Paglialonga Fabio, Ardissino Gianluigi, Consolo Silvia, Ghio Luciana, Belingheri Mirco, Testa Sara, Biasuzzi Antonietta, Edefonti Alberto
Pediatric Nephrology and Dialysis Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy -
Pediatric Nephrology and Dialysis Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Minerva Pediatr. 2017 Apr;69(2):113-120. doi: 10.23736/S0026-4946.16.04152-9. Epub 2014 Nov 20.
Plasma-exchange (PEX) has been well described in pediatrics, but most of the current indications are derived from adult experience. Aim of the study was to review the PEX treatments in our Unit over a six-year period.
Three hundred and seventy-seven PEX sessions were performed in 38 patients (median age 12.1 years, range 0.6-20.5). Double-needle and single-needle PEX combined with hemodialysis and PEX combined with ultrafiltration were performed in 9, 1 and 3 patients respectively. The most common indications to PEX were atypical hemolytic uremic syndrome (aHUS, 9 patients), focal segmental glomerulosclerosis (FSGS, 9 cases), antibody mediated rejection (AMR) in renal transplant (rTx) recipients (8 patients) and hyperimmunization in patients waiting for rTx (4 cases).
We treated five patients with aHUS on native kidneys with PEX only, with complete remission in 4/6 recurrences; PEX was also successfully used to prevent HUS relapse in three patients undergoing rTx. Only one partial remission was obtained in four patients with FSGS on native kidneys, by means of treatment protocols based on PEX and immunosuppressants; conversely, a partial remission was observed in 6/6 patients with recurrence of FSGS on rTx. Immunosuppressive protocols combined with PEX proved useful in sensitized cadaveric rTx recipients (2/4 successfully transplanted), but failed in 6 patients with chronic AMR. As regards complications, two severe adverse reactions occurred: an anaphylactic shock after the use of albumin and an abdominal hemorrhage.
PEX is a relatively safe procedure in children. Pediatric patients with aHUS, recurrent FSGS and sensitized rTx recipients seem to benefit from treatment strategies including PEX.
血浆置换（PEX）在儿科已有充分描述，但目前大多数适应症源自成人经验。本研究的目的是回顾我们科室六年期间的PEX治疗情况。
对38例患者进行了377次PEX治疗（中位年龄12.1岁，范围0.6 - 20.5岁）。分别有9例、1例和3例患者接受了双针和单针PEX联合血液透析以及PEX联合超滤治疗。PEX最常见的适应症是非典型溶血性尿毒症综合征（aHUS，9例）、局灶节段性肾小球硬化（FSGS，9例）、肾移植（rTx）受者的抗体介导排斥反应（AMR，8例）以及等待rTx患者的高免疫状态（4例）。
我们仅对5例患有aHUS的天然肾患者进行了PEX治疗，4/6次复发完全缓解；PEX还成功用于预防3例接受rTx患者的HUS复发。通过基于PEX和免疫抑制剂的治疗方案，仅1例天然肾FSGS患者获得部分缓解；相反，6/6例rTx后FSGS复发患者观察到部分缓解。免疫抑制方案联合PEX对致敏尸体肾rTx受者有用（2/4成功移植），但6例慢性AMR患者治疗失败。关于并发症，发生了两起严重不良反应：使用白蛋白后发生过敏性休克和腹部出血。
PEX在儿童中是一种相对安全的操作。患有aHUS、复发性FSGS的儿科患者以及致敏rTx受者似乎从包括PEX的治疗策略中获益。
