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Atypical HUS Proposed Physiology
- Loss-of-function mutation in regulatory gene (CFH) AND gain-of-function mutation in effector gene
- Trigger event (often infection)
- Uninhibited continuous activation of alternative pathway (C5) --> MAC
- Endothelium damage --> activation of the coagulation cascade and TMA
**Mutations in complement factor H are most common**
Posted 03/09/17 09:09:45 AM by Matthew Cummings
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PLEX vs FFP for TTP
- PLEX superior to FFP (NEJM 1991)
- HOWEVER -- if in resource-limited setting without access to PLEX -- FFP is best option prior to transfer for PLEX
Posted 03/09/17 09:26:40 AM by Matthew Cummings
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Eculizimab for atypical HUS
- First-line therapy: humanized monoclonal antibody to C5
- Binds to C5 --> prevents its cleavage --> inhibits production of C5a and then MAC
- IV infusion -- induction followed by maintenance dosing
- Unclear whether should be discontinued if positive response or if should be continued indefinitely
- Adverse effects: Severe meningococcal infections (should be vaccinated!)
Posted 03/09/17 09:32:09 AM by Matthew Cummings
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For further reading...
Posted 03/09/17 10:02:51 AM by Matthew Cummings
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