Atypical hemolytic-uremic syndrome: Difference between revisions

Revision as of 11:29, 15 August 2020

Congenital defect leading to dysregulation of the alternative complement pathway, which leads to increased complement activity

Genetic mutation analysis of complement regulatory proteins (CFH, CFI, MCP, C3, CFB, THBD) and anti-CFH antibodies

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 an aHUS-specific treatment
- Eculizumab to inhibit complement

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-== Pathophysiology ==
+== Background ==
+===Pathophysiology===
-* Congenital defect leading to dysregulation of the alternative complement pathway, which leads to increased complement activity
+*Congenital defect leading to dysregulation of the alternative complement pathway, which leads to increased complement activity
-== Investigations ==
+==Diagnosis==
-* Diagnosis with genetic mutation analysis of complement regulatory proteins (CFH, CFI, MCP, C3, CFB, THBD) and anti-CFH antibodies
+*Genetic mutation analysis of complement regulatory proteins (CFH, CFI, MCP, C3, CFB, THBD) and anti-CFH antibodies
-== Management ==
+==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 aHUS-specific treatment
+*Often unable to distinguish from TTP, so [[plasma exchange]] should be initiated promptly
-* Eculizumab
+*If no improvement on PLEX and there is significant renal involvement, consider an aHUS-specific treatment
+**[[Eculizumab]] to inhibit complement
 [[Category:Hematology]]