Olie Karolien H, Goodship Timothy H J, Verlaak René, Florquin Sandrine, Groothoff Jaap W, Strain Lisa, Weening Jan J, Davin Jean-Claude
Paediatric Nephrology Unit, Emma Children's Hospital/Academic Medical Centre, Amsterdam, The Netherlands.
Am J Kidney Dis. 2005 Jan;45(1):e12-5. doi: 10.1053/j.ajkd.2004.09.012.
Atypical hemolytic uremic syndrome (HUS) can recur after renal transplantation and often leads to graft loss. In some series of familial HUS, the risk of early graft loss due to recurrence of HUS approaches 100% despite any therapy. This led some authors to claim that kidney transplantation is contraindicated in those patients. The authors describe an 8-year-old girl with end-stage renal failure owing to familial atypical HUS with a factor H mutation who underwent successful transplantation using continuous prophylactic plasma exchange (PE). Twenty-four months after transplantation, plasma creatinine level is 1.2 mg/dL (106 micromol/L) despite 2 recurrences of HUS contemporaneous to 2 cytomegalovirus infections, which resolved with PE intensification and ganciclovir. This strongly suggests that cytomegalovirus infection may trigger posttransplant recurrent HUS. The feasibility of kidney transplantation in case of atypical HUS related to factor H mutation using continuous prophylactic PE intensified during relapses should be confirmed in prospective studies.
非典型溶血性尿毒症综合征(HUS)在肾移植后可能复发,并常常导致移植肾丧失。在一些家族性HUS系列研究中,尽管采取了任何治疗措施,因HUS复发导致早期移植肾丧失的风险接近100%。这使得一些作者宣称,肾移植对于这些患者是禁忌的。作者描述了一名8岁的终末期肾衰竭女童,其患有家族性非典型HUS且存在H因子突变,通过持续预防性血浆置换(PE)成功接受了移植手术。移植后24个月,尽管在2次巨细胞病毒感染同时发生了2次HUS复发,但血浆肌酐水平为1.2mg/dL(106μmol/L),通过强化PE和更昔洛韦治疗后病情得到缓解。这有力地表明,巨细胞病毒感染可能触发移植后复发性HUS。前瞻性研究应证实,对于与H因子突变相关的非典型HUS患者,在复发期间使用强化的持续预防性PE进行肾移植的可行性。