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

Autor:	Teoh, Chia Wei, Gorman, Kathleen Mary, Lynch, Bryan, Goodship, Timothy H. J., Dolan, Niamh Marie, Waldron, Mary, Riordan, Michael, Awan, Atif
Jazyk:	angličtina
Rok vydání:	2018
Předmět:	Article Subject hemic and lymphatic diseases urologic and male genital diseases
Zdroj:	Case Reports in Nephrology.
ISSN:	2090-6641
DOI:	10.1155/2018/2781789
Popis:	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.
Databáze:	OpenAIRE
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