Back to Search Start Over

Transplantation in pediatric aHUS within the era of eculizumab therapy.

Authors :
Özçakar, Zeynep Birsin
Ozaltin, Fatih
Gülhan, Bora
Çomak, Elif
Parmaksız, Gönül
Baskın, Esra
Topaloğlu, Rezan
Kasap Demir, Belde
Canpolat, Nur
Yuruk Yildirim, Zeynep
Demircioğlu Kılıç, Beltinge
Yüksel, Selçuk
Söylemezoğlu, Oğuz
Source :
Pediatric Transplantation; May2021, Vol. 25 Issue 3, p1-7, 7p
Publication Year :
2021

Abstract

aHUS is caused by the over‐activation and dysregulation of the alternative complement pathway. Data regarding outcomes of pediatric aHUS patients after kidney transplantation are still very scarce. Accordingly, the aim of this study was to describe the clinical findings and outcomes of pediatric aHUS patients after renal transplantation. This is a retrospective, multicenter study including 12 patients from the national registry system. Among the 12 patients, eight had received prophylactic eculizumab and none of those patients (except one) had experienced aHUS recurrence during a median follow‐up period of 58.5 (min‐max, 4‐94) months. Although eculizumab had been started on the day before transplantation in one of them, aHUS recurrence occurred during the transplantation procedure. Eculizumab had been stopped in only one patient who had no complement gene mutation after 35 months of therapy, and recurrence had not been observed during the 19 months of follow‐up. In three patients, maintenance doses had been spaced out without any recurrence. One additional patient with anti‐CFH antibody received only two doses of eculizumab for transplantation and had been followed for 46 months without aHUS recurrence. The remaining three patients had not received anti‐C5 therapy and none of those patients experienced aHUS recurrence during a median follow‐up period of 21 (min‐max, 9‐42) months. Prophylactic eculizumab is a safe and effective treatment for the prevention of aHUS recurrence. Eculizumab interval prolongation, discontinuation, and transplantation without eculizumab prophylaxis can be tried in selected patients with close follow‐up. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
13973142
Volume :
25
Issue :
3
Database :
Complementary Index
Journal :
Pediatric Transplantation
Publication Type :
Academic Journal
Accession number :
149879906
Full Text :
https://doi.org/10.1111/petr.13914