Kim M S, Stablein D, Harmon W E
Department of Pediatrics, Boston Medical Center and Boston University School of Medicine, Massachusetts, USA.
Pediatr Transplant. 1998 Nov;2(4):305-8.
The database of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) was examined to identify factors that contribute to the poor transplant outcome rate seen in patients with Congenital Nephrotic Syndrome (CNS) (1). Between January 1, 1987 and January, 1997, 132 transplant recipients with the primary diagnosis of CNS were registered. Analysis of the index renal transplants for 78 living donor transplants (LDTx) and 54 cadaver transplants (CADTx) revealed a graft failure rate of 20.5% and 50.0%, respectively. A proportional hazards regression analysis of the CNS patients indicated that cadaver donor source (relative risk increase of 3.9, p<0.001) and recipient age less than 2 years of age (relative risk increase of 2.6, p=0.002) were simultaneous significant predictors of poor graft survival. Patients with CNS demonstrated decreased graft survival compared to the remainder of the registry adjusted for age and donor source (p=0.04). Graft failures were attributed to vascular thrombosis (26%), patient death with functioning graft (23%), chronic rejection (21%) and acute rejection (19%). Graft failure attributed to thrombosis occurred more frequently in CNS patients than in patients with other primary diseases (8.3% vs. 2.9%, p=0.002). Graft failure due to patient death with a functioning graft also occurred more frequently in CNS patients than in patients with other primary diseases (7.5% vs. 2.6%, p<0.003). Infections were the causes of death in 50% (5 of 10) of CNS patients with a functioning graft. Infection as a cause of death with functioning grafts was significantly greater in CNS patients (3.8%) than the rest of the registry (0.8%, p<0.006). We conclude that there is a high rate of renal graft failure in pediatric patients with CNS. Vascular thrombosis and death with a functioning graft were more frequent in patients with CNS compared to patients with other primary diseases. Care should be taken to eliminate risk factors for hypercoagulability and infections prior to transplantation in children with CNS.
对北美儿科肾移植协作研究（NAPRTCS）数据库进行了检查，以确定导致先天性肾病综合征（CNS）患者移植结果较差的因素（1）。在1987年1月1日至1997年1月期间，登记了132例原发性诊断为CNS的移植受者。对78例活体供肾移植（LDTx）和54例尸体供肾移植（CADTx）的首次肾移植分析显示，移植失败率分别为20.5%和50.0%。对CNS患者进行的比例风险回归分析表明，尸体供肾来源（相对风险增加3.9，p<0.001）和受者年龄小于2岁（相对风险增加2.6，p=0.002）是移植存活不良的同时显著预测因素。与根据年龄和供肾来源调整后的登记处其他患者相比，CNS患者的移植存活率降低（p=0.04）。移植失败归因于血管血栓形成（26%）、移植肾仍有功能时患者死亡（23%）、慢性排斥反应（21%）和急性排斥反应（19%）。CNS患者中因血栓形成导致的移植失败比其他原发性疾病患者更常见（8.3%对2.9%，p=0.002）。移植肾仍有功能时因患者死亡导致的移植失败在CNS患者中也比其他原发性疾病患者更常见（7.5%对2.6%，p<0.003）。感染是10例移植肾仍有功能的CNS患者中50%（5例）的死亡原因。移植肾仍有功能时感染作为死亡原因在CNS患者中（3.8%）明显高于登记处其他患者（0.8%，p<0.006）。我们得出结论，CNS儿科患者的肾移植失败率很高。与其他原发性疾病患者相比，CNS患者中血管血栓形成和移植肾仍有功能时患者死亡更为常见。对于患有CNS的儿童，在移植前应注意消除高凝状态和感染的危险因素。
