Clinical Grand Rounds: Atypical Hemolytic Uremic Syndrome
| Autor: | Jerome C. Lane, Kavita S. Hodgkins, Amy E. Bobrowski, Craig B. Langman |
|---|---|
| Rok vydání: | 2012 |
| Předmět: |
Thrombotic microangiopathy
Complement Membrane Attack Complex Antibodies Monoclonal Humanized urologic and male genital diseases hemic and lymphatic diseases Atypical hemolytic uremic syndrome medicine Humans Atypical Hemolytic Uremic Syndrome business.industry Autoantibody Infant Eculizumab medicine.disease Kidney Transplantation Complement system Treatment Outcome Nephrology Factor H Hemolytic-Uremic Syndrome Immunology Alternative complement pathway Female Complement membrane attack complex business medicine.drug |
| Zdroj: | American Journal of Nephrology. 35:394-400 |
| ISSN: | 1421-9670 0250-8095 |
| DOI: | 10.1159/000337954 |
| Popis: | Atypical hemolytic uremic syndrome (aHUS) is a rare, lifethreatening, chronic, genetic disease of uncontrolled alternative pathway complement activation. The understanding of the pathophysiology and genetics of this disease has expanded over recent decades and promising new developments in the management of aHUS have emerged. Regardless of the cause of aHUS, with or without a demonstrated mutation or autoantibody, blockade of terminal complement activation through C5 is of high interest as a mechanism to ameliorate the disease. Eculizumab, an existing monoclonal antibody directed against C5 with high affinity, prevents the perpetuation of the downstream activation of the complement cascade and the damage caused by generation of the anaphylotoxin C5a and the membrane attack complex C5b-9, by blocking C5 cleavage. We report the successful use of eculizumab in a patient after kidney transplantation and discuss the disease aHUS. |
| Databáze: | OpenAIRE |
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