Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Mache, CJ; Acham-Roschitz, B; Frémeaux-Bacchi, V; Kirschfink, M; Zipfel, PF; Roedl, S; Vester, U; Ring, E.
Complement inhibitor eculizumab in atypical hemolytic uremic syndrome.
Clin J Am Soc Nephrol. 2009; 4(8):1312-1316
Doi: 10.2215/CJN.01090209
(- Case Report)
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- Führende Autor*innen der Med Uni Graz
- Mache Christoph
- Co-Autor*innen der Med Uni Graz
- Acham-Roschitz Birgit
- Ring Ekkehard
- Roedl Siegfried
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- Abstract:
- Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous complement activation on host cells causing inflammation and damage. Eculizumab, a humanized mAb against complement protein C5, inhibits activation of the terminal complement pathway. We report an adolescent with relapsing unclassified aHUS. On admission, a high plasma creatinine level indicated a poor prognosis, and hemodialysis had to be started. Plasma exchanges were initially effective against the microangiopathic hemolytic activity and allowed a temporary improvement of renal function with termination of hemodialysis after 7 wk. Subsequently, plasma exchanges (three times per week) failed to prevent ongoing aHUS activity and progressive renal failure. After 12 wk, aHUS treatment was switched to eculizumab. Eculizumab was effective in terminating the microangiopathic hemolytic process in two aHUS relapses; however, after normalization of complement activity, aHUS recurred and ultimately led to anuric end-stage renal failure. In this patient, complement inhibition by eculizumab temporarily terminated the microangiopathic hemolytic activity. Nevertheless, renal damage as a result of preceding and subsequent aHUS activity resulted in end-stage renal failure; therefore, therapeutic success may depend on early administration of eculizumab. The optimal duration of treatment may be variable and remains to be determined.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent -
- Antibodies, Monoclonal - administration & dosage
- Antibodies, Monoclonal, Humanized -
- Complement Activation - drug effects
- Hemolytic-Uremic Syndrome - complications Hemolytic-Uremic Syndrome - drug therapy Hemolytic-Uremic Syndrome - immunology
- Humans -
- Immunologic Factors - administration & dosage
- Kidney Failure, Chronic - immunology Kidney Failure, Chronic - therapy
- Male -
- Plasma Exchange -
- Recurrence -
- Renal Dialysis -
- Treatment Outcome -