Clinical Relapses of Atypical HUS on Eculizumab: Clinical Gap for Monitoring and Individualised Therapy

Atypical hemolytic uremic syndrome (aHUS) is caused by dysregulation of the complement system. A humanised anti-C5 monoclonal antibody (eculizumab) is available for the treatment of aHUS. We present the first description of atypical HUS in a child with a coexistent diagnosis of a POL-III leukodystro...

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Published inCase Reports in Nephrology Vol. 2018; no. 2018; pp. 1 - 4
Main Authors Awan, Atif, Waldron, Mary, Dolan, Niamh Marie, Goodship, Timothy H. J., Lynch, Bryan, Gorman, Kathleen Mary, Teoh, Chia Wei, Riordan, Michael
Format Journal Article
LanguageEnglish
Published Cairo, Egypt Hindawi Publishing Corporation 2018
Hindawi
Hindawi Limited
Wiley
Subjects
Antibodies
Blood platelets
Case Report
Case reports
Eculizumab
Hematology
Hospitals
Hypertension
Laboratories
Monoclonal antibodies
Mutation
Nephrology
Pediatrics
Plasma
RNA polymerase
Transplants & implants
Canada
United States > US
Ireland
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Summary:Atypical hemolytic uremic syndrome (aHUS) is caused by dysregulation of the complement system. A humanised anti-C5 monoclonal antibody (eculizumab) is available for the treatment of aHUS. We present the first description of atypical HUS in a child with a coexistent diagnosis of a POL-III leukodystrophy. On standard eculizumab dosing regime, there was evidence of ongoing C5 cleavage and clinical relapses when immunologically challenged. Eculizumab is an effective therapy for aHUS, but the recommended doses may not be adequate for all patients, highlighting the need for ongoing efforts to develop a strategy for monitoring of treatment efficacy and potential individualisation of therapy.
Bibliography:Academic Editor: Salih Kavukcu
ISSN:2090-6641
2090-665X
DOI:10.1155/2018/2781789