Eculizumab for Congenital Atypical Hemolytic–Uremic Syndrome
To the Editor: Atypical hemolytic–uremic syndrome of infancy is a rare disorder that is associated with thrombotic microangiopathy and acute renal failure. It often involves complement dysregulation. 1 , 2 Plasma infusions have variable efficacy, and end-stage renal disease often develops in childre...
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Veröffentlicht in: | The New England journal of medicine 2009-01, Vol.360 (5), p.544-546 |
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Sprache: | eng |
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Zusammenfassung: | To the Editor:
Atypical hemolytic–uremic syndrome of infancy is a rare disorder that is associated with thrombotic microangiopathy and acute renal failure. It often involves complement dysregulation.
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2
Plasma infusions have variable efficacy, and end-stage renal disease often develops in children who are unresponsive to plasma therapy.
1
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2
We report on a patient with congenital relapsing atypical hemolytic–uremic syndrome who was unresponsive to plasma therapy but had a response to eculizumab, a humanized monoclonal antibody against terminal complement protein C5.
3
An 18-month-old boy was admitted with a fourth relapse of congenital atypical hemolytic–uremic syndrome. He was born at 34 weeks' . . . |
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ISSN: | 0028-4793 1533-4406 |
DOI: | 10.1056/NEJMc0809959 |