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先天性肾脏和尿路畸形(CAKUT)及非CAKUT病因所致慢性肾病患儿的肾移植:他们的预后相同吗?

Kidney transplantation in children with CAKUT and non-CAKUT causes of chronic kidney disease: Do they have the same outcomes?

作者信息

Monteverde Marta Lidia, Paz Marcos, Ibáñez Juan Pedro, Chaparro Alicia Beatriz, Solernou Veronica, Sager Cristian, Tessi Catalina, Locane Fabrizio

机构信息

Nephrology Unit, Hospital de Pediatría JP Garrahan, Buenos Aires, Argentina.

Pathology Unit, Hospital de Pediatría JP Garrahan, Buenos Aires, Argentina.

出版信息

Pediatr Transplant. 2020 Dec;24(8):e13763. doi: 10.1111/petr.13763. Epub 2020 Oct 4.

DOI:10.1111/petr.13763
PMID:33012072
Abstract

Almost half the children who undergo kidney transplantation (KTx) have congenital abnormalities of the kidney and urinary tract (CAKUT). We compared patient, graft survival, and kidney function at last follow-up between CAKUT and non-CAKUT patients after KTx. We divided the analysis into two eras: 1988-2000 and 2001-2019. Of 923 patients, 52% had CAKUT and 48% non-CAKUT chronic kidney disease (CKD). Of the latter, 341 (77%) had glomerular disease, most frequently typical HUS (32%) and primary FSGS (27%); 102 had non-glomerular disease. CAKUT patients were more often boys, younger at KTx, transplanted more frequently preemptively, but with longer time on chronic dialysis. They had less delayed graft function (DGF) and better eGFR, but higher incidence of urinary tract infection (1 year post-KTx). In both eras, 1-, 5-, and 10-year patient survival was similar in the groups, but graft survival was better in CAKUT recipients vs those with primary glomerular and primary recurrent glomerular disease: Era 1, 92.3%, 80.7%, and 63.6% vs 86.9%, 70.6%, and 49.5% (P = .02), and 76.7%, 56.6%, and 34% (P = .0003); Era 2, 96.2%, 88%, and 73.5% vs 90.3%, 76.1%, and 61% (P = .0075) and 75.4%, 54%, and 25.2% (P < .0001), respectively. Main predictors of graft loss were DGF, late acute rejection (AR), and age at KTx in CAKUT group and disease relapse, DGF, early AR, and number of HLA mismatches in recipients with glomerular disease. Graft survival was better in CAKUT patients. DGF was the main predictor of graft loss in all groups. Disease recurrence and early AR predicted graft failure in patients with glomerular disease.

摘要

接受肾移植(KTx)的儿童中,近一半患有先天性肾脏和尿路异常(CAKUT)。我们比较了KTx后CAKUT患者和非CAKUT患者在最后一次随访时的患者生存率、移植物生存率和肾功能。我们将分析分为两个时期:1988 - 2000年和2001 - 2019年。在923例患者中,52%患有CAKUT,48%患有非CAKUT慢性肾脏病(CKD)。在后者中,341例(77%)患有肾小球疾病,最常见的是典型溶血尿毒综合征(32%)和原发性局灶节段性肾小球硬化(27%);102例患有非肾小球疾病。CAKUT患者男孩更多,KTx时年龄更小,更多地进行了抢先移植,但慢性透析时间更长。他们的移植肾功能延迟(DGF)较少,估算肾小球滤过率(eGFR)较好,但尿路感染者的发生率更高(KTx后1年)。在两个时期,两组的1年、5年和10年患者生存率相似,但CAKUT受者的移植物生存率高于原发性肾小球疾病和原发性复发性肾小球疾病受者:时期1,分别为92.3%、80.7%和63.6%,对比86.9%、70.6%和49.5%(P = 0.02),以及76.7%、56.6%和34%(P = 0.0003);时期2,分别为96.2%、88%和73.5%,对比90.3%、76.1%和61%(P = 0.0075)以及75.4%、54%和25.2%(P < 0.0001)。CAKUT组移植物丢失的主要预测因素是DGF、晚期急性排斥反应(AR)和KTx时的年龄,而肾小球疾病受者中疾病复发、DGF、早期AR和HLA错配数是移植物丢失的预测因素。CAKUT患者的移植物生存率更好。DGF是所有组中移植物丢失的主要预测因素。疾病复发和早期AR预测了肾小球疾病患者的移植物失败。

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