Complement inhibitor eculizumab in atypical hemolytic uremic syndrome
| Autor: | Birgit Acham-Roschitz, Siegfried Roedl, Peter F. Zipfel, Michael Kirschfink, Véronique Frémeaux-Bacchi, Udo Vester, Christoph J. Mache, Ekkehard Ring |
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| Rok vydání: | 2009 |
| Předmět: |
Male
Adolescent Epidemiology medicine.medical_treatment Renal function urologic and male genital diseases Critical Care and Intensive Care Medicine Antibodies Monoclonal Humanized Complement inhibitor Recurrence Renal Dialysis hemic and lymphatic diseases Atypical hemolytic uremic syndrome Medicine Humans Immunologic Factors Complement Activation Transplantation Plasma Exchange business.industry Antibodies Monoclonal Original Articles Eculizumab medicine.disease Hemolytic Process Complement system Treatment Outcome Nephrology Immunology Hemolytic-Uremic Syndrome Alternative complement pathway Kidney Failure Chronic Hemodialysis business medicine.drug |
| Zdroj: | Clinical journal of the American Society of Nephrology : CJASN. 4(8) |
| ISSN: | 1555-905X |
| Popis: | Background and objectives: 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. Design, setting, participants, & measurements: 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. Results: 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. Conclusions: 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. |
| Databáze: | OpenAIRE |
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