423例尸体肾移植患者的长期随访

目的:探讨肾移植患者较理想的免疫抑制治疗方案.方法:收集我院1992年1月以前成功实施的肾移植手术共423例,按免疫抑制方案分为:环孢素(CsA)二联用药组248例、CsA三联用药组70例、硫唑嘌呤 强的松组(Aza Pred)105例.统计分析各治疗组患者及移植肾第三、五、十年的存活率,各组患者心血管疾病、肿瘤、糖尿病等严重并发症的发生率,及各组患者的急性排斥发生率、逆转率和带肾死亡率.结果:423例肾移植患者中,第三、五、十年患者的存活率分别为79.4%、70.9%、58.9%,移植肾存活率为74.0%、60.3%、45.4%.CsA二联组与CsA三联组各阶段的人、肾存活率在统计学上均无明显差异;Aza Pred治疗组在第三、五年的人、肾存活率与前两组相比明显降低(P＜0.05),但十年存活率无显著差异.各组患者在不同时期均有排斥发生,仅以急性排斥计,所有患者的排斥发生率为38.3%.CsA二联组与CsA三联组在急性排斥的发生率上无明显统计学差异;而Aza Pred组与前两组相比,急性排斥的发生率明显增加,逆转率降低.比较三组患者的并发症发生率和带肾死亡率发现,CsA三联组的感染和糖尿病的发生率较其他两组相比显著增高,带肾死亡率也明显增加;Aza组患者带肾死亡率最低.结论:CsA治疗明显提高患者及移植肾的近期存活率,可能与其明显降低急性排斥的发生率、增加急性排斥的逆转率有关.但比较患者和移植肾的长期存活率可见,CsA治疗组(二联与三联治疗)与Aza组无明显不同.     

脑-心双死亡供肾与尸体供肾移植后疗效的比较

目的:比较脑-心双死亡器官捐赠(DBCD)供肾与传统尸体供肾移植的3年疗效差异。方法:回顾性分析四川省人民医院器官移植中心2009年11月至2012年1月的24例DBCD供肾移植受者及同期59例传统尸体肾移植受者,所有受者术后随访3年,比较两组受者肾移植术后围手术期并发症发生率,及3年内感染、排斥反应发生率,术后糖尿病、高血压、高脂血症发生率,术后收缩压、舒张压、胆固醇、三酰甘油、低密度脂蛋白、高密度脂蛋白,术后新发肿瘤发生率、钙调神经蛋白抑制剂(CNI)药物转化率;以及肾移植术后估算的肾小球滤过率(e GFR)情况,人/肾1年、2年、3年存活率等方面的差异。结果:DBCD肾移植组术后移植肾功能恢复延迟(DGF)和感染的发生率为33.3%和37.5%,而传统尸体肾移植组为11.9%和20.3%,差异有统计学意义(P0.05)。术后7d和14d,DBCD肾移植组的e GFR低于传统尸体肾移植组(P0.05),但术后1个月至3年,两组受者的e GFR差异无统计学意义。DBCD肾移植组术后1年、2年和3年的移植人/肾存活率分别为95.8%、87.5%、83.3%和95.8%、83.3%、75.0%,而传统尸体肾移植组分别为98.3%、94.9%、88.1%和96.6%、93.2%、78.0%,两组的差异无统计学意义。结论:DBCD肾移植术后发生DGF及围手术期感染的发生率较高,但其长期疗效与传统尸体肾移植相当。     

术前非供者特异性HLA抗体弱阳性的肾移植受者术后早期肾功能、移植排斥反应、移植肾存活情况

目的 观察术前非供者特异性人类白细胞抗原（HLA）抗体弱阳性的肾移植受者术后早期肾功能、移植排斥反应、移植肾存活情况。方法 接受公民逝世后器官捐献供肾移植的肾移植受者57例，依据肾移植术前HLA抗体检测结果分为HLA抗体弱阳性组26例、HLA抗体阴性组31例。观察受者术后早期肾功能，以术后1周移植物功能延迟恢复（DGF）率表示。对术后怀疑发生排斥反应的受者进行移植肾穿刺病理活检和病理诊断，测算移植排斥反应发生率。术后随访满5年，观察并比较两组受者移植肾5年存活率。结果 移植术后1周，HLA抗体弱阳性组受者DGF率为34.62%(9/26),HLA抗体阴性组受者DGF率为9.68%(3/31)，两组相比，P<0.05。HLA抗体弱阳性组受者移植排斥反应发生率为26.92%(7/26),HLA抗体阴性组受者移植排斥反应发生率为6.45%(2/31)，两组相比，P>0.05。HLA抗体弱阳性组受者移植肾5年存活率为73.08%(19/26),HLA抗体阴性组受者移植肾5年存活率为87.1%(27/31)，两组相比，P>0.05。结论 与HLA抗体阴性受者相比，术前非供者特异...     

心脏死亡器官捐献供肾术前病理改变对肾移植预后的影响

目的:应用Pirani评分系统评估心脏死亡后器官捐献(DCD)供肾的组织病变程度,追踪不同程度病变的供肾对肾移植患者短期预后的影响。方法:(1)研究对象于2015-02-01～2017-02-01在郑州人民医院行DCD供肾移植患者,供肾获取后移植术前行活检病理,即经快速切片后行苏木素-伊红(HE)染色,采用Pirani评分系统评估供肾组织病变程度。(2)根据供肾Pirani病理评分分为两组,低分组(0～3分),高分组(4～6分)。(3)观察指标:比较两组肾移植患者的预后,包括移植肾功能延迟恢复(DGF)、术后1月、12月和24月血清肌酐(SCr)及12～36月累计移植肾存活率。结果:供肾及相应受者共92例纳入本研究,低分组68例(73. 9%),高分组24例(26. 1%)。受体总体DGF发生率为15. 2%,高分组与低分组DGF发生率分别为37. 5%(9/24)和7. 0%(5/68)(P=0. 028)。受者术后1月、12月、24月低分组SCr值分别为(104. 9±16. 8)μmol/L、(94. 9±16. 7)μmol/L、(91. 8±9. 3)μmol/L,高分组分别为(116. 2±34. 4)μmol/L、(116. 5±14. 9)μmol/L、(111. 7±10. 2)μmol/L。低分组SCr两年内各个时间点SCr值均低于高分组(P=0. 041,P=0. 044,P=0. 038,P0. 05)。随访12～36个月,Kaplan-meier分析结果表明高分组与低分组移植肾存活率无明显差异(log-Rank检验,p=0. 499)。结论:DCD供肾病理Pirani评分越高,受者肾移植术后DGF发生率越高,短期移植肾功能越差,但短期内存活率无明显差异。     

肾移植急性排斥患者肾组织C4d的沉积及其意义

目的 :探讨肾组织C4d的沉积在诊断移植肾急性排斥反应 (AR)、指导治疗和判断预后中的作用。　方法 :选择 6 6例肾移植AR患者 ,其中急性间质性排斥 (AIR) 30例 ,急性血管性排斥 (AVR) 36例 ,应用间接免疫荧光法检测肾组织中C4d的沉积 ,并与临床表现、移植肾组织病理改变及治疗和预后的关系进行分析。　 结果 :①正常肾组织肾小管周毛细血管无C4d的沉积。AVR患者肾小管周毛细血管C4d沉积的阳性率明显高于AIR患者 (5 6 %vs6 7% ,P <0 0 1)。②在C4d阳性AVR患者中 ,女性患者明显高于C4d阴性患者 (P <0 0 5 )。群体反应性抗体水平升高的发生率 ,C4d阳性患者也明显高于阴性者 (45 %vs1 2 % ,P <0 0 5 )。③AVR患者C4d阳性者移植肾功能延迟恢复(46 %vs13% ,P <0 0 5 )和移植肾失功的发生率 (35 %vs6 3% ,P <0 0 5 )均明显高于C4d阴性者。④ 4例C4d阳性AVR患者在接受抗排斥治疗后 ,有 3例患者肾功能恢复正常 ,重复肾活检显示肾组织C4d沉积亦随之消失。　 结论 :移植肾组织C4d沉积的发生与排斥反应的类型有关 ,体液免疫反应亢进者发生率高。存在致排斥发生高危因素者发生率高。AR伴肾组织C4d沉积者预后不佳。积极有效的治疗能在逆转病情的同时 ,使肾组织C4d的沉积随之消失。移植肾组织C4d检测对于提高排斥     

新疆地区亲属活体肾移植176例临床疗效分析

目的:总结和分析新疆地区176例活体肾移植的临床疗效和经验。方法:回顾性研究176例活体肾移植受者的术前一般情况、术后急性排斥反应发生率、并发症、人/肾存活率。结果:1999年8月至2008年9月间在新疆本地医院共完成异体肾移植779例,活体肾移植179例(23.0%),但2006年后活体肾移植占同期移植量的69.4%。其中3例失访,资料完整者176例,包括亲属供肾175例,非亲属供肾1例(汉族)。亲属供肾包括直系亲属15例,汉族占66.7%(10/15例);三代旁系157例,少数民族占78.3%(123/157例),夫妻间3例。176例肾移植受者中,少数民族132例,男性144例。1年、3年,5年及5年以上人/肾存活率分别为94.3%/93.2%,92.0%/88.6%,92.0%/88.1%,92.0%/86.9%,急性排斥反应发生率为9.1%,肺部感染18例(10.2%),其中死亡11例(6.3%)。结论:近三年新疆地区活体肾移植呈快速增长,2006年后约占同期肾移植总量的69.4%。患者以少数民族居多(占74.4%),供者多为三代旁系亲属,而汉族患者则以直系亲属供肾为主。本组人/肾短期和中期存活率明显高于尸体肾移植,并与国内、外报道活体肾移植人/肾存活率相近,急性排斥和肺部感染发生率分别为9.1%和10.2%,死亡率为6.3%,其长期存活率有待进一步观察。     

中老年肾移植患者早期撤除激素的免疫抑制方案研究

目的 评价中老年肾移植患者早期撤除激素的安全性及有效性. 方法 80例中老年肾移植患者随机分为撤激素组39例和常规治疗组41例.所有患者开始均采用环孢素A(CsA)+吗替麦考酚酯(MMF)+泼尼松(Pred)三联免疫抑制方案,Pred开始剂量为20 mg/d,撤激素组Pred逐渐减量(每周减量5 mg),术后1个月停用;常规治疗组Pred 3个月后减量为10 mg/d,6个月后减为5 mg/d维持.MMF、CsA起始用量相同.随访观察患者急性排斥反应(AR)发生率、移植肾功能、人存活率、肾存活率,感染情况、血糖、血压、体质量、血脂等指标. 结果 撤激素组、常规治疗组AR发生率相似(分别为23.1%和19.5%,χ~2=0.15,P>0.05).两组患者1、2、3年人存活率分别为97.4%、94.8%、88.0 0A和97.6%、97.6%、87.8%,差异无统计学意义(χ~2=0.1 7,P>0.05);肾存活率分别为94.9%、88.6%、83.7%和95.1%、91.5%、79.5%,差异无统计学意义(χ~2=0.07,P>0.05). 结论 老年肾移植患者早期撤除激素是可行的.     

抗中性粒细胞胞质抗体相关肾炎患者肾移植的转归

目的:回顾性分析抗中性粒细胞胞质抗体(ANCA)相关肾炎(AAGN)患者接受肾移植后的转归。方法:11例AAGN患者[男4例,女7例,中位年龄48(33.5~52.5)岁]接受肾移植。采用激素、吗替麦考酚酯联合他克莫司/环孢素A三联抗排斥治疗。肾移植后排斥反应及其类型均经移植肾活检病理诊断。回顾性分析移植肾预后、血管炎复发及血清ANCA对移植肾预后的影响。结果:11例AAGN均为MPO-ANCA相关血管炎,10例在肾移植前接受肾脏替代治疗,中位时间为30.5(14.8~50.5)个月。肾移植前均无血管炎活动(BVAS 0分),7例血清MPO-ANCA阳性。肾移植术后均未出现移植肾功能延迟恢复,4例(36.4%)发生急性细胞性排斥反应,分别有1例术后18个月和22个月发生血管炎复发和慢性体液性排斥反应。术后中位随访56(46.5~135)个月,随访末8例(72.7%)血清肌酐水平正常,2例血清肌酐升高,1例移植肾失功。5年人、肾生存率分别为100%和90.9%。术前ANCA阳性与阴性患者比较移植肾排斥反应发生率和血管炎复发率均无显著差异。结论:AAGN患者肾脏移植后远期预后好,血管炎复发率低,但术后早期急性排斥反应发生率高,应加强预防抗排斥反应。     

1 118例肾脏移植临床总结

目的 :回顾总结肾脏移植经验、改善临床肾脏移植效果。　 方法 :总结 1992年 7月～ 2 0 0 0年 10月1118例肾脏移植的临床资料 ,研究组织配型、器官摘取情况、移植手术、移植并发症、免疫抑制剂等因素对移植效果的影响。　 结果 :存活率 :术后 1、3、5年人 /肾存活率分别为 93%/ 91 2 %、80 2 %/ 74 5 %、72 %/ 6 6 1%。超急排斥反应发生率 1 2 %,急性排斥反应发生率 2 3 5 %,慢性排斥反应发生率 16 7%。并发症发生率 5 5 %。死亡率16 6 %。　 结论 :①加强供受者组织配型、提高器官摘取以及移植手术质量可以明显提高移植物的存活 ,减少外科并发症的发生 ;②并发症是影响人 /肾长期存活的重要因素 ;③合理应用免疫抑制剂可以延长移植肾的存活 ,减少并发症的发生。     

影响肾移植术后移植肾失功的因素

目的分析影响肾移植手术后移植肾失功的因素。方法接受同种异体肾移植手术的患者共750例,分析移植肾失功率及失功原因。应用Logistic分析影响移植肾失功的独立因素。结果 5年内移植肾失功率为3.87%,存活率为96.13%,肾移植受者的存活率为97.73%;移植肾功能延迟恢复、急性排斥反应、加速性排斥反应、感染,显著影响移植肾的存活率(P0.05)。年龄、免疫抑制方案对移植肾存活的影响无统计学意义(P0.05)。移植肾冷缺血时间、肌酐恢复正常时间、患者1个月内状况(移植肾功能延迟恢复、急性排斥反应、加速性排斥反应、感染)均属影响移植术后移植肾存活的独立因素。结论对肾移植术后患者进行移植肾功能延迟恢复、急性排斥反应、加速性排斥反应、感染的发生进行严格控制,对提高移植肾的存活率具有重要的临床意义。     

Living Donor Kidney Transplantation: Improving Efficiencies in Live Kidney Donor Evaluation–Recommendations from a Consensus Conference

The education, evaluation, and support of living donors before, during, and after donation have historically been considered the roles and responsibilities of transplant programs. Although intended to protect donors, ensure true informed consent, and prevent coercion, this structure often leaves referring nephrologists unclear about the donor process and uncertain regarding the ultimate outcome of potential donors for their patients. The aim of this article is to help the referring nephrologist understand the donor referral and evaluation process, help the referring nephrologist understand the responsibilities of the transplant program, and offer suggestions about how the referring nephrologist can help to improve efficiencies in the process of donor education and evaluation. A partnership between referring nephrologists and transplant programs is an important step in advancing living kidney donation. The referring nephrologists are the frontline providers and are in a unique position to offer education about living donation and improve efficiencies in the process. Understanding the donor referral and evaluation process, the responsibilities of the transplant program, and the potential role referring nephrologists can play in the process is critical to establishing such a partnership.     

同种异体双肾移植8例报告

目的：利用部分“边缘肾”，或因血型特殊局部地区无适合受体的供肾进行双肾移植，探讨双肾移植的l临床疗效。方法：自2001年3月至2002年10月，我们共利用该类“边缘肾”与“无人要的肾脏”为8例患者进行了双肾移植。采用右下腹“L”型切口，来自8个供体的16个肾脏被分别移植于8例受者的右侧髂窝内。免疫抑制方案为皮质激素 霉酚酸酯 环孢素A，剂量同单肾移植。结果：在1-12个月的随访期内发生急性排斥(AR)2例共2次(25％)，1只肾由于肾动脉栓塞失功。5例随访12个月肾功能均正常。结论：双边缘肾移植有助于充分利用日趋紧张的肾源，提供给患者更多的有效肾单位。肾移植的免疫抑制方案，AR发生率，与单肾移植效果相同。     

Clinical and Genetic Analysis of Patients with Cystinuria in the United Kingdom

Background and objectives

Cystinuria is a rare inherited renal stone disease. Mutations in the amino acid exchanger System b^0,+, the two subunits of which are encoded by SLC3A1 and SLC7A9, predominantly underlie this disease. The work analyzed the epidemiology of cystinuria and the influence of mutations in these two genes on disease severity in a United Kingdom cohort.

Design, setting, participants, & measurements

Prevalent patients were studied from 2012 to 2014 in the northeast and southwest of the United Kingdom. Clinical phenotypes were defined, and genetic analysis of SLC3A1 and SLC7A9 combining Sanger sequencing and multiplex ligation probe–dependent amplification was performed.

Results

In total, 76 patients (42 men and 34 women) were studied. All subjects had proven cystine stones. Median age of presentation (first stone episode) was 24 years old, but 21% of patients presented after 40 years old. Patients had varied clinical courses, with 37% of patients having ≥10 stone episodes; 70% had evidence of CKD, and 9% had reached ESRD as a result of cystinuria and its complications. Patients with cystinuria received a variety of different therapies, with no obvious treatment consensus. Notably, 20% of patients had staghorn calculi, with associated impaired renal function in 80% of these patients. Genetic analysis revealed that biallelic mutations were present in either SLC3A1 (n=27) or SLC7A9 (n=20); 22 patients had only one mutated allele detected (SLC3A1 in five patients and SLC7A9 in 17 patients). In total, 37 different mutant variant alleles were identified, including 12 novel mutations; 22% of mutations were caused by large gene rearrangements. No genotype-phenotype association was detected in this cohort.

Conclusions

Patients with cystinuria in the United Kingdom often present atypically with staghorn calculi at ≥40 years old and commonly develop significant renal impairment. There is no association of clinical course with genotype. Treatments directed toward reducing stone burden need to be rationalized and developed to optimize patient care.     

Relationship between cortical and medullary thickness and glomerular filtration rate among living kidney donors

The relationship between the kidney cortex and medulla is not well understood in healthy populations. This study characterised the relationship between cortical/medullary thickness and measured glomerular filtration rate (GFR) in 390 living kidney donors. A positive relationship was observed between medullary, but not cortical, thickness and GFR. We propose that this reflects a correlation between juxtamedullary nephron number and GFR.     

20665例普通人群单次尿液和肾功能检查结果及其意义探讨

目的:了解普通人群健康体检时尿检异常(蛋白尿、血尿、白细胞尿)和肾功能减退的检出率,探讨其临床意义。方法:筛查2008-08-12复旦大学附属中山医院健康体检人群的尿液和肾功能检查资料。肾功能减退标准为血清肌酐mg/dl(μmol/L):(1)KEEP标准男性1.5(133),女性1.3(115);(2)NHANES Ⅲ标准男性1.4(124),女性1.2(106),老年(≥65岁)男性≥1.3(115),女性≥1.0(88.4)。结果:共20 665例,其中男性13 320例(64.46%),女性7 345例(35.54%)。尿检异常检出率5.27%,其中男性3.64%,女性8.22%(P0.01);蛋白尿2.68%,男性2.72%,女性2.60%,P0.01;血尿2.41%,男性1.47%,女性4.11%,P0.01;白细胞尿1.71%,男性0.46%,女性4.00%,P0.01。按照KEEP和NHANES Ⅲ标准,肾功能减退检出率分别为0.26%和0.76%,男性和女性分别为0.20%和0.38%(P0.05),0.35%和0.98%(P0.01)。尿检异常人群肾功能减退的检出率显著高于尿检正常人群(KEEP标准1.3%和0.2%,P0.01;NHANES Ⅲ标准2.0%和0.5%,P0.01);肾功能减退人群尿检异常的检出率显著高于肾功能正常人群(KEEP标准25.9%和5.2%,P0.01;NHANES Ⅲ标准18.6%和5.2%,P0.01)。结论:普通人群尿检异常和肾功能减退检出率较高。在健康体检中应重视尿液和肾功能检查,以提高肾脏病的检出率。     

Forced expression of laminin beta1 in podocytes prevents nephrotic syndrome in mice lacking laminin beta2, a model for Pierson syndrome

Pierson syndrome is a congenital nephrotic syndrome with ocular and neurological defects caused by mutations in LAMB2, the gene encoding the basement membrane protein laminin β2 (Lamβ2). It is the kidney glomerular basement membrane (GBM) that is defective in Pierson syndrome, as Lamβ2 is a component of laminin-521 (LM-521; α5β2γ1), the major laminin in the mature GBM. In both Pierson syndrome and the Lamb2(-/-) mouse model for this disease, laminin β1 (Lamβ1), a structurally similar homolog of Lamβ2, is marginally increased in the GBM, but it fails to fully compensate for the loss of Lamβ2, leading to the filtration barrier defects and nephrotic syndrome. Here we generated several lines of Lamβ1 transgenic mice and used them to show that podocyte-specific Lamβ1 expression in Lamb2(-/-) mice abrogates the development of nephrotic syndrome, correlating with a greatly extended lifespan. In addition, the more Lamβ1 was expressed, the less urinary albumin was excreted. Transgenic Lamβ1 expression increased the level of Lamα5 in the GBM of rescued mice, consistent with the desired increased deposition of laminin-511 (α5β1γ1) trimers. Ultrastructural analysis revealed occasional knob-like subepithelial GBM thickening but intact podocyte foot processes in aged rescued mice. These results suggest the possibility that up-regulation of LAMB1 in podocytes, should it become achievable, would likely lessen the severity of nephrotic syndrome in patients carrying LAMB2 mutations.     

Different Aspects of Kidney Function in Well-Controlled Congenital Hypothyroidism

Objective: Congenital hypothyroidism (CH) increases the prevalence of kidney and urogenital malformations. There are limited studies considering different aspects of kidney function in well-controlled CH patients. We evaluated some features of kidney function in euthyroid children with CH who have been receiving thyroxine hormone since early life.Methods: This cross-sectional study was conducted in Isfahan, Iran, on 74 children aged 2-15 years old (36 CH patients and 38 healthy children). Inclusion criteria for CH patients were euthyroidism at the time of the survey and initiation of replacement therapy during the early neonatal period. Kidney ultrasound evaluation was performed in all participants. Serum biochemistry included urea, creatinine, sodium (Na), potassium (K), magnesium, calcium, and cystatin C levels. Urine electrolytes, fraction excretion (FE) of electrolytes and microalbumin, and glomerular filtration rate (GFR) were also determined.Results: The male/female ratio was 0.8/1 and 1.5/1 in the patient and control groups, respectively. Mean age and height did not differ significantly between the two groups. Ultrasound evaluation of the kidney revealed that the anteroposterior diameter of the right kidney was significantly higher in CH patients as compared to healthy subjects. No significant difference was observed between GFRs in patients with CH and healthy children. The mean values for FENa and FEK were significantly higher in the patient group.Conclusions: Increased FENa and FEK may be a manifestation of impaired tubular maturation in CH. More longitudinal studies are needed to evaluate kidney function in CH patients. Conflict of interest:None declared.