Hyperammonemia - Revision history

Ostermayer: Prepared the page for translation

2026-01-04T23:08:12Z

Prepared the page for translation

← Revisión anterior		Revisión del 23:08 4 ene 2026
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			<languages/>
			<translate>

	==Background==		==Background==


	===Pathophysiology===		===Pathophysiology===

	*Acquired: liver failure results in shunting of blood from the liver to the inferior vena cava, decreased filtration of blood and removal of nitrogen-containing toxins by the liver, and then hyperammonemia.		*Acquired: liver failure results in shunting of blood from the liver to the inferior vena cava, decreased filtration of blood and removal of nitrogen-containing toxins by the liver, and then hyperammonemia.
	*Congenital: Defect in one of the enzymes of the urea cycle, which leads to lower production of urea from ammonia.		*Congenital: Defect in one of the enzymes of the urea cycle, which leads to lower production of urea from ammonia.


	===Types===		===Types===

	*Primary		*Primary
	**Caused by several [[inborn errors of metabolism]]		**Caused by several [[Special:MyLanguage/inborn errors of metabolism\|inborn errors of metabolism]]
	**Characterized by reduced activity of urea cycle enzymes		**Characterized by reduced activity of urea cycle enzymes
	*Secondary		*Secondary
	**Caused by inborn errors of intermediary metabolism (i.e. reduced activity of enzymes that are not part of the urea cycle)		**Caused by inborn errors of intermediary metabolism (i.e. reduced activity of enzymes that are not part of the urea cycle)
	**Typically from acute or chronic [[liver failure]]		**Typically from acute or chronic [[Special:MyLanguage/liver failure\|liver failure]]


	==Clinical Features==		==Clinical Features==
	*[[AMS]]- ranging from mild apathy to frank coma
			*[[Special:MyLanguage/AMS\|AMS]]- ranging from mild apathy to frank coma
	* +/- asterexis		* +/- asterexis
	*Signs/symptoms of underlying disease or trigger for exacerbation		*Signs/symptoms of underlying disease or trigger for exacerbation


	==Differential Diagnosis==		==Differential Diagnosis==


	===Elevated ammonia level<ref>Ferenci, P. Hepatic encephalopathy in adults: Clinical manifestations and diagnosis. In: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2020</ref>===		===Elevated ammonia level<ref>Ferenci, P. Hepatic encephalopathy in adults: Clinical manifestations and diagnosis. In: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2020</ref>===
	*[[Liver failure]]
	*[[Inborn errors of metabolism]] with urea cycle defects and organic acidemia		*[[Special:MyLanguage/Liver failure\|Liver failure]]
	*[[Shock]], severe [[dehydration]]		*[[Special:MyLanguage/Inborn errors of metabolism\|Inborn errors of metabolism]] with urea cycle defects and organic acidemia
	*[[Renal failure]]		*[[Special:MyLanguage/Shock\|Shock]], severe [[Special:MyLanguage/dehydration\|dehydration]]
	*[[Gastrointestinal bleeding]]		*[[Special:MyLanguage/Renal failure\|Renal failure]]
	*[[Urinary tract infection]] with a urease-producing organism (i.e. [[proteus mirabilis]]), systemic infection with [[mycoplasma]] hominis or ureasplasma species in [[lung transplant]] patients		*[[Special:MyLanguage/Gastrointestinal bleeding\|Gastrointestinal bleeding]]
	*[[Reye syndrome]]		*[[Special:MyLanguage/Urinary tract infection\|Urinary tract infection]] with a urease-producing organism (i.e. [[Special:MyLanguage/proteus mirabilis\|proteus mirabilis]]), systemic infection with [[Special:MyLanguage/mycoplasma\|mycoplasma]] hominis or ureasplasma species in [[Special:MyLanguage/lung transplant\|lung transplant]] patients
			*[[Special:MyLanguage/Reye syndrome\|Reye syndrome]]
	*Any cause of portosystemic shunting of blood		*Any cause of portosystemic shunting of blood
	*Ureterosigmoidostomy		*Ureterosigmoidostomy
Línea 33:		Línea 47:
	*Medication toxicity, including:		*Medication toxicity, including:
	**High dose chemotherapy		**High dose chemotherapy
	**[[valproate OD\|Valproic acid]]		**[[Special:MyLanguage/valproate OD\|Valproic acid]]
	**[[Barbiturate toxicity\|Barbiturates]]		**[[Special:MyLanguage/Barbiturate toxicity\|Barbiturates]]
	**[[ETOH intoxication]]\|ETOH]]		**[[Special:MyLanguage/ETOH intoxication\|ETOH intoxication]]\|ETOH]]
	**[[Diuretics]]		**[[Special:MyLanguage/Diuretics\|Diuretics]]
	**[[Salicylate toxicity\|ASA]]		**[[Special:MyLanguage/Salicylate toxicity\|ASA]]
	**Glycine toxicity		**Glycine toxicity

			</translate>
	{{Template:AMS DDX}}		{{Template:AMS DDX}}
			<translate>


	==Evaluation==		==Evaluation==
	*Evaluate for suspected underlying etiology as above and/or alternate etiology of presenting symptoms (e.g. workup for other causes of [[AMS]])
			*Evaluate for suspected underlying etiology as above and/or alternate etiology of presenting symptoms (e.g. workup for other causes of [[Special:MyLanguage/AMS\|AMS]])
	*Normal range of ammonia in adult is typically 10-80 mcg/dL or 6-47 μmol/L, <100 in neonate, HOWEVER severity of hepatic encephalopathy is inconsistently correlated to severity of ammonia elevation<ref>Ong JP, Aggarwal A, Krieger D, Easley KA, Karafa MT, Van Lente F, Arroliga AC, Mullen KD. Correlation between ammonia levels and the severity of hepatic encephalopathy. Am J Med. 2003 Feb 15;114(3):188-93. doi: 10.1016/s0002-9343(02)01477-8. PMID: 12637132.</ref>		*Normal range of ammonia in adult is typically 10-80 mcg/dL or 6-47 μmol/L, <100 in neonate, HOWEVER severity of hepatic encephalopathy is inconsistently correlated to severity of ammonia elevation<ref>Ong JP, Aggarwal A, Krieger D, Easley KA, Karafa MT, Van Lente F, Arroliga AC, Mullen KD. Correlation between ammonia levels and the severity of hepatic encephalopathy. Am J Med. 2003 Feb 15;114(3):188-93. doi: 10.1016/s0002-9343(02)01477-8. PMID: 12637132.</ref>
	**Value of ammonia level in liver disease more helpful to trend rather than as absolute number		**Value of ammonia level in liver disease more helpful to trend rather than as absolute number


	==Management==		==Management==

	*Treat underlying disorder		*Treat underlying disorder
	*If [[hepatic encephalopathy]]:		*If [[Special:MyLanguage/hepatic encephalopathy\|hepatic encephalopathy]]:
	**[[Lactulose]] 20g PO or (300mL in 700cc H2O retention enema x30min)		**[[Special:MyLanguage/Lactulose\|Lactulose]] 20g PO or (300mL in 700cc H2O retention enema x30min)
	***In colon degrades into lactic acid: acidic environment traps ammonia		***In colon degrades into lactic acid: acidic environment traps ammonia
	***Also inhibits ammonia production in gut wall		***Also inhibits ammonia production in gut wall
	*If secondary to inborn error of metabolism		*If secondary to inborn error of metabolism
	**[[sodium phenylacetate/sodium benzoate]] (Ammonul) 250mg/kg in D10 over 90min; then 250mg/kg/d infusion		**[[Special:MyLanguage/sodium phenylacetate/sodium benzoate\|sodium phenylacetate/sodium benzoate]] (Ammonul) 250mg/kg in D10 over 90min; then 250mg/kg/d infusion
	**Arginine 210mg/kg IV/IO in D10 over 90min; then 210mg/kg/d infusion		**Arginine 210mg/kg IV/IO in D10 over 90min; then 210mg/kg/d infusion
	**+/- [[levocarnitine\|carnitine]], 400 mg IV/IO in consultation w/specialist		**+/- [[Special:MyLanguage/levocarnitine\|carnitine]], 400 mg IV/IO in consultation w/specialist
	**May require [[dialysis]] if refractory/severe		**May require [[Special:MyLanguage/dialysis\|dialysis]] if refractory/severe


	==Disposition==		==Disposition==

	*Depends on cause		*Depends on cause


	==See Also==		==See Also==
	*[[Inborn errors of metabolism]]
	*[[Hepatic encephalopathy]]		*[[Special:MyLanguage/Inborn errors of metabolism\|Inborn errors of metabolism]]
			*[[Special:MyLanguage/Hepatic encephalopathy\|Hepatic encephalopathy]]


	==External Links==		==External Links==
Línea 70:		Línea 95:

	==References==		==References==

	<references/>		<references/>

	[[Category:GI]]		[[Category:GI]]
			</translate>

Rossdonaldson1 at 02:30, 27 November 2021

2021-11-27T02:30:04Z

← Revisión anterior		Revisión del 02:30 27 nov 2021
Línea 71:		Línea 71:
	==References==		==References==
	<references/>		<references/>

			[[Category:GI]]

ClaireLewis at 05:02, 21 April 2021

2021-04-21T05:02:53Z

← Revisión anterior		Revisión del 05:02 21 abr 2021
Línea 10:		Línea 10:
	*Secondary		*Secondary
	**Caused by inborn errors of intermediary metabolism (i.e. reduced activity of enzymes that are not part of the urea cycle)		**Caused by inborn errors of intermediary metabolism (i.e. reduced activity of enzymes that are not part of the urea cycle)
	***Normally from acute or chronic [[liver failure]]		**Typically from acute or chronic [[liver failure]]

	==Clinical Features==		==Clinical Features==
			*[[AMS]]- ranging from mild apathy to frank coma
			* +/- asterexis
			*Signs/symptoms of underlying disease or trigger for exacerbation

	==Differential Diagnosis==		==Differential Diagnosis==
			===Elevated ammonia level<ref>Ferenci, P. Hepatic encephalopathy in adults: Clinical manifestations and diagnosis. In: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2020</ref>===
			*[[Liver failure]]
			*[[Inborn errors of metabolism]] with urea cycle defects and organic acidemia
			*[[Shock]], severe [[dehydration]]
			*[[Renal failure]]
			*[[Gastrointestinal bleeding]]
			*[[Urinary tract infection]] with a urease-producing organism (i.e. [[proteus mirabilis]]), systemic infection with [[mycoplasma]] hominis or ureasplasma species in [[lung transplant]] patients
			*[[Reye syndrome]]
			*Any cause of portosystemic shunting of blood
			*Ureterosigmoidostomy
			*Cigarette smoking
			*Transient hyperammonemia in newborns
			*Parenteral nutrition
			*Medication toxicity, including:
			**High dose chemotherapy
			**[[valproate OD\|Valproic acid]]
			**[[Barbiturate toxicity\|Barbiturates]]
			**[[ETOH intoxication]]\|ETOH]]
			**[[Diuretics]]
			**[[Salicylate toxicity\|ASA]]
			**Glycine toxicity

			{{Template:AMS DDX}}

	==Evaluation==		==Evaluation==
	~~===Acquired vs. Congenital===~~		*Evaluate for suspected underlying etiology as above and/or alternate etiology of presenting symptoms (e.g. workup for other causes of [[AMS]])
	*~~Acquired~~		*Normal range of ammonia in adult is typically 10-80 mcg/dL or 6-47 μmol/L, <100 in neonate, HOWEVER severity of hepatic encephalopathy is inconsistently correlated to severity of ammonia elevation<ref>Ong JP, Aggarwal A, Krieger D, Easley KA, Karafa MT, Van Lente F, Arroliga AC, Mullen KD. Correlation between ammonia levels and the severity of hepatic encephalopathy. Am J Med. 2003 Feb 15;114(3):188-93. doi: 10.1016/s0002-9343(02)01477-8. PMID: 12637132.</ref>
	**Diseases that result in [[liver failure]]		**Value of ammonia level in liver disease more helpful to trend rather than as absolute number
	**Medications (e.g. [[~~valproic acid~~]] ~~overdose~~)
	**Severe dehydration (~~from small intestinal bacterial overgrowth~~)
	**Glycine toxicity (~~CNS symptoms and nausea~~)
	*~~Congenital~~
	**Genetic

	==Management==		==Management==
	*Treat underlying disorder		*Treat underlying disorder
			*If [[hepatic encephalopathy]]:
			**[[Lactulose]] 20g PO or (300mL in 700cc H2O retention enema x30min)
			***In colon degrades into lactic acid: acidic environment traps ammonia
			***Also inhibits ammonia production in gut wall
			*If secondary to inborn error of metabolism
			**[[sodium phenylacetate/sodium benzoate]] (Ammonul) 250mg/kg in D10 over 90min; then 250mg/kg/d infusion
			**Arginine 210mg/kg IV/IO in D10 over 90min; then 210mg/kg/d infusion
			**+/- [[levocarnitine\|carnitine]], 400 mg IV/IO in consultation w/specialist
			**May require [[dialysis]] if refractory/severe

	==Disposition==		==Disposition==

Rossdonaldson1 at 10:45, 18 July 2020

2020-07-18T10:45:52Z

← Revisión anterior		Revisión del 10:45 18 jul 2020
Línea 5:		Línea 5:

	===Types===		===Types===
	~~====Primary vs. secondary====~~
	*Primary		*Primary
	**Caused by several [[inborn errors of metabolism]]		**Caused by several [[inborn errors of metabolism]]
Línea 13:		Línea 12:
	***Normally from acute or chronic [[liver failure]]		***Normally from acute or chronic [[liver failure]]

	====Acquired vs. ~~congenital=~~===		==Clinical Features==


			==Differential Diagnosis==


			==Evaluation==
			===Acquired vs. Congenital===
	*Acquired		*Acquired
	**Diseases that result in [[liver failure]]		**Diseases that result in [[liver failure]]
Línea 21:		Línea 27:
	*Congenital		*Congenital
	**Genetic		**Genetic

	~~==Clinical Features==~~


	~~==Differential Diagnosis==~~


	~~==Evaluation==~~


	==Management==		==Management==
			*Treat underlying disorder

	==Disposition==		==Disposition==
			*Depends on cause

	==See Also==		==See Also==

Rossdonaldson1: /* Acquired vs. congenital */

2020-07-18T10:43:33Z

Acquired vs. congenital

← Revisión anterior		Revisión del 10:43 18 jul 2020
Línea 17:		Línea 17:
	**Diseases that result in [[liver failure]]		**Diseases that result in [[liver failure]]
	**Medications (e.g. [[valproic acid]] overdose)		**Medications (e.g. [[valproic acid]] overdose)
	*Severe dehydration (from small intestinal bacterial overgrowth)		**Severe dehydration (from small intestinal bacterial overgrowth)
	*Glycine toxicity (CNS symptoms and nausea)		**Glycine toxicity (CNS symptoms and nausea)
	*Congenital		*Congenital
	**Genetic		**Genetic

Rossdonaldson1: /* Specific types */

2020-07-18T10:23:23Z

Specific types

@@ Línea 21: / Línea 21: @@
 *Congenital
 **Genetic
 ==Clinical Features==

Rossdonaldson1: /* Background */

2020-07-18T10:23:08Z

Background

← Revisión anterior		Revisión del 10:23 18 jul 2020
Línea 1:		Línea 1:
	==Background==		==Background==
			===Pathophysiology===
			*Acquired: liver failure results in shunting of blood from the liver to the inferior vena cava, decreased filtration of blood and removal of nitrogen-containing toxins by the liver, and then hyperammonemia.
			*Congenital: Defect in one of the enzymes of the urea cycle, which leads to lower production of urea from ammonia.

			===Types===
			====Primary vs. secondary====
			*Primary
			**Caused by several [[inborn errors of metabolism]]
			**Characterized by reduced activity of urea cycle enzymes
			*Secondary
			**Caused by inborn errors of intermediary metabolism (i.e. reduced activity of enzymes that are not part of the urea cycle)
			***Normally from acute or chronic [[liver failure]]

			====Acquired vs. congenital====
			*Acquired
			**Diseases that result in [[liver failure]]
			**Medications (e.g. [[valproic acid]] overdose)
			*Severe dehydration (from small intestinal bacterial overgrowth)
			*Glycine toxicity (CNS symptoms and nausea)
			*Congenital
			**Genetic

			====Specific types====

			The following list includes such examples:
			* {{OMIM\|311250}} - hyperammonemia due to [[ornithine transcarbamylase deficiency]]
			* {{OMIM\|606762}} - [[hyperinsulinism-hyperammonemia syndrome]] ([[glutamate dehydrogenase 1]])
			* {{OMIM\|238970}} - [[hyperornithinemia-hyperammonemia-homocitrullinuria]]
			* {{OMIM\|237310}} - hyperammonemia due to [[N-acetylglutamate synthetase deficiency]]
			* {{OMIM\|237300}} - hyperammonemia due to [[carbamoyl phosphate synthetase I deficiency]] ([[carbamoyl phosphate synthetase I]])
			* {{OMIM\|238750}} - [[hyperlysinuria]] with hyperammonemia (genetics unknown)
			* [[Methylmalonic acidemia]]
			* [[Isovaleric acidemia]]
			* [[Propionic acidemia]]
			* [[Carnitine palmitoyltransferase II deficiency]]
			* [[Transient hyperammonemia of the newborn]], specifically in the preterm

	==Clinical Features==		==Clinical Features==

Rossdonaldson1: /* See Also */

2020-07-18T10:08:53Z

Rossdonaldson1: Created page with "==Background== ==Clinical Features== ==Differential Diagnosis== ==Evaluation== ==Management== ==Disposition== ==See Also== *Inborn errors of metabolism ==Exte..."

2020-07-14T16:48:40Z

Created page with "==Background== ==Clinical Features== ==Differential Diagnosis== ==Evaluation== ==Management== ==Disposition== ==See Also== *Inborn errors of metabolism ==Exte..."

Página nueva

==Background==

==Clinical Features==

==Differential Diagnosis==

==Evaluation==

==Management==

==Disposition==

==See Also==
*[[Inborn errors of metabolism]]

==External Links==

==References==
<references/>

← Revisión anterior		Revisión del 10:08 18 jul 2020
Línea 19:		Línea 19:
	==See Also==		==See Also==
	*[[Inborn errors of metabolism]]		*[[Inborn errors of metabolism]]
			*[[Hepatic encephalopathy]]

	==External Links==		==External Links==