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Transplantation in pediatric aHUS within the era of eculizumab therapy
Author(s) -
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
Publication year - 2021
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.13914
Subject(s) - eculizumab , medicine , atypical hemolytic uremic syndrome , discontinuation , transplantation , kidney transplantation , surgery , retrospective cohort study , pediatrics , complement system , antibody , immunology
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.