Alport综合征肾移植11例报告

Outcome of renal transplantation in patients with Alport syndrome： report of 11 cases

目的评估Alport综合征患者肾移植术后的预后情况。方法2009年3月至2016年2月间11例Alport综合征患者施行肾移植术，其中男性10例（91％），女性1例（9％），年龄为（23．5±5．1）岁（13-29岁）。供者中男性7例（64％），女性4例（36％），年龄为（38．9±12．3）岁（23～54岁）。供肾来源亲属为6例（55％）。在免疫诱导方案中，使用巴利昔单抗（商品名：舒莱）诱导6例（55％），使用抗胸腺细胞球蛋白（ATG）者2例（18％），未诱导者3例（27％）。术后采用他克莫司＋吗替麦考酚酯＋皮质激素预防排斥反应。移植肾排斥反应的诊断依据移植肾肾穿活检证实。移植肾功能恢复延迟（DC-F）定义为术后第1周内需要透析治疗。随访时间中位数为44个月。同时，根据肾移植受者基本信息及供者情况，以1：2匹配设立非Alport综合征患者对照组（n=22例），比较两组间移植肾恢复及预后情况。结果Alport综合征患者肾移植后移植肾存活率及人存活率均为100％，移植肾排斥反应发生率为18．2％（2例受者中1例为急性T淋巴细胞介导的排斥反应，1例为慢性体液性排斥反应），DGF发生率为9．1％（1例）。有1例患者（9．1％）发生了移植肾多瘤病毒相关性肾病。肾移植术后监测抗肾小球基底膜（GBM）抗体均为阴性（7例），本研究Alport综合征患者肾移植术后均未发生抗GBM肾炎。截止末次随访，平均血肌酐水平为（115．7±32．4）μmol／L。对照组中，移植肾存活率及人存活率均为100％，急性排斥反应发生率为18．2％，DGF发生率为4．5％，与Alport综合征组比较，未见显著性差异。对照组末次随访血肌酐水平为（134．1±76．2）μmol／L。结论肾移植是Alport综合征尿毒症患者行之有效的治疗手段。肾移植术后发生抗GBM肾炎少见，但仍需密切监测随访。

Objective To evaluate the outcomes of renal transplantation in patients with Alport syndrome. Methods A total of 11 patients （male/female.. 10[91%]/1[9%]） with Alport syndrome underwent kidney transplantation at our center from March 2009 to June 2014. Mean ages of patients and dolaors （living： 6/11, 55%） were 23. 5 ± 5.1 years （range, 13 to 29 years） and 38. 9 ± 12. 3 years （range, 23 to 54 years）, respectively. As induction therapy, basiliximab was used for 6（55%） （20 mg,day 0 and 4）, anti-thymocyte globulin for 2 （18%） and none of induction therapy for 3 （27%）. Tacrolimus plus mycophenolic acid plus glucocorticoid was used as immunosuppressive therapy. Acute rejection was diagnosed using biopsy. Need for dialysis at the first postoperative week was accepted as delayed graft function （DGF）. Median follow-up duration was 44 months. Patients in our study group were compared with a control renal transplant group （n = 22） that had similar demographic factors but without Alportsyndrome. Results Graft survival rate and patient survival rate were 100%, and acute rejection rate was 18. 2% （2 cases; 1 was cellular and 1 in 1 （9. 1%） case, and 1 patient （9. 1%） had BK virus serum creatinine level inAlport syndrome group was was antibody-mediated）. DGF was observed nephropathy. At the recent follow-up, mean （115. 7 ± 32. 4） μmol/L. Anti-glomerular basement membrane （GBM） antibody was negative for 7 patients during follow-up. None of anti-GBM nephritis was observed. In the control group, graft survival rate and patient survival rate were also 100%. Acute rejection rate （18. 2%） and DGF rate （4. 5%） were not different between Alport syndrome group and control group. The mean serum creatininein control group was （134. 1 ± 76. 2） μmol/L. Conclusion Despite the risk of anti-GBM nephritis, renal transplantation is a successful treatment modality for end-stage renal disease related to Alport syndrome.