MCID: HPT019
MIFTS: 57

Hepatic Encephalopathy malady

Rare diseases, Neuronal diseases, Liver diseases categories
Download this MalaCard

Summaries for Hepatic Encephalopathy

About this section


Fully expand this MalaCard
NIH Rare Diseases:42 Hepatic encephalopathy is a syndrome observed in some patients with cirrhosis. it is defined as a spectrum of neuropsychiatric abnormalities in patients with liver dysfunction, when other known brain disease has been excluded. signs and symptoms may be debilitating, and they can begin mildly and gradually, or occur suddenly and severely. they may include personality or mood changes, intellectual impairment, abnormal movements, a depressed level of consciousness, and other symptoms. there are several theories regarding the exact cause, but development of the condition is probably at least partially due to the effect of substances that are toxic to nerve tissue (neurotoxic), which are typically present with liver damage and/or liver disease. treatment depends upon the severity of mental status changes and upon the certainty of the diagnosis. last updated: 1/24/2011

MalaCards based summary: Hepatic Encephalopathy, also known as portal-systemic encephalopathy, is related to brain edema and liver cirrhosis. An important gene associated with Hepatic Encephalopathy is TSPO (translocator protein (18kDa)), and among its related pathways are Neurotransmitter Release Cycle and Alanine, aspartate and glutamate metabolism. The drugs lactulose and kanamycin sulfate and the compounds lazabemide and pyrazoline have been mentioned in the context of this disorder. Affiliated tissues include liver, brain and testes, and related mouse phenotype behavior/neurological.

Wikipedia:65 Hepatic encephalopathy (also known as portosystemic encephalopathy) is the occurrence of confusion,... more...

Aliases & Classifications for Hepatic Encephalopathy

About this section

Hepatic Encephalopathy, Aliases & Descriptions:

Name: Hepatic Encephalopathy 8 42 10 44 62
Portal-Systemic Encephalopathy 8 62
 
Encephalopathy, Hepatic 42
Hepatoencephalopathy 42


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Neuronal diseases, Liver diseases


External Ids:

Disease Ontology8 DOID:13413
ICD9CM27 572.2
MeSH34 D006501
NCIt39 C79596

Related Diseases for Hepatic Encephalopathy

About this section

Diseases related to Hepatic Encephalopathy via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 100)
idRelated DiseaseScoreTop Affiliating Genes
1brain edema31.0AQP4, GLUL
2liver cirrhosis31.0MAOA, GLUL, GLS
3blindness30.8TSPO, MAOB
4encephalitis30.1TSPO, AQP4
5psychotic disorder29.9MAOA, MAOB
6dementia29.8TSPO, MAOA, MAOB, GLS
7schizophrenia28.9GLS, MAOB, GLUL, AQP4, TSPO, MAOA
8alzheimer's disease28.9GLS, MAOA, MAOB, GLUL, AQP4, TSPO
9hepatitis11.4
10cerebritis10.6
11anxiety disorder10.4MAOA
12antisocial personality disorder10.4MAOA
13acute liver failure10.4
14attention deficit hyperactivity disorder10.3MAOA
15neuronitis10.3
16alcoholic hepatitis10.3
17liver disease10.3
18diabetes mellitus10.2
19hypertension10.2
20status epilepticus10.2
21portal vein thrombosis10.2
22cortical blindness10.2
23hepatic coma10.2
24combined oxidative phosphorylation deficiency 110.2
25generalized anxiety disorder10.2TSPO, MAOA
26stroke, ischemic10.2TSPO, AQP4
27obsessive-compulsive disorder10.2MAOA, TSPO
28panic disorder10.2TSPO, MAOA
29wilson disease10.2
30spastic paraparesis10.2
31spasticity10.2
32metabolic acidosis10.1GLS, GLUL
33urea cycle disorder10.1
34portal hypertension10.1
35autonomic neuropathy10.1
36neuropathy10.1
37peritonitis10.1
38infections, recurrent, with encephalopathy, hepatic dysfunction, and cardiovasuclar malformations10.1
39endotheliitis10.1
40brain disease10.1TSPO, AQP4
41focal epilepsy10.1TSPO, MAOB
42duchenne muscular dystrophy10.1SGCB, AQP4
43memory impairment10.1TSPO, MAOB
44multiple system atrophy10.1MAOB, TSPO
45personality disorder10.0MAOB, MAOA
46pure autonomic failure10.0MAOB, MAOA
47dilated cardiomyopathy10.0
48hepatitis b10.0
49hepatocellular carcinoma10.0
50malaria10.0

Graphical network of the top 20 diseases related to Hepatic Encephalopathy:



Diseases related to hepatic encephalopathy

Symptoms for Hepatic Encephalopathy

About this section

Drugs & Therapeutics for Hepatic Encephalopathy

About this section

Drug clinical trials:

Search ClinicalTrials for Hepatic Encephalopathy

Search NIH Clinical Center for Hepatic Encephalopathy

Inferred drug relations via UMLS62/NDF-RT40:

Genetic Tests for Hepatic Encephalopathy

About this section

Anatomical Context for Hepatic Encephalopathy

About this section

MalaCards organs/tissues related to Hepatic Encephalopathy:

32
Liver, Brain, Testes, Cortex, Cerebellum, Endothelial, Prefrontal cortex, Small intestine, Colon, Cingulate cortex, Globus pallidus, Thalamus

Animal Models for Hepatic Encephalopathy or affiliated genes

About this section

MGI Mouse Phenotypes related to Hepatic Encephalopathy:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053867.7GLS, GLUL, MAOB, MAOA, AQP4

Publications for Hepatic Encephalopathy

About this section

Articles related to Hepatic Encephalopathy:

(show top 50)    (show all 626)
idTitleAuthorsYear
1
Hyperammonemia and systemic inflammatory response syndrome predicts presence of hepatic encephalopathy in dogs with congenital portosystemic shunts. (24392080)
2014
2
Liver: The gut is a key target of therapy in hepatic encephalopathy. (25348849)
2014
3
Low grade cirrhosis induces cognitive impairment and motor dysfunction in rats: Could be a model for minimal hepatic encephalopathy. (24334168)
2014
4
Efficacy of liver assisting in patients with hepatic encephalopathy with special focus on plasma exchange. (23572273)
2013
5
The nutritional management of hepatic encephalopathy in patients with cirrhosis: International Society for Hepatic Encephalopathy and Nitrogen Metabolism Consensus. (23471642)
2013
6
Cerebral oedema is rare in acute-on-chronic liver failure patients presenting with high-grade hepatic encephalopathy. (23844567)
2013
7
Matrix metalloproteinase-9 as a therapeutic target for the progression of fulminant liver failure with hepatic encephalopathy: A pilot study in mice. (23672352)
2013
8
Neurotransmitter receptor alterations in hepatic encephalopathy: a review. (23466244)
2013
9
Early markers of neural dysfunction and compensation: A model from minimal hepatic encephalopathy. (24333166)
2013
10
Systemic inflammation and ammonia in hepatic encephalopathy. (23224356)
2013
11
Disaccharides in the treatment of hepatic encephalopathy. (23456517)
2013
12
Psychometric hepatic encephalopathy score for diagnosis of minimal hepatic encephalopathy in China. (24379595)
2013
13
Randomized, double-blind, controlled study of glycerol phenylbutyrate in hepatic encephalopathy. (23847109)
2013
14
Lack of Clostridium difficile infection in patients treated with rifaximin for hepatic encephalopathy: a retrospective analysis. (23314671)
2013
15
Lactulose is highly potential in prophylaxis of hepatic encephalopathy in patients with cirrhosis and upper gastrointestinal bleeding: results of a controlled randomized trial. (23485720)
2013
16
GABAergic transmission in hepatic encephalopathy. (23624382)
2013
17
Hyaluronic acid levels predict risk of hepatic encephalopathy and liver-related death in HIV/viral hepatitis coinfected patients. (23724041)
2013
18
Rifaximin: a nonsystemic antibiotic for hepatic encephalopathy. (23549218)
2013
19
Gene expression profiling in the cerebral cortex of patients with cirrhosis with and without hepatic encephalopathy. (23325665)
2013
20
Glutamine in the pathogenesis of acute hepatic encephalopathy. (22285152)
2012
21
Psychometric hepatic encephalopathy score for the detection of minimal hepatic encephalopathy in Korean patients with liver cirrhosis. (22743001)
2012
22
In-hospital mortality and economic burden associated with hepatic encephalopathy in the United States from 2005 to 2009. (22642955)
2012
23
Brain default-mode network abnormalities in hepatic encephalopathy: a resting-state functional MRI study. (21567661)
2012
24
Minimal hepatic encephalopathy: follow-up 10 years after successful liver transplantation. (22245876)
2012
25
Hepatic encephalopathy: an update. Preface. (22321472)
2012
26
Psychogenic tetraparesis and bilateral upper limb dystonia, regressive under short propofol-induced sedation and during hepatic encephalopathy. (22902089)
2012
27
Minimal hepatic encephalopathy is associated with falls. (20978484)
2011
28
Ayurveda in critical care: Illustrating Ayurvedic intervention in a case of hepatic encephalopathy. (22529648)
2011
29
Fecal calprotectin is a useful screening parameter for hepatic encephalopathy and spontaneous bacterial peritonitis in cirrhosis. (22093455)
2011
30
Rifaximin improves psychometric performance and health-related quality of life in patients with minimal hepatic encephalopathy (the RIME Trial). (21157444)
2011
31
Role of oxidative/nitrative stress in hepatic encephalopathy induced by thioacetamide. (21699073)
2011
32
To pee or not to pee: ammonia hypothesis of hepatic encephalopathy revisited. (21537121)
2011
33
Absence of neuropsychological impairment in hyperammonaemia in healthy young adults; possible synergism in development of hepatic encephalopathy (HE) symptoms? (21773808)
2011
34
Review of the final report of the 1998 Working Party on definition, nomenclature and diagnosis of hepatic encephalopathy. (22228879)
2011
35
Portal-systemic shunt between the inferior mesenteric vein and inferior vena cava in a patient with hepatic encephalopathy: successful occlusion by balloon-occluded retrograde transvenous obliteration. (21675043)
2011
36
An unusual trigger for a neurogenic left ventricular dysfunction: Tako-Tsubo syndrome associated with hepatic encephalopathy. (21199295)
2011
37
Abnormal brain processing in hepatic encephalopathy: evidence of cerebral reorganization? (20661140)
2010
38
Rifaximin for the treatment of hepatic encephalopathy. (21108586)
2010
39
Effect of surgical portosystemic shunt on prevalence of minimal hepatic encephalopathy in children with extrahepatic portal venous obstruction: assessment by magnetic resonance imaging and psychometry. (20890215)
2010
40
Increase brain lactate in hepatic encephalopathy: cause or consequence? (20600436)
2010
41
Persistence of cognitive impairment after resolution of overt hepatic encephalopathy. (20178797)
2010
42
Effect of oral zinc in hepatic encephalopathy remains unclear. (21050244)
2010
43
Rifaximin in hepatic encephalopathy: more than just a non-absorbable antibiotic? (20561708)
2010
44
Rifaximin treatment in hepatic encephalopathy. (20578275)
2010
45
Performance of the hepatic encephalopathy scoring algorithm in a clinical trial of patients with cirrhosis and severe hepatic encephalopathy. (19455117)
2009
46
Noncirrhotic portal vein thrombosis exhibits neuropsychological and MR changes consistent with minimal hepatic encephalopathy. (16557541)
2006
47
Role of zinc in subclinical hepatic encephalopathy: comparison with somatosensory-evoked potentials. (15012773)
2004
48
Ammonia and GABA-ergic neurotransmission in pathogenesis of hepatic encephalopathy]. (15058165)
2003
49
A study of 3H-PK 11,195 binding to "peripheral-type" benzodiazepine receptors on human lymphocytes. Evidence of decreased binding in hepatic encephalopathy. (1649262)
1991
50
Endogenous benzodiazepine receptor ligands in human and animal hepatic encephalopathy. (2172467)
1990

Variations for Hepatic Encephalopathy

About this section

Expression for genes affiliated with Hepatic Encephalopathy

About this section
Expression patterns in normal tissues for genes affiliated with Hepatic Encephalopathy

Search GEO for disease gene expression data for Hepatic Encephalopathy.

Pathways for genes affiliated with Hepatic Encephalopathy

About this section

Pathways related to Hepatic Encephalopathy according to GeneCards/GeneDecks:

(show all 16)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.5GLS, MAOA
29.5GLS, GLUL
3
Show member pathways
choline degradation I37
glycine biosynthesis III37
serine and glycine biosynthesis37
glycine betaine degradation37
glycine biosynthesis37
9.1MAOB, MAOA
4
Show member pathways
tryptophan degradation X (mammalian, via tryptamine)37
2-amino-3-carboxymuconate semialdehyde degradation to glutaryl-CoA37
tryptophan degradation37
glutaryl-CoA degradation37
serotonin degradation37
NAD de novo biosynthesis37
superpathway of melatonin degradation37
tryptophan degradation to 2-amino-3-carboxymuconate semialdehyde37
tryptophan utilization I37
melatonin degradation I37
9.1MAOA, MAOB
5
Show member pathways
phenylalanine utilization37
noradrenaline and adrenaline degradation37
phenylalanine degradation IV37
9.1MAOB, MAOA
6
Show member pathways
9.1MAOB, MAOA
7
Show member pathways
histamine biosynthesis37
9.1MAOB, MAOA
89.1MAOA, MAOB
9
Show member pathways
9.1MAOB, MAOA
109.1MAOB, MAOA
119.1MAOB, MAOA
129.1MAOA, MAOB
13
Show member pathways
9.1MAOB, MAOA
14
Show member pathways
9.0MAOA, GLUL, GLS
15
Show member pathways
8.2GLS, GLUL, MAOB, MAOA
16
Show member pathways
citrulline-nitric oxide cycle37
arginine degradation I (arginase pathway)37
proline degradation37
glutamine degradation I37
proline biosynthesis I37
superpathway of citrulline metabolism37
proline biosynthesis II (from arginine)37
citrulline biosynthesis37
arginine degradation VI (arginase 2 pathway)37
Urea cycle and metabolism of amino groups37
asparagine biosynthesis I37
4-hydroxyproline degradation I37
citrulline degradation37
8.2GLS, GLUL, MAOB, MAOA

Compounds for genes affiliated with Hepatic Encephalopathy

About this section

Compounds related to Hepatic Encephalopathy according to GeneCards/GeneDecks:

(show top 50)    (show all 161)
idCompoundScoreTop Affiliating Genes
1lazabemide449.6MAOB, MAOA
2pyrazoline449.6MAOA, MAOB
3pirlindole449.5MAOA, MAOB
41,4-diphenyl-2-butene449.5MAOB, MAOA
51-methyl-4-phenyl-2,3-dihydropyridinium449.5MAOA, MAOB
6lazabemide hydrochloride619.5MAOB, MAOA
7rasagiline mesylate619.5MAOB, MAOA
8iproniazid44 1110.5MAOB, MAOA
9isocarboxazid44 1110.5MAOB, MAOA
10bifemelane hydrochloride619.5MAOA, MAOB
11(r)-(-)-deprenyl hydrochloride619.5MAOA, MAOB
124,6-Dihydroxyquinoline249.5MAOB, MAOA
13phenethylamine,449.5MAOA, MAOB
14brofaromine449.5MAOA, MAOB
15toloxatone449.5MAOA, MAOB
163-methoxytyramine44 2410.5MAOA, MAOB
171-Methylhistamine249.4MAOB, MAOA
18milacemide449.4MAOB, MAOA
195-Hydroxykynurenamine249.4MAOB, MAOA
20kynuramine449.4MAOA, MAOB
21dopeg449.4MAOB, MAOA
22harman449.4MAOA, MAOB
23m-tyramine449.4MAOA, MAOB
24oxazolidinone449.4MAOA, MAOB
25monamine449.3MAOA, MAOB
26ladostigil449.3MAOB, MAOA
27linezolid44 1110.2MAOB, MAOA
28harmine449.2TSPO, MAOA, MAOB
29beta-carboline449.2TSPO, MAOA, MAOB
30isatin44 1110.2MAOA, MAOB
31d amphetamine449.2TSPO, MAOA, MAOB
32dimethylamine44 2410.1MAOA, MAOB
33pargyline44 1110.1MAOB, MAOA
34mptp449.1MAOA, MAOB, TSPO
35Ammonia249.1MAOB, GLS, MAOA
36nialamide44 1110.0MAOA, MAOB
37flavin448.9SGCB, MAOA, MAOB
38alpha-ketoglutarate448.9GLS, GLUL, MAOB
39cyclopropane448.8MAOB, MAOA
40norepinephrine44 24 1110.6MAOB, GLUL, MAOA, TSPO
415-hydroxytryptamine448.6MAOA, MAOB, TSPO, SGCB
42ascorbic acid44 249.3GLUL, MAOB, SGCB, MAOA
43gaba448.3GLUL, TSPO, MAOA, MAOB, GLS
44dexamethasone44 50 28 1111.2AQP4, MAOA, MAOB, GLUL, GLS
45histamine44 28 2410.1MAOB, AQP4, SGCB, TSPO, MAOA
46h2o2448.0MAOA, GLUL, TSPO, SGCB, MAOB
47oxygen44 248.9SGCB, GLS, GLUL, MAOB, MAOA
48nmda44 288.7SGCB, TSPO, MAOA, MAOB, GLUL, GLS
49cysteine447.6GLS, MAOB, SGCB, AQP4, GLUL, MAOA
50glutamate447.2TSPO, SGCB, AQP4, MAOA, MAOB, GLUL

GO Terms for genes affiliated with Hepatic Encephalopathy

About this section

Cellular components related to Hepatic Encephalopathy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1mitochondrial outer membraneGO:0057418.8MAOB, MAOA, TSPO
2mitochondrionGO:0057397.5GLS, GLUL, MAOB, MAOA, TSPO

Biological processes related to Hepatic Encephalopathy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cellular amino acid biosynthetic processGO:0086529.4GLS, GLUL
2neurotransmitter secretionGO:0072699.2MAOA, GLS
3synaptic transmissionGO:0072688.7GLS, GLUL, MAOA, TSPO
4small molecule metabolic processGO:0442818.2GLS, GLUL, MAOB, MAOA

Molecular functions related to Hepatic Encephalopathy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1primary amine oxidase activityGO:0081319.1MAOB, MAOA

Products for genes affiliated with Hepatic Encephalopathy

About this section
  • Antibodies
  • Proteins
  • Lysates

Sources for Hepatic Encephalopathy

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet