Living-donor kidney transplantation for atypical haemolytic uremic syndrome with pre-emptive eculizumab use

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023001%3A_____%2F15%3A00059363" target="_blank" >RIV/00023001:_____/15:00059363 - isvavai.cz</a>

Výsledek na webu

<a href="http://onlinelibrary.wiley.com/doi/10.1111/tri.12440/epdf" target="_blank" >http://onlinelibrary.wiley.com/doi/10.1111/tri.12440/epdf</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1111/tri.12440" target="_blank" >10.1111/tri.12440</a>

Jazyk výsledku

angličtina

Název v původním jazyce

Living-donor kidney transplantation for atypical haemolytic uremic syndrome with pre-emptive eculizumab use

Popis výsledku v původním jazyce

Post-transplant recurrence of atypical hemolytic uremic syndrome (aHUS), an ultra-rare genetic disease, is high and associated with a poor prognosis. Several recent reports have provided encouraging findings regarding the use of eculizumab, an anti-C5 terminal complement inhibitor, when given at transplantation or, in the case of disease recurrence, after transplantation. The complement system is an important mediator of renal ischemia-reperfusion injury. Therefore, pre-emptive eculizumab might be effective to attenuate complement activation. Here, we report an aHUS patient with associated complement factor H (CFH) mutation who underwent eculizumab therapy for 2 months before living-unrelated donor kidney transplantation. Despite a successful post-operative course, the patient suffered from polyoma BK nephropathy at 3 months. After reduction of immunosuppression, he experienced steroid-sensitive acute rejection, despite receiving therapeutic levels of eculizumab. Otherwise, the 14-month eculizumab therapy follow-up was uneventful, and the graft function remained stable at 1.7 mg dL-1 at 12 months after transplantation. This case highlights the successful use of eculizumab therapy before and after transplantation to prevent aHUS recurrence and shows the possible pitfalls that may accompany such therapy.

Název v anglickém jazyce

Living-donor kidney transplantation for atypical haemolytic uremic syndrome with pre-emptive eculizumab use

Popis výsledku anglicky

Post-transplant recurrence of atypical hemolytic uremic syndrome (aHUS), an ultra-rare genetic disease, is high and associated with a poor prognosis. Several recent reports have provided encouraging findings regarding the use of eculizumab, an anti-C5 terminal complement inhibitor, when given at transplantation or, in the case of disease recurrence, after transplantation. The complement system is an important mediator of renal ischemia-reperfusion injury. Therefore, pre-emptive eculizumab might be effective to attenuate complement activation. Here, we report an aHUS patient with associated complement factor H (CFH) mutation who underwent eculizumab therapy for 2 months before living-unrelated donor kidney transplantation. Despite a successful post-operative course, the patient suffered from polyoma BK nephropathy at 3 months. After reduction of immunosuppression, he experienced steroid-sensitive acute rejection, despite receiving therapeutic levels of eculizumab. Otherwise, the 14-month eculizumab therapy follow-up was uneventful, and the graft function remained stable at 1.7 mg dL-1 at 12 months after transplantation. This case highlights the successful use of eculizumab therapy before and after transplantation to prevent aHUS recurrence and shows the possible pitfalls that may accompany such therapy.

Druh

J_x - Nezařazeno - Článek v odborném periodiku (Jimp, Jsc a Jost)

CEP obor

FJ - Chirurgie včetně transplantologie

OECD FORD obor

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Projekt

—

Návaznosti

N - Vyzkumna aktivita podporovana z neverejnych zdroju

Rok uplatnění

2015

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Transplant international

ISSN

0934-0874

e-ISSN

—

Svazek periodika

28

Číslo periodika v rámci svazku

3

Stát vydavatele periodika

GB - Spojené království Velké Británie a Severního Irska

Počet stran výsledku

4

Strana od-do

366-369

Kód UT WoS článku

000350987000014

EID výsledku v databázi Scopus

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