Monitoring and modeling treatment of atypical hemolytic uremic syndrome

S Heinen, FG Pluthero, VF van Eimeren… - Molecular …, 2013 - Elsevier
Atypical hemolytic uremic syndrome (aHUS), is mainly present in children, who have high
risks of end-stage kidney disease (ESKD), post-transplant recurrence and death. aHUS is
linked to defective regulation of the complement alternative pathway (AP), with a prominent
cause being mutation/inhibition of the negative regulator complement factor H (CFH). CFH
function can be restored via infusion of fresh frozen plasma (FFP), a treatment that was
effective for several years in a patient heterozygous for a cfh mutation, before the patient …
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