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BMJ Case Rep. 2016 Feb 15;2016. pii: bcr2015213928. doi: 10.1136/bcr-2015-213928.

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

Author information

1
The AI Evdokimov Moscow State University of Medicine and Dentistry, Moscow, Russian Federation.
2
Department of Paediatric Nephrology, Radboud University Medical Center, Nijmegen, The Netherlands.
3
Department of Laboratory Medicine, Radboud University Medical Center, Nijmegen, The Netherlands.
4
Department of Paediatric Nephrology, Radboud University Medical Center, Nijmegen, The Netherlands Department of Laboratory Medicine, Radboud University Medical Center, Nijmegen, The Netherlands.

Erratum in

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.

PMID:
26880823
PMCID:
PMC4840264
DOI:
10.1136/bcr-2015-213928
[Indexed for MEDLINE]
Free PMC Article

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