Atypical hemolytic uremic syndrome responsive to steroids and intravenous immune globulin.

Abstract

Atypical hemolytic uremic syndrome remains a challenge to diagnose and treat, with significant acute morbidity and risk for progression to end stage renal disease. Treatment strategies center on plasma exchange but do not necessarily affect the progression of disease. We report the case of a patient with atypical HUS resulting from a mutation in the complement pathway who responded to treatment with steroids and IVIG, therefore avoiding transfusion or plasma exchange.

• Authors: Tanya Watt, Barry Warshaw, Howard M Katzenstein
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 53 Issue 1 Pg. 90-1 (Jul 2009) ISSN: 1545-5017 [Electronic] United States
• PMID: 19301397 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright 2009 Wiley-Liss, Inc.

Chemical References

• CD46 protein, human
• Glucocorticoids
• Immunoglobulins, Intravenous
• Immunologic Factors
• Membrane Cofactor Protein
• Methylprednisolone

Topics

• Blood Transfusion
• Glucocorticoids (therapeutic use)
• Hemolytic-Uremic Syndrome (drug therapy, genetics)
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunologic Factors (therapeutic use)
• Infant
• Male
• Membrane Cofactor Protein (genetics)
• Methylprednisolone (therapeutic use)
• Mutation
• Treatment Outcome