Straatmann Caroline, Kallash Mahmoud, Killackey Mary, Iorember Franca, Aviles Diego, Bamgbola Oluwatoyin, Carson Thomas, Florman Sander, Vehaskari Matti V
Department of Pediatrics, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, NY, USA.
Pediatr Transplant. 2014 Feb;18(1):29-34. doi: 10.1111/petr.12185. Epub 2013 Nov 25.
FSGS recurs in approximately 30% of transplanted kidneys and may lead to graft loss. We retrospectively examined the efficacy of early and intensive PP without additional IS in pediatric kidney transplant patients with recurrent FSGS at our center. Seven of 24 patients (29%) had nephrotic proteinuria and histologic evidence of FSGS recurrence within 1-5 days post-transplantation. PP was initiated early after transplantation and initially performed daily until sustained decline in proteinuria. PP frequency was then individually tapered according to proteinuria. Recurrent FSGS in all seven patients responded to a four- to 32-wk course of PP. Two of seven patients had a second recurrence of FSGS, and both recurrences remitted after an additional 3-6 wk of PP. Median observation period was 4.5 yr (0.8-16.3 yr). Complete remission of recurrent FSGS has been sustained in all seven patients, and all patients have stable graft function with recent plasma creatinine <1.5 mg/dL in six of seven patients. Most recent urine protein/creatinine is 0.13-0.61 mg/mg in six of seven patients. One patient has heavy proteinuria secondary to chronic allograft nephropathy 16 yr post-transplant. Intensive and prolonged PP, when initiated early in the post-operative period, is effective in treating recurrent FSGS and preventing graft loss without the use of additional immunosuppressants.
局灶节段性肾小球硬化(FSGS)在约30%的移植肾中复发,并可能导致移植肾丧失。我们回顾性研究了在我们中心接受肾移植的小儿复发性FSGS患者中,早期强化血浆置换(PP)且不额外使用免疫抑制剂(IS)的疗效。24例患者中有7例(29%)在移植后1 - 5天内出现肾病性蛋白尿及FSGS复发的组织学证据。PP在移植后早期开始,最初每天进行,直至蛋白尿持续下降。然后根据蛋白尿情况个体化调整PP频率。所有7例患者的复发性FSGS对4至32周疗程的PP均有反应。7例患者中有2例FSGS再次复发,再次复发后经额外3至6周的PP治疗均缓解。中位观察期为4.5年(0.8至16.3年)。所有7例患者复发性FSGS均持续完全缓解,所有患者移植肾功能稳定,7例患者中有6例近期血浆肌酐<1.5mg/dL。7例患者中有6例最近的尿蛋白/肌酐为0.13至0.61mg/mg。1例患者在移植后16年因慢性移植肾肾病出现大量蛋白尿。术后早期开始的强化和延长的PP在不使用额外免疫抑制剂的情况下,对治疗复发性FSGS和预防移植肾丧失有效。