MCID: HPT019
MIFTS: 51

Hepatic Encephalopathy malady

Rare diseases, Liver diseases categories

Aliases & Classifications for Hepatic Encephalopathy

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Hepatic Encephalopathy, Aliases & Descriptions:

Name: Hepatic Encephalopathy 9 41 11 43 60
Portal-Systemic Encephalopathy 9
 
Encephalopathy, Hepatic 41
Hepatoencephalopathy 41


Classifications:

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


External Ids:

Disease Ontology9 DOID:13413
NCIt38 C79596
MeSH33 D006501
ICD9CM27 572.2

Summaries for Hepatic Encephalopathy

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NIH Rare Diseases:41 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 skeletal muscle, and related mouse phenotype behavior/neurological.

Wikipedia:63 Hepatic encephalopathy (also known as portosystemic encephalopathy) is the occurrence of confusion,... more...

Related Diseases for Hepatic Encephalopathy

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

(show top 50)    (show all 96)
idRelated DiseaseScoreTop Affiliating Genes
1brain edema31.0AQP4, GLUL
2liver cirrhosis30.9GLS, GLUL, MAOA
3blindness30.8MAOB, TSPO
4encephalitis30.1AQP4, TSPO
5psychotic disorder29.9MAOA, MAOB
6dementia29.8GLS, MAOB, MAOA, TSPO
7schizophrenia28.9AQP4, TSPO, MAOA, MAOB, GLUL, GLS
8hepatitis11.4
9cerebritis10.7
10anxiety disorder10.4MAOA
11acute liver failure10.4
12hepatoencephalopathy due to combined oxidative phosphorylation deficiency type 110.4
13antisocial personality disorder10.4MAOA
14infections, recurrent, with encephalopathy, hepatic dysfunction, and cardiovasuclar malformations10.3
15liver disease10.3
16neuronitis10.3
17attention deficit-hyperactivity disorder10.3MAOA
18alcoholic hepatitis10.3
19status epilepticus10.2
20portal vein thrombosis10.2
21cortical blindness10.2
22generalized anxiety disorder10.2MAOA, TSPO
23stroke, ischemic10.2AQP4, TSPO
24obsessive-compulsive disorder10.2MAOA, TSPO
25panic disorder10.2MAOA, TSPO
26wilson disease10.2
27spastic paraparesis10.2
28spasticity10.2
29hepatitis b10.1
30urea cycle disorder10.1
31portal hypertension10.1
32hypokalemia10.1
33hepatic coma10.1
34autonomic neuropathy10.1
35neuropathy10.1
36peritonitis10.1
37patent ductus venosus10.1
38endotheliitis10.1
39metabolic acidosis10.1GLUL, GLS
40brain disease10.1AQP4, TSPO
41focal epilepsy10.1TSPO, MAOB
42duchenne muscular dystrophy10.1AQP4, SGCB
43multiple system atrophy10.1TSPO, MAOB
44personality disorder10.0MAOB, MAOA
45pure autonomic failure10.0MAOB, MAOA
46hepatocellular carcinoma10.0
47hepatitis c virus10.0
48orotic aciduria10.0
49cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 210.0
50lymphoproliferative syndrome, x-linked, 110.0

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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Drug clinical trials:

Search ClinicalTrials for Hepatic Encephalopathy

Search NIH Clinical Center for Hepatic Encephalopathy

Inferred drug relations via UMLS60/NDF-RT39:

Genetic Tests for Hepatic Encephalopathy

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Anatomical Context for Hepatic Encephalopathy

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MalaCards organs/tissues related to Hepatic Encephalopathy:

31
Liver, Brain, Skeletal muscle, Testes, Cortex, Thyroid, Cerebellum, Endothelial, Prefrontal cortex, Small intestine, Colon, Cingulate cortex, Globus pallidus, Thalamus

Animal Models for Hepatic Encephalopathy or affiliated genes

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MGI Mouse Phenotypes related to Hepatic Encephalopathy:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053867.7GLS, GLUL, MAOB, MAOA, AQP4

Publications for Hepatic Encephalopathy

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Articles related to Hepatic Encephalopathy:

(show top 50)    (show all 650)
idTitleAuthorsYear
1
A genetic variant in the promoter of phosphate-activated glutaminase (GLS) is associated with hepatic encephalopathy. (25880019)
2015
2
Expression of glutamine transporter isoforms in cerebral cortex of rats with chronic hepatic encephalopathy. (25842041)
2015
3
Is it time to target gut dysbiosis and immune dysfunction in the therapy of hepatic encephalopathy? (25846450)
2015
4
Hyperammonemia and systemic inflammatory response syndrome predicts presence of hepatic encephalopathy in dogs with congenital portosystemic shunts. (24392080)
2014
5
Liver: The gut is a key target of therapy in hepatic encephalopathy. (25348849)
2014
6
A SPECIAL MEETING REVIEW EDITIONAdvances in Hepatic Encephalopathy From EASL 2014: A Review of Selected Presentations From the 49th Annual Meeting of the European Association for the Study of the LiverApril 9-13, 2014 a8c London, United KingdomWith Expert Commentary by: Steven L. Flamm, MDProfessor of Medicine and SurgeryDivision of Gastroenterology and HepatologyChief of the Liver Transplantation ProgranNorthwestern University Feinberg School of MedicineChicago, Illinois. (25892983)
2014
7
Low grade cirrhosis induces cognitive impairment and motor dysfunction in rats: Could be a model for minimal hepatic encephalopathy. (24334168)
2014
8
Efficacy of liver assisting in patients with hepatic encephalopathy with special focus on plasma exchange. (23572273)
2013
9
The nutritional management of hepatic encephalopathy in patients with cirrhosis: International Society for Hepatic Encephalopathy and Nitrogen Metabolism Consensus. (23471642)
2013
10
Cerebral oedema is rare in acute-on-chronic liver failure patients presenting with high-grade hepatic encephalopathy. (23844567)
2013
11
Matrix metalloproteinase-9 as a therapeutic target for the progression of fulminant liver failure with hepatic encephalopathy: A pilot study in mice. (23672352)
2013
12
Neurotransmitter receptor alterations in hepatic encephalopathy: a review. (23466244)
2013
13
Early markers of neural dysfunction and compensation: A model from minimal hepatic encephalopathy. (24333166)
2013
14
Systemic inflammation and ammonia in hepatic encephalopathy. (23224356)
2013
15
Disaccharides in the treatment of hepatic encephalopathy. (23456517)
2013
16
Psychometric hepatic encephalopathy score for diagnosis of minimal hepatic encephalopathy in China. (24379595)
2013
17
Randomized, double-blind, controlled study of glycerol phenylbutyrate in hepatic encephalopathy. (23847109)
2013
18
Lack of Clostridium difficile infection in patients treated with rifaximin for hepatic encephalopathy: a retrospective analysis. (23314671)
2013
19
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
20
GABAergic transmission in hepatic encephalopathy. (23624382)
2013
21
Hyaluronic acid levels predict risk of hepatic encephalopathy and liver-related death in HIV/viral hepatitis coinfected patients. (23724041)
2013
22
Rifaximin: a nonsystemic antibiotic for hepatic encephalopathy. (23549218)
2013
23
Glutamine in the pathogenesis of acute hepatic encephalopathy. (22285152)
2012
24
Psychometric hepatic encephalopathy score for the detection of minimal hepatic encephalopathy in Korean patients with liver cirrhosis. (22743001)
2012
25
In-hospital mortality and economic burden associated with hepatic encephalopathy in the United States from 2005 to 2009. (22642955)
2012
26
Brain default-mode network abnormalities in hepatic encephalopathy: a resting-state functional MRI study. (21567661)
2012
27
Minimal hepatic encephalopathy: follow-up 10 years after successful liver transplantation. (22245876)
2012
28
Hepatic encephalopathy: an update. Preface. (22321472)
2012
29
Psychogenic tetraparesis and bilateral upper limb dystonia, regressive under short propofol-induced sedation and during hepatic encephalopathy. (22902089)
2012
30
Minimal hepatic encephalopathy is associated with falls. (20978484)
2011
31
Ayurveda in critical care: Illustrating Ayurvedic intervention in a case of hepatic encephalopathy. (22529648)
2011
32
Fecal calprotectin is a useful screening parameter for hepatic encephalopathy and spontaneous bacterial peritonitis in cirrhosis. (22093455)
2011
33
Rifaximin improves psychometric performance and health-related quality of life in patients with minimal hepatic encephalopathy (the RIME Trial). (21157444)
2011
34
Role of oxidative/nitrative stress in hepatic encephalopathy induced by thioacetamide. (21699073)
2011
35
To pee or not to pee: ammonia hypothesis of hepatic encephalopathy revisited. (21537121)
2011
36
Absence of neuropsychological impairment in hyperammonaemia in healthy young adults; possible synergism in development of hepatic encephalopathy (HE) symptoms? (21773808)
2011
37
Review of the final report of the 1998 Working Party on definition, nomenclature and diagnosis of hepatic encephalopathy. (22228879)
2011
38
Abnormal brain processing in hepatic encephalopathy: evidence of cerebral reorganization? (20661140)
2010
39
Rifaximin for the treatment of hepatic encephalopathy. (21108586)
2010
40
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
41
Increase brain lactate in hepatic encephalopathy: cause or consequence? (20600436)
2010
42
Persistence of cognitive impairment after resolution of overt hepatic encephalopathy. (20178797)
2010
43
Effect of oral zinc in hepatic encephalopathy remains unclear. (21050244)
2010
44
Rifaximin in hepatic encephalopathy: more than just a non-absorbable antibiotic? (20561708)
2010
45
Rifaximin treatment in hepatic encephalopathy. (20578275)
2010
46
Performance of the hepatic encephalopathy scoring algorithm in a clinical trial of patients with cirrhosis and severe hepatic encephalopathy. (19455117)
2009
47
Noncirrhotic portal vein thrombosis exhibits neuropsychological and MR changes consistent with minimal hepatic encephalopathy. (16557541)
2006
48
Role of zinc in subclinical hepatic encephalopathy: comparison with somatosensory-evoked potentials. (15012773)
2004
49
Ammonia and GABA-ergic neurotransmission in pathogenesis of hepatic encephalopathy]. (15058165)
2003
50
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

Variations for Hepatic Encephalopathy

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Expression for genes affiliated with Hepatic Encephalopathy

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Search GEO for disease gene expression data for Hepatic Encephalopathy.

Pathways for genes affiliated with Hepatic Encephalopathy

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Pathways related to Hepatic Encephalopathy according to GeneCards Suite gene sharing:

(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 I36
glycine biosynthesis III36
serine and glycine biosynthesis36
glycine betaine degradation36
glycine biosynthesis36
9.1MAOB, MAOA
4
Show member pathways
tryptophan degradation X (mammalian, via tryptamine)36
2-amino-3-carboxymuconate semialdehyde degradation to glutaryl-CoA36
tryptophan degradation36
glutaryl-CoA degradation36
serotonin degradation36
NAD de novo biosynthesis36
superpathway of melatonin degradation36
tryptophan degradation to 2-amino-3-carboxymuconate semialdehyde36
tryptophan utilization I36
melatonin degradation I36
9.1MAOA, MAOB
5
Show member pathways
phenylalanine utilization36
noradrenaline and adrenaline degradation36
phenylalanine degradation IV36
9.1MAOB, MAOA
6
Show member pathways
9.1MAOB, MAOA
7
Show member pathways
histamine biosynthesis36
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 cycle36
arginine degradation I (arginase pathway)36
proline degradation36
glutamine degradation I36
proline biosynthesis I36
superpathway of citrulline metabolism36
proline biosynthesis II (from arginine)36
citrulline biosynthesis36
arginine degradation VI (arginase 2 pathway)36
Urea cycle and metabolism of amino groups36
asparagine biosynthesis I36
4-hydroxyproline degradation I36
citrulline degradation36
8.2GLS, GLUL, MAOB, MAOA

Compounds for genes affiliated with Hepatic Encephalopathy

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Compounds related to Hepatic Encephalopathy according to GeneCards Suite gene sharing:

(show top 50)    (show all 161)
idCompoundScoreTop Affiliating Genes
1lazabemide439.6MAOB, MAOA
2pyrazoline439.6MAOA, MAOB
3pirlindole439.5MAOA, MAOB
41,4-diphenyl-2-butene439.5MAOB, MAOA
51-methyl-4-phenyl-2,3-dihydropyridinium439.5MAOA, MAOB
6lazabemide hydrochloride599.5MAOB, MAOA
7rasagiline mesylate599.5MAOB, MAOA
8iproniazid43 1210.5MAOB, MAOA
9isocarboxazid43 1210.5MAOB, MAOA
10bifemelane hydrochloride599.5MAOA, MAOB
11(r)-(-)-deprenyl hydrochloride599.5MAOA, MAOB
124,6-Dihydroxyquinoline249.5MAOB, MAOA
13phenethylamine,439.5MAOA, MAOB
14brofaromine439.5MAOA, MAOB
15toloxatone439.5MAOA, MAOB
163-methoxytyramine43 2410.5MAOA, MAOB
171-Methylhistamine249.4MAOB, MAOA
18milacemide439.4MAOB, MAOA
195-Hydroxykynurenamine249.4MAOB, MAOA
20kynuramine439.4MAOA, MAOB
21dopeg439.4MAOB, MAOA
22harman439.4MAOA, MAOB
23m-tyramine439.4MAOA, MAOB
24oxazolidinone439.4MAOA, MAOB
25monamine439.3MAOA, MAOB
26ladostigil439.3MAOB, MAOA
27linezolid43 1210.2MAOB, MAOA
28harmine439.2TSPO, MAOA, MAOB
29beta-carboline439.2TSPO, MAOA, MAOB
30isatin43 1210.2MAOA, MAOB
31d amphetamine439.2TSPO, MAOA, MAOB
32dimethylamine43 2410.1MAOA, MAOB
33pargyline43 1210.1MAOB, MAOA
34mptp439.1MAOA, MAOB, TSPO
35Ammonia249.1MAOB, GLS, MAOA
36nialamide43 1210.0MAOA, MAOB
37flavin438.9SGCB, MAOA, MAOB
38alpha-ketoglutarate438.9GLS, GLUL, MAOB
39cyclopropane438.8MAOB, MAOA
40norepinephrine43 24 1210.6MAOB, GLUL, MAOA, TSPO
415-hydroxytryptamine438.6MAOA, MAOB, TSPO, SGCB
42ascorbic acid43 249.3GLUL, MAOB, SGCB, MAOA
43gaba438.3GLUL, TSPO, MAOA, MAOB, GLS
44dexamethasone43 49 28 1211.2AQP4, MAOA, MAOB, GLUL, GLS
45histamine43 28 2410.1MAOB, AQP4, SGCB, TSPO, MAOA
46h2o2438.0MAOA, GLUL, TSPO, SGCB, MAOB
47oxygen43 248.9SGCB, GLS, GLUL, MAOB, MAOA
48nmda43 288.7SGCB, TSPO, MAOA, MAOB, GLUL, GLS
49cysteine437.6GLS, MAOB, SGCB, AQP4, GLUL, MAOA
50glutamate437.2TSPO, SGCB, AQP4, MAOA, MAOB, GLUL

GO Terms for genes affiliated with Hepatic Encephalopathy

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Cellular components related to Hepatic Encephalopathy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1mitochondrial outer membraneGO:00057418.8MAOB, MAOA, TSPO
2mitochondrionGO:00057397.5GLS, GLUL, MAOB, MAOA, TSPO

Biological processes related to Hepatic Encephalopathy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cellular amino acid biosynthetic processGO:00086529.4GLS, GLUL
2neurotransmitter secretionGO:00072699.2MAOA, GLS
3synaptic transmissionGO:00072688.7GLS, GLUL, MAOA, TSPO
4small molecule metabolic processGO:00442818.2GLS, GLUL, MAOB, MAOA

Molecular functions related to Hepatic Encephalopathy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1primary amine oxidase activityGO:00081319.1MAOB, MAOA

Products for genes affiliated with Hepatic Encephalopathy

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Sources for Hepatic Encephalopathy

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet