How is pediatric atypical hemolytic uremic syndrome (aHUS) treated?

Updated: Nov 12, 2018
  • Author: Robert S Gillespie, MD, MPH; Chief Editor: Craig B Langman, MD  more...
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Answer

Management of aHUS is very difficult and remains incompletely understood. Clinicians caring for patients with aHUS should search recent literature and confer with physicians with expertise in this disorder. [6, 20]

Discontinue the offending agent if a drug-associated cause is identified.

Treat bacterial infections (eg, S pneumoniae) promptly and aggressively.

Prior to the development of eculizumab, plasma therapies formed the mainstay of treatment for most forms of aHUS. These therapies use donor plasma products to replace the deficient or abnormal von Willebrand factor (vWF) metalloproteinase or complement factors.


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