Recovery of renal function after long-term dialysis and resolution of cardiomyopathy in a patient with aHUS receiving eculizumab

BMJ Case Reports
Khadizha EmirovaBert van den Heuvel

Abstract

We present the case of a 18-month-old girl with renal and cardiac manifestations of atypical haemolytic uraemic syndrome (aHUS), and a novel complement factor H mutation. Transient haematological remission was achieved with intensive plasmapheresis, but cardiac function deteriorated and renal function was not restored. Initiation of eculizumab after 6 months of dialysis significantly improved organ function. At 43 months after presentation, haematological values had normalised and cardiac function had improved. Dialysis was discontinued after 10 months (the longest reported time in a patient with aHUS) and the estimated glomerular filtration rate had recovered to 70 mL/min/1.73 m(2). In conclusion, treatment of aHUS with eculizumab, even after long-term dialysis, can significantly improve renal function. Discontinuation of dialysis and resolution of cardiac function has implications on the potential recovery and treatment choice of such patients. Earlier initiation of eculizumab, however, might have prevented the irreversible renal sclerosis and cardiac dysfunction.

Citations

Mar 1, 2017·Lancet·Fadi FakhouriChantal Loirat
Sep 15, 2019·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Orly HaskinMiriam Davidovits
Jun 15, 2017·Case Reports in Nephrology and Dialysis·Rafael Alonso ValenteCándido Díaz Rodríguez
Sep 22, 2017·Clinical Pediatrics·Deeksha BorkarPriya Verghese
Apr 3, 2018·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·Natalia L KozlovskayaLarisa A Bobrova

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