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Eculizumab as rescue therapy for atypical hemolytic uremic syndrome with normal platelet count
- Source :
- Pediatric Nephrology. July 1, 2012, Vol. 27 Issue 7, p1193, 3 p.
- Publication Year :
- 2012
-
Abstract
- Background Atypical hemolytic uremic syndrome (aHUS) in childhood is a rare disease with frequent progression to end-stage renal disease and a high recurrence after kidney transplantation. Eculizumab, a humanized monoclonal antibody that binds to complement protein C5, may be beneficial in the treatment of aHUS. Case-diagnosis/treatment A 6-year-old girl developed aHUS with only slightly elevated C3d (4.4%), no mutations in complement factors, and no antibodies against factor H. Plasma exchange treatment was successful initially, until aHUS recurred. After reinitiating plasma exchange, normalization of the platelet count and improvement of hemolysis occurred, but renal function worsened. Renal function then improved dramatically promptly after the switch to eculizumab. Conclusions This case demonstrates that platelet count is not always a reliable marker for improvement of aHUS and that eculizumab can prevent dialysis in plasma-resistant aHUS patients. Keywords Eculizumab. Atypical hemolytic uremic syndrome. Acute kidney failure. Child<br />Introduction Hemolytic uremic syndrome (HUS) is a severe disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. In children, over 90% of HUS is caused [...]
Details
- Language :
- English
- ISSN :
- 0931041X
- Volume :
- 27
- Issue :
- 7
- Database :
- Gale General OneFile
- Journal :
- Pediatric Nephrology
- Publication Type :
- Academic Journal
- Accession number :
- edsgcl.331925968
- Full Text :
- https://doi.org/10.1007/s00467-012-2130-z