Saeed Bassam, Mazloum Houda, Askar Munaf
Pediatric Nephrology Department, Kidney Hospital, Damascus, Syria.
Saudi J Kidney Dis Transpl. 2011 Nov;22(6):1219-22.
A 12-year-old girl with a history of steroid and cyclosporine (CsA) resistant nephrotic syndrome owing to focal and segmental glomerulosclerosis (FSGS) has progressed to end-stage renal disease (ESRD) for which she underwent hemodialysis for 18 months before she successfully received a fully matched kidney transplant from her sister at the age of nine years. The post transplantation (Tx) period was marked by an early and massive proteinuria indicating recurrent FSGS for which she received 12 sessions of plasmapheresis (PP); unfortunately, she did not appear to have any response to the PP therapy; thereafter, a conservative management comprising essentially enalapril and losartan has been initiated and was also not successful during the first four months, however, a very gradual response has been noticed to occur after five months of conservative therapy and ultimately, the patient attained complete remission after 21 months of treatment. Amazingly, 15 months after discontinuation of enalapril and losartan, she remained in a complete and sustained remission with a good renal allograft function. To the best of our knowledge, this is the first case ever reported in the literature of a "spontaneous" remission of post transplant recurrent FSGS.
一名12岁女孩，因局灶节段性肾小球硬化（FSGS）导致类固醇和环孢素（CsA）抵抗性肾病综合征，已进展至终末期肾病（ESRD），在9岁时成功接受来自其姐姐的完全匹配肾脏移植前，她接受了18个月的血液透析。移植后阶段的特征是早期出现大量蛋白尿，提示FSGS复发，为此她接受了12次血浆置换（PP）；不幸的是，她似乎对PP治疗没有任何反应；此后，开始了以依那普利和氯沙坦为主的保守治疗，在前四个月也未成功，然而，在保守治疗五个月后，注意到有非常缓慢的反应出现，最终，患者在治疗21个月后完全缓解。令人惊讶的是，停用依那普利和氯沙坦15个月后，她仍处于完全且持续的缓解状态，移植肾肾功能良好。据我们所知，这是文献中首次报道的移植后复发性FSGS“自发”缓解的病例。
