MCID: HPT019
MIFTS: 55

Hepatic Encephalopathy malady

Rare diseases, Neuronal diseases, Liver diseases categories

Summaries for Hepatic Encephalopathy

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Sources:
44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 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: Hepatic Encephalopathy, also known as portal-systemic encephalopathy, is related to liver cirrhosis and blindness. 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:66 Hepatic encephalopathy (also known as portosystemic encephalopathy) is the occurrence of confusion,... more...

Aliases & Classifications for Hepatic Encephalopathy

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Sources:
9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 46Novoseek, 63UMLS, 59SNOMED-CT, 28ICD9CM, 36MeSH, 41NCIt
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Classifications:

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


Aliases & Descriptions:

hepatic encephalopathy 9 44 11 46 63
portal-systemic encephalopathy 9
encephalopathy, hepatic 44
hepatoencephalopathy 44


External Ids:

Disease Ontology9 DOID:13413
ICD9CM28 572.2
MeSH36 D006501
NCIt41 C79596

Related Diseases for Hepatic Encephalopathy

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Sources:
18GeneCards, 19GeneDecks
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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
1liver cirrhosis31.0GLS, GLUL, MAOA
2blindness30.8MAOB, TSPO
3brain edema30.7AQP4, GLUL
4dementia30.1GLS, MAOB, MAOA, TSPO
5encephalitis30.0AQP4, TSPO
6psychotic disorder29.9MAOA, MAOB
7schizophrenia29.7AQP4, TSPO, MAOA, MAOB, GLUL, GLS
8alzheimer's disease29.7AQP4, TSPO, MAOA, MAOB, GLUL, GLS
9hepatitis11.4
10cerebritis10.6
11acute liver failure10.4
12neuronitis10.3
13alcoholic hepatitis10.3
14liver disease10.3
15status epilepticus10.2
16cortical blindness10.2
17hepatic coma10.2
18diabetes mellitus10.2
19hypertension10.2
20combined oxidative phosphorylation deficiency 110.2
21wilson disease10.1
22spastic paraparesis10.1
23spasticity10.1
24portal hypertension10.1
25portal vein thrombosis10.1
26autonomic neuropathy10.1
27neuropathy10.1
28peritonitis10.1
29urea cycle disorder10.1
30infections, recurrent, with encephalopathy, hepatic dysfunction, and cardiovasuclar malformations10.1
31endotheliitis10.1
32anxiety disorder10.1MAOA
33antisocial personality disorder10.1MAOA
34attention deficit hyperactivity disorder10.1MAOA
35generalized anxiety disorder10.1MAOA, TSPO
36obsessive-compulsive disorder10.0MAOA, TSPO
37stroke, ischemic10.0AQP4, TSPO
38panic disorder10.0MAOA, TSPO
39metabolic acidosis10.0GLUL, GLS
40brain disease10.0AQP4, TSPO
41focal epilepsy10.0TSPO, MAOB
42duchenne muscular dystrophy10.0AQP4, SGCB
43memory impairment10.0MAOB, TSPO
44multiple system atrophy10.0TSPO, MAOB
45personality disorder10.0MAOB, MAOA
46pure autonomic failure10.0MAOB, MAOA
47galactosemia10.0SGCB, TSPO, GLUL
48glioblastoma multiforme10.0GLUL, TSPO, AQP4
49huntington's disease10.0GLS, MAOB, TSPO
50hepatitis c10.0

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



Diseases related to hepatic encephalopathy

Symptoms for Hepatic Encephalopathy

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Drugs & Therapeutics for Hepatic Encephalopathy

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Hepatic Encephalopathy

Drug clinical trials:

Search ClinicalTrials for Hepatic Encephalopathy

Search NIH Clinical Center for Hepatic Encephalopathy

Search CenterWatch for Hepatic Encephalopathy

Inferred drug relations via UMLS63/NDF-RT42:

Genetic Tests for Hepatic Encephalopathy

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Anatomical Context for Hepatic Encephalopathy

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Sources:
34MalaCards
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MalaCards organs/tissues related to Hepatic Encephalopathy:

34
Liver, Brain, Testes, Cortex, Cerebellum, Endothelial, Prefrontal cortex, Small intestine, Colon, Cingulate cortex, Globus pallidus, Thalamus

Animal Models for Hepatic Encephalopathy or affiliated genes

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Sources:
38MGI
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MGI Mouse Phenotypes related to Hepatic Encephalopathy:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053867.7GLS, GLUL, MAOB, MAOA, AQP4

Publications for Hepatic Encephalopathy

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Sources:
53PubMed
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Articles related to Hepatic Encephalopathy:

(show top 50)    (show all 603)
idTitleAuthorsYear
1
Hyperammonemia and systemic inflammatory response syndrome predicts presence of hepatic encephalopathy in dogs with congenital portosystemic shunts. (24392080)
2014
2
Low grade cirrhosis induces cognitive impairment and motor dysfunction in rats: Could be a model for minimal hepatic encephalopathy. (24334168)
2014
3
Efficacy of liver assisting in patients with hepatic encephalopathy with special focus on plasma exchange. (23572273)
2013
4
The nutritional management of hepatic encephalopathy in patients with cirrhosis: International Society for Hepatic Encephalopathy and Nitrogen Metabolism Consensus. (23471642)
2013
5
Cerebral oedema is rare in acute-on-chronic liver failure patients presenting with high-grade hepatic encephalopathy. (23844567)
2013
6
Matrix metalloproteinase-9 as a therapeutic target for the progression of fulminant liver failure with hepatic encephalopathy: A pilot study in mice. (23672352)
2013
7
Neurotransmitter receptor alterations in hepatic encephalopathy: a review. (23466244)
2013
8
Early markers of neural dysfunction and compensation: A model from minimal hepatic encephalopathy. (24333166)
2013
9
Systemic inflammation and ammonia in hepatic encephalopathy. (23224356)
2013
10
Disaccharides in the treatment of hepatic encephalopathy. (23456517)
2013
11
Psychometric hepatic encephalopathy score for diagnosis of minimal hepatic encephalopathy in China. (24379595)
2013
12
Randomized, double-blind, controlled study of glycerol phenylbutyrate in hepatic encephalopathy. (23847109)
2013
13
Lack of Clostridium difficile infection in patients treated with rifaximin for hepatic encephalopathy: a retrospective analysis. (23314671)
2013
14
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
15
GABAergic transmission in hepatic encephalopathy. (23624382)
2013
16
Hyaluronic acid levels predict risk of hepatic encephalopathy and liver-related death in HIV/viral hepatitis coinfected patients. (23724041)
2013
17
Rifaximin: a nonsystemic antibiotic for hepatic encephalopathy. (23549218)
2013
18
Gene expression profiling in the cerebral cortex of patients with cirrhosis with and without hepatic encephalopathy. (23325665)
2013
19
Glutamine in the pathogenesis of acute hepatic encephalopathy. (22285152)
2012
20
Psychometric hepatic encephalopathy score for the detection of minimal hepatic encephalopathy in Korean patients with liver cirrhosis. (22743001)
2012
21
In-hospital mortality and economic burden associated with hepatic encephalopathy in the United States from 2005 to 2009. (22642955)
2012
22
Brain default-mode network abnormalities in hepatic encephalopathy: a resting-state functional MRI study. (21567661)
2012
23
Minimal hepatic encephalopathy: follow-up 10 years after successful liver transplantation. (22245876)
2012
24
Hepatic encephalopathy: an update. Preface. (22321472)
2012
25
Psychogenic tetraparesis and bilateral upper limb dystonia, regressive under short propofol-induced sedation and during hepatic encephalopathy. (22902089)
2012
26
Minimal hepatic encephalopathy is associated with falls. (20978484)
2011
27
Ayurveda in critical care: Illustrating Ayurvedic intervention in a case of hepatic encephalopathy. (22529648)
2011
28
Fecal calprotectin is a useful screening parameter for hepatic encephalopathy and spontaneous bacterial peritonitis in cirrhosis. (22093455)
2011
29
Rifaximin improves psychometric performance and health-related quality of life in patients with minimal hepatic encephalopathy (the RIME Trial). (21157444)
2011
30
Role of oxidative/nitrative stress in hepatic encephalopathy induced by thioacetamide. (21699073)
2011
31
To pee or not to pee: ammonia hypothesis of hepatic encephalopathy revisited. (21537121)
2011
32
Absence of neuropsychological impairment in hyperammonaemia in healthy young adults; possible synergism in development of hepatic encephalopathy (HE) symptoms? (21773808)
2011
33
Review of the final report of the 1998 Working Party on definition, nomenclature and diagnosis of hepatic encephalopathy. (22228879)
2011
34
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
35
An unusual trigger for a neurogenic left ventricular dysfunction: Tako-Tsubo syndrome associated with hepatic encephalopathy. (21199295)
2011
36
Spectral and dynamic electroencephalogram abnormalities are correlated to psychometric test performance in hepatic encephalopathy. (21612322)
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

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Expression for genes affiliated with Hepatic Encephalopathy

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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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

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Sources:
51PathCards, 56Reactome, 39NCBI BioSystems Database, 31KEGG, 52PharmGKB, 61Thomson Reuters
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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 I39
glycine biosynthesis III39
serine and glycine biosynthesis39
glycine betaine degradation39
glycine biosynthesis39
9.1MAOB, MAOA
4
Show member pathways
tryptophan degradation X (mammalian, via tryptamine)39
2-amino-3-carboxymuconate semialdehyde degradation to glutaryl-CoA39
tryptophan degradation39
glutaryl-CoA degradation39
serotonin degradation39
NAD de novo biosynthesis39
superpathway of melatonin degradation39
tryptophan degradation to 2-amino-3-carboxymuconate semialdehyde39
tryptophan utilization I39
melatonin degradation I39
9.1MAOA, MAOB
5
Show member pathways
phenylalanine utilization39
noradrenaline and adrenaline degradation39
phenylalanine degradation IV39
9.1MAOB, MAOA
6
Show member pathways
9.1MAOB, MAOA
7
Show member pathways
histamine biosynthesis39
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 cycle39
arginine degradation I (arginase pathway)39
proline degradation39
glutamine degradation I39
proline biosynthesis I39
superpathway of citrulline metabolism39
proline biosynthesis II (from arginine)39
citrulline biosynthesis39
arginine degradation VI (arginase 2 pathway)39
Urea cycle and metabolism of amino groups39
asparagine biosynthesis I39
4-hydroxyproline degradation I39
citrulline degradation39
8.2GLS, GLUL, MAOB, MAOA

Compounds for genes affiliated with Hepatic Encephalopathy

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Sources:
46Novoseek, 62Tocris Bioscience, 12DrugBank, 25HMDB, 52PharmGKB, 30IUPHAR
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Compounds related to Hepatic Encephalopathy according to GeneCards/GeneDecks:

(show top 50)    (show all 161)
idCompoundScoreTop Affiliating Genes
1lazabemide469.6MAOB, MAOA
2pyrazoline469.6MAOA, MAOB
3pirlindole469.5MAOA, MAOB
41,4-diphenyl-2-butene469.5MAOB, MAOA
51-methyl-4-phenyl-2,3-dihydropyridinium469.5MAOA, MAOB
6lazabemide hydrochloride629.5MAOB, MAOA
7rasagiline mesylate629.5MAOB, MAOA
8iproniazid46 1210.5MAOB, MAOA
9isocarboxazid46 1210.5MAOB, MAOA
10bifemelane hydrochloride629.5MAOA, MAOB
11(r)-(-)-deprenyl hydrochloride629.5MAOA, MAOB
124,6-Dihydroxyquinoline259.5MAOB, MAOA
13phenethylamine,469.5MAOA, MAOB
14brofaromine469.5MAOA, MAOB
15toloxatone469.5MAOA, MAOB
163-methoxytyramine46 2510.5MAOA, MAOB
171-Methylhistamine259.4MAOB, MAOA
18milacemide469.4MAOB, MAOA
195-Hydroxykynurenamine259.4MAOB, MAOA
20kynuramine469.4MAOA, MAOB
21dopeg469.4MAOB, MAOA
22harman469.4MAOA, MAOB
23m-tyramine469.4MAOA, MAOB
24oxazolidinone469.4MAOA, MAOB
25monamine469.3MAOA, MAOB
26ladostigil469.3MAOB, MAOA
27linezolid46 1210.2MAOB, MAOA
28harmine469.2TSPO, MAOA, MAOB
29beta-carboline469.2TSPO, MAOA, MAOB
30isatin46 1210.2MAOA, MAOB
31d amphetamine469.2TSPO, MAOA, MAOB
32dimethylamine46 2510.1MAOA, MAOB
33pargyline46 1210.1MAOB, MAOA
34mptp469.1MAOA, MAOB, TSPO
35Ammonia259.1MAOB, GLS, MAOA
36nialamide46 1210.0MAOA, MAOB
37flavin468.9SGCB, MAOA, MAOB
38alpha-ketoglutarate468.9GLS, GLUL, MAOB
39cyclopropane468.8MAOB, MAOA
40norepinephrine46 25 1210.6MAOB, GLUL, MAOA, TSPO
415-hydroxytryptamine468.6MAOA, MAOB, TSPO, SGCB
42ascorbic acid46 259.3GLUL, MAOB, SGCB, MAOA
43gaba468.3GLUL, TSPO, MAOA, MAOB, GLS
44dexamethasone46 52 30 1211.2AQP4, MAOA, MAOB, GLUL, GLS
45histamine46 30 2510.1MAOB, AQP4, SGCB, TSPO, MAOA
46h2o2468.0MAOA, GLUL, TSPO, SGCB, MAOB
47oxygen46 258.9SGCB, GLS, GLUL, MAOB, MAOA
48nmda46 308.7SGCB, TSPO, MAOA, MAOB, GLUL, GLS
49cysteine467.6GLS, MAOB, SGCB, AQP4, GLUL, MAOA
50glutamate467.2TSPO, SGCB, AQP4, MAOA, MAOB, GLUL

GO Terms for genes affiliated with Hepatic Encephalopathy

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17Gene Ontology
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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

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Hepatic Encephalopathy

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet