Atypical hemolytic-uremic syndrome: Difference between revisions
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==Background== |
==Background== |
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| − | * |
+ | *One of the [[thrombotic microangiopathy|thrombotic microangiopathies]] |
| − | * |
+ | *See also [[hemolytic-uremic syndrome|typical hemolytic-uremic syndrome]], which occurs after infectious diarrhea |
===Pathophysiology=== |
===Pathophysiology=== |
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| − | * |
+ | *Congenital defect leading to dysregulation of the alternative complement pathway, which leads to increased complement activity |
==Diagnosis== |
==Diagnosis== |
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*Often unable to distinguish from TTP, so [[plasma exchange]] should be initiated promptly |
*Often unable to distinguish from TTP, so [[plasma exchange]] should be initiated promptly |
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| − | *If no improvement on PLEX and there is significant renal involvement, consider |
+ | *If no improvement on PLEX and there is significant renal involvement, consider: |
| − | **[[Eculizumab]] to inhibit complement |
+ | **[[Eculizumab]] to inhibit C5 complement |
| + | **Ideally with full meningococcal vaccination beforehand |
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[[Category:Hematology]] |
[[Category:Hematology]] |
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Revision as of 07:34, 15 August 2020
Background
- One of the thrombotic microangiopathies
- See also typical hemolytic-uremic syndrome, which occurs after infectious diarrhea
Pathophysiology
- Congenital defect leading to dysregulation of the alternative complement pathway, which leads to increased complement activity
Diagnosis
- Genetic mutation analysis of complement regulatory proteins (CFH, CFI, MCP, C3, CFB, THBD) and anti-CFH antibodies
Management
- Often unable to distinguish from TTP, so plasma exchange should be initiated promptly
- If no improvement on PLEX and there is significant renal involvement, consider:
- Eculizumab to inhibit C5 complement
- Ideally with full meningococcal vaccination beforehand
