然一些新型治疗药物已应用于该问题的管理,但仍没有经过验证的治疗方法。在本综述中,我们总结了接受肾移植的儿科患者复发性FSGS在流行病学、病理生理学和治疗方面的最新进展。