P.317 Kidney transplant in atypical HUS during Eculizumab Era. A single Center experience
Monday May 06, 2019 from 17:00 to 18:00
Exhibit-Poster Area
Presenter

Sara Testa, Italy

Medical Doctor

Pediatric nephrology, dialysis and transplantation Unit

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

Abstract

Kidney transplant in atypical HUS during Eculizumab Era. A single Center experience

Sara Testa1, Patrizia Di Matteo2, Gianluigi Ardissino1, Francesca Tel1, Valentina Capone1, Donata Cresseri3, Antenore Giussani4, Bice Strumbo5, Massimo Cugno7, Lucrezia Furian6, Silvana Tedeschi5, Giovanni Montini1.

1Pediatric Nephrology, Dialysis and Transplantation Unit, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy; 2Pediatric Department, Policlinico , Università Federico II , Naples, Italy; 3Nephrology Unit, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy; 4Kidney Transplant Unit, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy; 5Molecular Biology Laboratory, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy; 6Transplantation Unit, University Hospital, Padova, Italy; 7Internal Medicine, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy

Center for HUS Prevention, Control and Management.

Introduction: Patients (pts) with atypical hemolityc uremic syndrome (aHUS) have long been considered ineligible for kidney transplantation (KTx) because of the high risk of disease recurrence, graft loss and life-threatening complications. The availability of eculizumab (ECU) has now overcome this problem, but the best approach to treatment (Rx) timing, the maintenance schedule, the possibility of discontinuation, and patient monitoring has not been established. Aim of this study is to describe the experience on KTx in patients with aHUS at our Center during the last decades.

Methods: During the past 20 years, a total 22 pts (9 children at diagnosis) with aHUS have
undergone 28 KTx (16 pts first kTx, 6 pts had a previous one), the vast majority (18) of which have been performed since 2010 after ECU became available. The median time on RRT prior to KTx was 5.5 years. One pt has received the graft from a living-related donor. CFH-related disease (n:16) was the most common etiology followed by mutations on CFI (n:3), C3 (n:1), MCP (n:1) and Idiopathic (n:1). Based on the strategy used to prevent disease relapse before and after KTx, 3 groups can be identified (some pts were exposed to multiple preventive strategies): 
A: no prophylaxis (n:8); B: plasmaexchange/plasmainfusion (n:6); C: ECU (n:14).

Results: In Tab 1 are shown kTx results in the 3 groups. Pts in group B were switched to ECU once available. One pt discontinued ECU as soon as AntiCFHAb were no longer detectable.Conclusions: Our experience favours the prophylactic use of ECU in pts undergoing KTx with an history of aHUS. We recommend complete characterization (as to disease etiology) pre-KTx and
that maintenance Rx is continued lifelong.

Progetto Alice ONLUS.


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