MCID: EXT006
MIFTS: 38

Extrahepatic Cholestasis

Categories: Gastrointestinal diseases

Aliases & Classifications for Extrahepatic Cholestasis

MalaCards integrated aliases for Extrahepatic Cholestasis:

Name: Extrahepatic Cholestasis 12 15 17
Cholestasis, Extrahepatic 44 72
Extrahepatic Obstructive Biliary Disease 12
Extrahepatic Biliary Stasis 12
Cholestasis Extrahepatic 55

Classifications:



External Ids:

Disease Ontology 12 DOID:13619
MeSH 44 D001651
NCIt 50 C34420
SNOMED-CT 68 20719006 8262006
UMLS 72 C0005398

Summaries for Extrahepatic Cholestasis

MalaCards based summary : Extrahepatic Cholestasis, also known as cholestasis, extrahepatic, is related to cholangitis and cholelithiasis, and has symptoms including icterus An important gene associated with Extrahepatic Cholestasis is ABCC3 (ATP Binding Cassette Subfamily C Member 3), and among its related pathways/superpathways are Glucose / Energy Metabolism and NRF2 pathway. The drugs DEFB1 protein, human and Diclofenac have been mentioned in the context of this disorder. Affiliated tissues include liver, testes and pancreas, and related phenotypes are homeostasis/metabolism and integument

Related Diseases for Extrahepatic Cholestasis

Diseases related to Extrahepatic Cholestasis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 62)
# Related Disease Score Top Affiliating Genes
1 cholangitis 30.5 GPT GGT1
2 cholelithiasis 30.3 GPT CYP7A1
3 choledocholithiasis 30.2 GPT ALPP
4 obstructive jaundice 30.0 GPT GGT1 ALPP
5 liver disease 29.4 GPT GGT1 ALPP
6 cholestasis 29.0 GPT GGT1 CYP7A1 ALPP ABCC3 ABCC2
7 biliary tract disease 28.8 GGT1 CYP7A1 ABCC3 ABCC2
8 fibrosis of extraocular muscles, congenital, 1 10.2
9 biliary atresia 10.2
10 liver cirrhosis 10.2
11 cholangiocarcinoma 10.2
12 cholecystitis 10.2
13 intrahepatic cholangiocarcinoma 10.2
14 pancreatitis 10.2
15 immune deficiency disease 10.1
16 pancreatic cancer 10.1
17 bile duct cysts 10.1
18 alcoholic hepatitis 10.1
19 viral hepatitis 10.1
20 gallbladder cancer 10.1
21 acquired immunodeficiency syndrome 10.1
22 alcohol dependence 9.9
23 autoimmune disease 9.9
24 hepatocellular carcinoma 9.9
25 alagille syndrome 1 9.9
26 kaposi sarcoma 9.9
27 neurofibromatosis, type i 9.9
28 neurofibromatosis, type iv, of riccardi 9.9
29 biliary atresia, extrahepatic 9.9
30 myeloma, multiple 9.9
31 pancreatic agenesis 1 9.9
32 gallbladder disease 1 9.9
33 gastrointestinal stromal tumor 9.9
34 pontine tegmental cap dysplasia 9.9
35 helix syndrome 9.9
36 metabolic acidosis 9.9
37 lymphoma 9.9
38 lymphoproliferative syndrome 9.9
39 portal hypertension 9.9
40 cardiac tamponade 9.9
41 hepatorenal syndrome 9.9
42 keratoconjunctivitis sicca 9.9
43 gangliocytoma 9.9
44 pancreas disease 9.9
45 acalculous cholecystitis 9.9
46 anuria 9.9
47 ischemia 9.9
48 ganglioneuroma 9.9
49 connective tissue disease 9.9
50 keratoconjunctivitis 9.9

Graphical network of the top 20 diseases related to Extrahepatic Cholestasis:



Diseases related to Extrahepatic Cholestasis

Symptoms & Phenotypes for Extrahepatic Cholestasis

UMLS symptoms related to Extrahepatic Cholestasis:


icterus

MGI Mouse Phenotypes related to Extrahepatic Cholestasis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.56 ABCC1 ABCC2 ABCC3 CYP7A1 FGF19 GGT1
2 integument MP:0010771 8.92 ABCC3 CYP7A1 GGT1 TFAM

Drugs & Therapeutics for Extrahepatic Cholestasis

Drugs for Extrahepatic Cholestasis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 29)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 DEFB1 protein, human Phase 3
2
Diclofenac Approved, Vet_approved 15307-86-5 3033
3
Phenobarbital Approved, Investigational 50-06-6 4763
4
Aspirin Approved, Vet_approved 50-78-2 2244
5 Neurotransmitter Agents
6 Cytochrome P-450 CYP3A Inducers
7 Cytochrome P-450 Enzyme Inducers
8 GABA Agents
9 Central Nervous System Depressants
10 Hypnotics and Sedatives
11 GABA Modulators
12 Excitatory Amino Acid Antagonists
13 Anticonvulsants
14 Excitatory Amino Acids
15 Hematinics
16 Liver Extracts
17 Antibodies
18 Immunologic Factors
19 Immunoglobulins
20 Cyclooxygenase Inhibitors
21 Analgesics, Non-Narcotic
22 Fibrinolytic Agents
23 Peripheral Nervous System Agents
24 Analgesics
25 Anti-Inflammatory Agents, Non-Steroidal
26 Platelet Aggregation Inhibitors
27 Anti-Inflammatory Agents
28 Antipyretics
29 Antirheumatic Agents

Interventional clinical trials:

(show all 14)
# Name Status NCT ID Phase Drugs
1 A Multi-Center, Single Arm, Prospective Study of the WallFlex™ Biliary Fully-covered Stent for the Palliative Treatment of Malignant Bile Duct Obstruction Completed NCT00433771 Phase 3
2 Multicenter Study of EUS-guided Biliary Drainage Using Partially Covered With a Dedicated Introducer or Fully Covered Antimigration Metallic Stents Completed NCT02114320 Phase 3
3 Reconstruction of Postcholecystectomy Bile Duct Injury and Bile Duct Defects With the Round Ligament Unknown status NCT03030573
4 Intramuscular Diclofenac in the Prevention of Post-ERCP Pancreatitis Unknown status NCT01717599 Diclofenac;normal saline
5 Role of Complete Blood Picture in Predicting the Etiology of Extrahepatic Cholestasis Completed NCT03237247
6 Multicenter Study of EUS-guided Biliary Drainage Versus Percutanenous Transhepatic Biliary Darinage for Malignant Biliary Obstruction After Failed ERCP Completed NCT02103413
7 Ultrasound-guided Percutaneous Biliary Drainage With Primary Metal Implantation by Endoscopic Luminal Guidance in Patients With Malignant Extrahepatic Bile Duct Obstruction Completed NCT03541590
8 Randomized Controlled Trial of Short Versus Long Wire and Small Versus Standard Sphincterotomes for Initial Biliary Cannulation Completed NCT02197338
9 Prospective Comparison of Transient Elastography and Mini-laparoscopic Guided Liver Biopsy in Autoimmune Hepatitis Completed NCT01905254
10 Treatment of Malignant Distal Biliary Obstruction - a Randomized Study Recruiting NCT03821025
11 Development of a New Immunochemistry Method Using Antibodies of Aminoacyl-tRNA Synthetases Group and Aminoacyl-transfer Ribonucleic Acid Synthetases-interacting Multifunctional protein2 Lacking Exon 2 in the Bile Duct Cancer Cell; Multicenter Study Recruiting NCT03708445
12 The Effect of Aspirin on Patency of Metal Stent in Malignant Distal Bile Duct Obstruction Recruiting NCT03279809 Aspirin;Placebo
13 Prospective Multi-site Study of Ultrasound-guided Percutaneous Biliary Drainage and Endoscopic Ultrasound-guided Biliary Drainage With Implantation of a Self-expanding Metal Stent in Patients With Malignant, Distal Bile Duct Obstruction Recruiting NCT03546049
14 Volumetric Laser Endomicroscopy's (VLE) Diagnostic Accuracy Validation Study: Impact on Clinical Management Study Enrolling by invitation NCT03951324

Search NIH Clinical Center for Extrahepatic Cholestasis

Cochrane evidence based reviews: cholestasis, extrahepatic

Genetic Tests for Extrahepatic Cholestasis

Anatomical Context for Extrahepatic Cholestasis

MalaCards organs/tissues related to Extrahepatic Cholestasis:

41
Liver, Testes, Pancreas, Brain, Kidney, T Cells, Small Intestine

Publications for Extrahepatic Cholestasis

Articles related to Extrahepatic Cholestasis:

(show top 50) (show all 562)
# Title Authors PMID Year
1
High expression of the bile salt-homeostatic hormone fibroblast growth factor 19 in the liver of patients with extrahepatic cholestasis. 9 38
19185005 2009
2
Gene expression of ABC proteins in hepatocellular carcinoma, perineoplastic tissue, and liver diseases. 9 38
12428063 2002
3
Hepatic secretion of conjugated drugs and endogenous substances. 9 38
11076395 2000
4
Lipoprotein-X in patients with cirrhosis: its relationship to cholestasis and hypercholesterolemia. 9 38
9794902 1998
5
[Respective value of serum activity of alkaline phosphatase, gamma-glutamyl transpeptidase and 5'-nucleotidase in the diagnosis of intra- and extrahepatic cholestasis]. 9 38
9208002 1997
6
Increased intercellular adhesion molecule-1 serum concentration in cholestasis. 9 38
9007716 1996
7
Serum gamma-glutamyl transpeptidase in chronic extrahepatic cholestasis. 9 38
1983169 1990
8
Bile Modulates Secretion of Incretins and Insulin: A Study of Human Extrahepatic Cholestasis. 38
30874733 2019
9
Persistent cholestasis resulting from duodenal papillary carcinoma in an adolescent male: A case report. 38
31145285 2019
10
Exogenous putrescine affects polyamine and arginine metabolism in rat liver following bile ductus ligation. 38
30193084 2018
11
Retrospective analysis of cases with an ectopic opening of the common bile duct into duodenal bulb. 38
30062869 2018
12
Acute Isolated Hyperbilirubinemia as a Presentation of Alcoholic Liver Disease: A Case Report and Literature Review. 38
29623226 2018
13
Prostaglandin E1 Preconditioning Attenuates Liver Ischemia Reperfusion Injury in a Rat Model of Extrahepatic Cholestasis. 38
29511679 2018
14
Protective effect of dehydroandrographolide on obstructive cholestasis in bile duct-ligated mice. 38
29152129 2017
15
Hepatoprotective activities of rosmarinic acid against extrahepatic cholestasis in rats. 38
28789951 2017
16
Adipose-derived mesenchymal stem cells slow disease progression of acute-on-chronic liver failure. 38
28501004 2017
17
Atorvastatin does not protect against ischemia-reperfusion damage in cholestatic rat livers. 38
28399849 2017
18
Use of 4-Fr versus 6-Fr Nasobiliary Catheter for Biliary Drainage: A Prospective, Multicenter, Randomized, Controlled Study. 38
28503061 2017
19
[Elevated liver enzymes]. 38
27824421 2016
20
Lipid profiling of lipoprotein X: Implications for dyslipidemia in cholestasis. 38
27112638 2016
21
Role of farnesoid X receptor in cholestasis. 38
27383832 2016
22
Qualification for endoscopic retrograde cholangiopancreatography in the diagnosis and treatment of extrahepatic cholestasis caused by choledocholithiasis. 38
26788082 2015
23
[CHOLESTASIS AND INFLAMMATION OF THE PANCREAS IN FAMILY MEDICINE]. 38
29083844 2015
24
Neonatal McCune-Albright syndrome with systemic involvement: a case report. 38
26341786 2015
25
Antioxidant Effect of Sepia Ink Extract on Extrahepatic Cholestasis Induced by Bile Duct Ligation in Rats. 38
26383596 2015
26
Expression of renal Oat5 and NaDC1 transporters in rats with acute biliary obstruction. 38
26269671 2015
27
Activation of the renin-angiotensin system stimulates biliary hyperplasia during cholestasis induced by extrahepatic bile duct ligation. 38
25678505 2015
28
Bile acid-induced necrosis in primary human hepatocytes and in patients with obstructive cholestasis. 38
25636263 2015
29
Efficacy of diffusion-weighted magnetic resonance imaging in the evaluation of extrahepatic cholestasis-related hepatic fibrosis. 38
26281340 2015
30
Biochemical profile of bile fluid in patients with malignant cholestasis in comparison with cholestasis due to gall stone. 38
26644885 2015
31
Factors That Could Impact on Liver Fibrosis Staging by Transient Elastography. 38
26770833 2015
32
SIRT1/PGC-1α signaling protects hepatocytes against mitochondrial oxidative stress induced by bile acids. 38
25789761 2015
33
γ-Glutamyl transpeptidase level as a screening marker among diverse etiologies of infantile intrahepatic cholestasis. 38
25141230 2014
34
Increase of glycosaminoglycans and metalloproteinases 2 and 9 in liver extracellular matrix on early stages of extrahepatic cholestasis. 38
25591159 2014
35
The canine hepatic progenitor cell niche: molecular characterisation in health and disease. 38
24923752 2014
36
[Effect of biliary obstruction on lipoprotein(a) concentration]. 38
24629889 2014
37
[The X of dyslipemias]. 38
24679041 2014
38
Influence of distal ileum exclusion on hepatic and renal functions in presence of extrahepatic cholestasis. 38
24918724 2014
39
Vitamin A supplementation alleviates extrahepatic cholestasis liver injury through Nrf2 activation. 38
25126202 2014
40
Deleterious effect of oltipraz on extrahepatic cholestasis in bile duct-ligated mice. 38
23978715 2014
41
Safety and pharmacology of gemcitabine and capecitabine in patients with advanced pancreatico-biliary cancer and hepatic dysfunction. 38
24166106 2014
42
[Mirizzi Syndrome: Prevalence, diagnosis and treatment]. 38
26753384 2014
43
Hepatotoxicity Associated with Herbal Tea Containing Kelp. 38
26157821 2013
44
[A quite usual pancreatitis?]. 38
24002875 2013
45
Intrathoracic major duodenal papilla with transhiatal herniation of the pancreas and duodenum: A case report and review of the literature. 38
23805366 2013
46
[Vitamin K deficiency bleeding: a case secondary to transient neonatal cholestasis]. 38
23566582 2013
47
[An infrequent cause of extrahepatic cholestasis]. 38
23650825 2013
48
Hepatoprotection in bile duct ligated mice mediated by darbepoetin-α is not caused by changes in hepatobiliary transporter expression. 38
23236546 2013
49
Loss of A(1) adenosine receptor attenuates alpha-naphthylisothiocyanate-induced cholestatic liver injury in mice. 38
22956627 2013
50
Mitofusin 2 protects hepatocyte mitochondrial function from damage induced by GCDCA. 38
23755235 2013

Variations for Extrahepatic Cholestasis

Expression for Extrahepatic Cholestasis

Search GEO for disease gene expression data for Extrahepatic Cholestasis.

Pathways for Extrahepatic Cholestasis

Pathways related to Extrahepatic Cholestasis according to GeneCards Suite gene sharing:

(show all 18)
# Super pathways Score Top Affiliating Genes
1 12.23 ABCC3 ABCC2 ABCC1
2 11.81 GGT1 ABCC3 ABCC2
3
Show member pathways
11.79 ABCC3 ABCC2 ABCC1
4 11.47 CYP7A1 ABCC3 ABCC2
5
Show member pathways
11.38 MPO ABCC2
6
Show member pathways
11.16 ABCC2 ABCC1
7 11.12 CYP7A1 ABCC3 ABCC2
8 11.05 ABCC3 ABCC2
9 11.04 ABCC3 ABCC2 ABCC1
10 11 MPO ABCC2
11 10.89 ABCC3 ABCC2
12 10.87 ABCC2 ABCC1
13
Show member pathways
10.8 ABCC3 ABCC2 ABCC1
14 10.77 FGF19 CYP7A1
15 10.69 ABCC3 ABCC2 ABCC1
16 10.56 CYP7A1 ABCC3 ABCC2
17 10.31 MPO ABCC3 ABCC1
18 9.8 ABCC3 ABCC2 ABCC1

GO Terms for Extrahepatic Cholestasis

Cellular components related to Extrahepatic Cholestasis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 vacuolar membrane GO:0005774 8.62 ABCC3 ABCC1

Biological processes related to Extrahepatic Cholestasis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 response to lipopolysaccharide GO:0032496 9.33 MPO GGT1 ABCC2
2 drug transmembrane transport GO:0006855 9.26 ABCC2 ABCC1
3 leukotriene metabolic process GO:0006691 8.96 GGT1 ABCC1
4 canalicular bile acid transport GO:0015722 8.62 ABCC3 ABCC2

Molecular functions related to Extrahepatic Cholestasis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 ATPase activity GO:0016887 9.43 ABCC3 ABCC2 ABCC1
2 transmembrane transporter activity GO:0022857 9.33 ABCC3 ABCC2 ABCC1
3 organic anion transmembrane transporter activity GO:0008514 8.96 ABCC3 ABCC2
4 ATPase activity, coupled to transmembrane movement of substances GO:0042626 8.8 ABCC3 ABCC2 ABCC1

Sources for Extrahepatic Cholestasis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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