Atypical hemolytic-uremic syndrome - Revision history

Aidan at 13:50, 20 April 2023

2023-04-20T13:50:31Z

← Older revision		Revision as of 13:50, 20 April 2023
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	*One of the [[thrombotic microangiopathy\|thrombotic microangiopathies]]		*One of the [[thrombotic microangiopathy\|thrombotic microangiopathies]]
	*~~See~~ ~~also~~ [[hemolytic-uremic syndrome\|typical hemolytic-uremic syndrome]]~~, which occurs~~ after ~~infectious~~ diarrhea		*Different pathophysiology and treatment from [[hemolytic-uremic syndrome\|typical hemolytic-uremic syndrome]] (after STEC diarrhea) and [[secondary hemolytic-uremic syndrome]]

	===Pathophysiology===		===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
			*Mutations can occur anywhere in the complement pathway or, occasionally, in unrelated proteins
			**Complement factor H (CFH), C3, factor B, factor I, CD46
			**Diacylglycerol kinase ε, plasminogen, factor XII (in the presence of anti-factor H autoantibodies), and thrombomodulin (CD141)

	==Diagnosis==		==Diagnosis==

Aidan: /* Management */

2020-08-15T11:34:50Z

Management

← Older revision		Revision as of 11:34, 15 August 2020
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	==Background==		==Background==

	* One of the [[thrombotic microangiopathy\|thrombotic microangiopathies]]		*One of the [[thrombotic microangiopathy\|thrombotic microangiopathies]]
	* See also [[hemolytic-uremic syndrome\|typical hemolytic-uremic syndrome]], which occurs after infectious diarrhea		*See also [[hemolytic-uremic syndrome\|typical hemolytic-uremic syndrome]], which occurs after infectious diarrhea

	===Pathophysiology===		===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

	==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
	*If no improvement on PLEX and there is significant renal involvement, consider ~~an aHUS-specific treatment~~		*If no improvement on PLEX and there is significant renal involvement, consider:
	**[[Eculizumab]] to inhibit complement~~, ideally with full meningococcal vaccination beforehand~~		**[[Eculizumab]] to inhibit C5 complement
			**Ideally with full meningococcal vaccination beforehand

	[[Category:Hematology]]		[[Category:Hematology]]

Aidan: /* Background */

2020-08-15T11:33:46Z

Background

← Older revision		Revision as of 11:33, 15 August 2020
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	==Background==		==Background==

			* One of the [[thrombotic microangiopathy\|thrombotic microangiopathies]]
			* See also [[hemolytic-uremic syndrome\|typical hemolytic-uremic syndrome]], which occurs after infectious diarrhea

	===Pathophysiology===		===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

	==Diagnosis==		==Diagnosis==

Aidan at 11:32, 15 August 2020

2020-08-15T11:32:45Z

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	[[Category:Hematology]]		[[Category:Hematology]]
	[[Category:Nephrology]]		[[Category:Nephrology]]
			[[Category:Thrombosis]]

Aidan at 11:30, 15 August 2020

2020-08-15T11:30:26Z

← Older revision		Revision as of 11:30, 15 August 2020
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	== Background ==		==Background==

	===Pathophysiology===		===Pathophysiology===
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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
	*If no improvement on PLEX and there is significant renal involvement, consider an aHUS-specific treatment		*If no improvement on PLEX and there is significant renal involvement, consider an aHUS-specific treatment
	**[[Eculizumab]] to inhibit complement		**[[Eculizumab]] to inhibit complement, ideally with full meningococcal vaccination beforehand

	[[Category:Hematology]]		[[Category:Hematology]]

Aidan: Aidan moved page Atypical hemolytic uremic syndrome to Atypical hemolytic-uremic syndrome without leaving a redirect

2020-08-15T11:29:28Z

Aidan moved page Atypical hemolytic uremic syndrome to Atypical hemolytic-uremic syndrome without leaving a redirect

← Older revision	Revision as of 11:29, 15 August 2020
(No difference)

Aidan at 11:29, 15 August 2020

2020-08-15T11:29:16Z

← Older revision		Revision as of 11:29, 15 August 2020
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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]]		[[Category:Hematology]]

Maintenance script: Imported from text file

2020-07-04T01:17:24Z

Imported from text file

New page

== Pathophysiology ==

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

== Investigations ==

* Diagnosis with 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 aHUS-specific treatment
* Eculizumab

[[Category:Hematology]]
[[Category:Nephrology]]