MCID: EXT006
MIFTS: 39

Extrahepatic Cholestasis

Categories: Gastrointestinal diseases

Aliases & Classifications for Extrahepatic Cholestasis

MalaCards integrated aliases for Extrahepatic Cholestasis:

Name: Extrahepatic Cholestasis 12 15 17
Cholestasis, Extrahepatic 43 71
Extrahepatic Obstructive Biliary Disease 12
Extrahepatic Biliary Stasis 12
Cholestasis Extrahepatic 54

Classifications:



External Ids:

Disease Ontology 12 DOID:13619
MeSH 43 D001651
NCIt 49 C34420
SNOMED-CT 67 20719006
UMLS 71 C0005398

Summaries for Extrahepatic Cholestasis

MalaCards based summary : Extrahepatic Cholestasis, also known as cholestasis, extrahepatic, is related to obstructive jaundice and choledocholithiasis, and has symptoms including icterus An important gene associated with Extrahepatic Cholestasis is GGT1 (Gamma-Glutamyltransferase 1), and among its related pathways/superpathways are Metabolism and Synthesis of bile acids and bile salts. 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 liver/biliary system

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 105)
# Related Disease Score Top Affiliating Genes
1 obstructive jaundice 30.7 GPT GGT1 ALPP ABCC2
2 choledocholithiasis 30.5 GPT GGT1 ALPP ABCB4
3 bile duct cysts 30.4 GPT GGT1 ABCB4
4 cholelithiasis 29.8 NR1H4 GPT GGT1 CYP7A1 ABCB4
5 biliary atresia 29.1 SLC10A1 NR1H4 GPT GGT1 ABCB4 ABCB11
6 cholangitis 28.9 NR1H4 MPO GPT GGT1 ALPP ABCB4
7 liver disease 28.4 SLC10A1 NR1H4 GPT GGT1 ALPP ABCC2
8 cholestasis 28.0 TFAM SLC51B SLC51A SLC10A1 NR1H4 GPT
9 biliary tract disease 26.2 SLC51A SLC10A1 NR1H4 GPT GGT1 CYP8B1
10 alkaline phosphatase, plasma level of, quantitative trait locus 1 10.3 GGT1 ALPP
11 viral laryngitis 10.3 GPT GGT1
12 acute cholangitis 10.3 GPT GGT1
13 fatty liver disease, nonalcoholic 1 10.3 GPT GGT1
14 primary hepatic neuroendocrine carcinoma 10.3 GPT ALPP
15 fibrosis of extraocular muscles, congenital, 1 10.3
16 granulomatous hepatitis 10.3 GPT ALPP
17 liver cirrhosis 10.2
18 hepatic tuberculosis 10.2 GPT GGT1 ALPP
19 protein-energy malnutrition 10.2 MPO GPT GGT1
20 fascioliasis 10.2 GPT GGT1 ALPP
21 cholangiocarcinoma 10.2
22 cholecystitis 10.2
23 intrahepatic cholangiocarcinoma 10.2
24 pancreatitis 10.2
25 suppurative cholangitis 10.2 GPT GGT1 ABCB4
26 common bile duct disease 10.2 GPT GGT1 ABCB4
27 atp8b1 deficiency 10.1 NR1H4 ABCB11
28 colchicine resistance 10.1 ABCC1 ABCB11
29 immune deficiency disease 10.1
30 pancreatic cancer 10.1
31 alcoholic hepatitis 10.1
32 viral hepatitis 10.1
33 gallbladder cancer 10.1
34 acquired immunodeficiency syndrome 10.1
35 hepatic vascular disease 10.1 NR1H4 GPT GGT1
36 hyperthyroidism 10.1 GPT CYP7A1 ALPP
37 cholestasis, intrahepatic, of pregnancy 3 10.0 ABCB4 ABCB11
38 hypercholesterolemia, familial, 1 10.0 NR1H4 GPT CYP7A1
39 cholangiolocellular carcinoma 10.0 ABCC3 ABCC1
40 outlet dysfunction constipation 10.0 FGFR4 FGF19 CYP7A1
41 non-alcoholic steatohepatitis 10.0 NR1H4 GPT GGT1
42 sitosterolemia 10.0 CYP7A1 ABCB4 ABCB11
43 choline deficiency disease 10.0 NR1H4 GPT
44 parenteral nutrition-associated cholestasis 10.0 GPT GGT1 ALPP ABCB4
45 alcohol dependence 9.9
46 autoimmune disease 9.9
47 hepatocellular carcinoma 9.9
48 kaposi sarcoma 9.9
49 neurofibromatosis, type i 9.9
50 myeloma, multiple 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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.83 ABCB11 ABCB4 ABCC1 ABCC2 ABCC3 CYP7A1
2 liver/biliary system MP:0005370 9.17 ABCB11 ABCB4 ABCC2 ABCC3 CYP7A1 CYP8B1

Drugs & Therapeutics for Extrahepatic Cholestasis

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

(show all 15)
# 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
Aspirin Approved, Vet_approved 50-78-2 2244
4 Antibodies
5 Immunoglobulins
6 Liver Extracts
7 Fibrinolytic Agents
8 Analgesics, Non-Narcotic
9 Cyclooxygenase Inhibitors
10 Analgesics
11 Platelet Aggregation Inhibitors
12 Antipyretics
13 Anti-Inflammatory Agents
14 Antirheumatic Agents
15 Anti-Inflammatory Agents, Non-Steroidal

Interventional clinical trials:

(show all 16)
# 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 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 Completed NCT03708445
8 Ultrasound-guided Percutaneous Biliary Drainage With Primary Metal Implantation by Endoscopic Luminal Guidance in Patients With Malignant Extrahepatic Bile Duct Obstruction Completed NCT03541590
9 Randomized Controlled Trial of Short Versus Long Wire and Small Versus Standard Sphincterotomes for Initial Biliary Cannulation Completed NCT02197338
10 Prospective Comparison of Transient Elastography and Mini-laparoscopic Guided Liver Biopsy in Autoimmune Hepatitis Completed NCT01905254
11 Treatment of Malignant Distal Biliary Obstruction - a Randomized Study Recruiting NCT03821025
12 A Prospective Study: Magnetic Compression Anastomosis for Recanalization of Biliary Stricture Recruiting NCT04170933
13 The Effect of Aspirin on Patency of Metal Stent in Malignant Distal Bile Duct Obstruction Recruiting NCT03279809 Aspirin;Placebo
14 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
15 Volumetric Laser Endomicroscopy's (VLE) Diagnostic Accuracy Validation Study: Impact on Clinical Management Study Enrolling by invitation NCT03951324
16 Partially Covered Metal Stents Efficacy in the Biliary Drainage of Malignant Extra-hepatic Biliary Obstruction, a Multicentric Randomized Trial Not yet recruiting NCT04431427

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:

40
Liver, Testes, Pancreas, Brain, Kidney, T Cells, Small Intestine

Publications for Extrahepatic Cholestasis

Articles related to Extrahepatic Cholestasis:

(show top 50) (show all 565)
# 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. 61 54
19185005 2009
2
Gene expression of ABC proteins in hepatocellular carcinoma, perineoplastic tissue, and liver diseases. 61 54
12428063 2002
3
Hepatic secretion of conjugated drugs and endogenous substances. 54 61
11076395 2000
4
Lipoprotein-X in patients with cirrhosis: its relationship to cholestasis and hypercholesterolemia. 61 54
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]. 54 61
9208002 1997
6
Increased intercellular adhesion molecule-1 serum concentration in cholestasis. 61 54
9007716 1996
7
Serum gamma-glutamyl transpeptidase in chronic extrahepatic cholestasis. 54 61
1983169 1990
8
Novel insights into the organic solute transporter alpha/beta, OSTα/β: From the bench to the bedside. 61
32247663 2020
9
Distribution of the organic anion transporters Oat1 and Oat3 between renal membrane microdomains in obstructive jaundice. 61
32451678 2020
10
Robustness testing and optimization of an adverse outcome pathway on cholestatic liver injury. 61
32152650 2020
11
Bile Modulates Secretion of Incretins and Insulin: A Study of Human Extrahepatic Cholestasis. 61
30874733 2019
12
Persistent cholestasis resulting from duodenal papillary carcinoma in an adolescent male: A case report. 61
31145285 2019
13
Exogenous putrescine affects polyamine and arginine metabolism in rat liver following bile ductus ligation. 61
30193084 2018
14
Retrospective analysis of cases with an ectopic opening of the common bile duct into duodenal bulb. 61
30062869 2018
15
Acute Isolated Hyperbilirubinemia as a Presentation of Alcoholic Liver Disease: A Case Report and Literature Review. 61
29623226 2018
16
Prostaglandin E1 Preconditioning Attenuates Liver Ischemia Reperfusion Injury in a Rat Model of Extrahepatic Cholestasis. 61
29511679 2018
17
Protective effect of dehydroandrographolide on obstructive cholestasis in bile duct-ligated mice. 61
29152129 2017
18
Hepatoprotective activities of rosmarinic acid against extrahepatic cholestasis in rats. 61
28789951 2017
19
Adipose-derived mesenchymal stem cells slow disease progression of acute-on-chronic liver failure. 61
28501004 2017
20
Atorvastatin does not protect against ischemia-reperfusion damage in cholestatic rat livers. 61
28399849 2017
21
Use of 4-Fr versus 6-Fr Nasobiliary Catheter for Biliary Drainage: A Prospective, Multicenter, Randomized, Controlled Study. 61
28503061 2017
22
[Elevated liver enzymes]. 61
27824421 2016
23
Lipid profiling of lipoprotein X: Implications for dyslipidemia in cholestasis. 61
27112638 2016
24
Role of farnesoid X receptor in cholestasis. 61
27383832 2016
25
Qualification for endoscopic retrograde cholangiopancreatography in the diagnosis and treatment of extrahepatic cholestasis caused by choledocholithiasis. 61
26788082 2015
26
[CHOLESTASIS AND INFLAMMATION OF THE PANCREAS IN FAMILY MEDICINE]. 61
29083844 2015
27
Neonatal McCune-Albright syndrome with systemic involvement: a case report. 61
26341786 2015
28
Antioxidant Effect of Sepia Ink Extract on Extrahepatic Cholestasis Induced by Bile Duct Ligation in Rats. 61
26383596 2015
29
Expression of renal Oat5 and NaDC1 transporters in rats with acute biliary obstruction. 61
26269671 2015
30
Activation of the renin-angiotensin system stimulates biliary hyperplasia during cholestasis induced by extrahepatic bile duct ligation. 61
25678505 2015
31
Bile acid-induced necrosis in primary human hepatocytes and in patients with obstructive cholestasis. 61
25636263 2015
32
Efficacy of diffusion-weighted magnetic resonance imaging in the evaluation of extrahepatic cholestasis-related hepatic fibrosis. 61
26281340 2015
33
Biochemical profile of bile fluid in patients with malignant cholestasis in comparison with cholestasis due to gall stone. 61
26644885 2015
34
Factors That Could Impact on Liver Fibrosis Staging by Transient Elastography. 61
26770833 2015
35
SIRT1/PGC-1α signaling protects hepatocytes against mitochondrial oxidative stress induced by bile acids. 61
25789761 2015
36
γ-Glutamyl transpeptidase level as a screening marker among diverse etiologies of infantile intrahepatic cholestasis. 61
25141230 2014
37
Increase of glycosaminoglycans and metalloproteinases 2 and 9 in liver extracellular matrix on early stages of extrahepatic cholestasis. 61
25591159 2014
38
The canine hepatic progenitor cell niche: molecular characterisation in health and disease. 61
24923752 2014
39
[Effect of biliary obstruction on lipoprotein(a) concentration]. 61
24629889 2014
40
[The X of dyslipemias]. 61
24679041 2014
41
Influence of distal ileum exclusion on hepatic and renal functions in presence of extrahepatic cholestasis. 61
24918724 2014
42
Deleterious effect of oltipraz on extrahepatic cholestasis in bile duct-ligated mice. 61
23978715 2014
43
Vitamin A supplementation alleviates extrahepatic cholestasis liver injury through Nrf2 activation. 61
25126202 2014
44
Safety and pharmacology of gemcitabine and capecitabine in patients with advanced pancreatico-biliary cancer and hepatic dysfunction. 61
24166106 2014
45
[Mirizzi Syndrome: Prevalence, diagnosis and treatment]. 61
26753384 2014
46
Hepatotoxicity Associated with Herbal Tea Containing Kelp. 61
26157821 2013
47
[A quite usual pancreatitis?]. 61
24002875 2013
48
Intrathoracic major duodenal papilla with transhiatal herniation of the pancreas and duodenum: A case report and review of the literature. 61
23805366 2013
49
[Vitamin K deficiency bleeding: a case secondary to transient neonatal cholestasis]. 61
23566582 2013
50
[An infrequent cause of extrahepatic cholestasis]. 61
23650825 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
Show member pathways
13.92 SLC10A1 NR1H4 GPT GGT1 CYP8B1 CYP7A1
2
Show member pathways
12.04 SLC10A1 NR1H4 CYP8B1 CYP7A1 ABCC3 ABCB11
3 11.91 GGT1 ABCC3 ABCC2
4
Show member pathways
11.9 ABCC3 ABCC2 ABCC1
5
Show member pathways
11.9 SLC10A1 ABCC2 ABCB4 ABCB11
6
Show member pathways
11.77 ABCC3 ABCC2 ABCB11
7 11.34 NR1H4 CYP8B1 CYP7A1 ABCC3 ABCC2 ABCB4
8 11.23 SLC51B SLC51A SLC10A1 NR1H4 CYP7A1 ABCC3
9 11.16 ABCC3 ABCC2 ABCC1
10 10.98 SLC10A1 NR1H4 FGF19 CYP8B1 CYP7A1 ABCB4
11
Show member pathways
10.95 ABCC3 ABCC2 ABCC1
12 10.92 ABCC3 ABCC2
13 10.91 ABCC2 ABCC1
14 10.89 ABCC3 ABCC2 ABCC1
15 10.79 ABCC3 ABCC2 ABCC1
16 10.79 SLC51B SLC51A SLC10A1 NR1H4 CYP7A1 ABCC3
17 10.76 MPO ABCC3 ABCC1
18 10.54 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 integral component of plasma membrane GO:0005887 9.8 SLC10A1 FGFR4 ABCC3 ABCC2 ABCC1 ABCB4
2 integral component of membrane GO:0016021 9.77 SLC51B SLC51A SLC10A1 GGT1 FGFR4 CYP8B1
3 basolateral plasma membrane GO:0016323 9.46 SLC51B SLC51A SLC10A1 ABCC1
4 intercellular canaliculus GO:0046581 8.8 ABCC2 ABCB4 ABCB11

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.76 SLC51B SLC51A SLC10A1 ABCC3 ABCC2 ABCC1
2 cholesterol homeostasis GO:0042632 9.7 NR1H4 FGFR4 CYP7A1
3 regulation of lipid metabolic process GO:0019216 9.69 FGFR4 CYP7A1 ABCB4
4 bile acid biosynthetic process GO:0006699 9.58 CYP8B1 CYP7A1 ABCB11
5 organic anion transport GO:0015711 9.55 ABCC3 ABCC2
6 leukotriene metabolic process GO:0006691 9.54 GGT1 ABCC1
7 bile acid secretion GO:0032782 9.5 SLC51B SLC51A ABCB4
8 xenobiotic transport GO:0042908 9.49 ABCC3 ABCC1
9 transepithelial transport GO:0070633 9.48 ABCC2 ABCC1
10 organic substance transport GO:0071702 9.46 SLC51B SLC51A
11 cellular response to bile acid GO:1903413 9.43 NR1H4 ABCB4
12 regulation of bile acid biosynthetic process GO:0070857 9.43 NR1H4 FGFR4 CYP7A1
13 leukotriene transport GO:0071716 9.37 ABCC2 ABCC1
14 bile acid and bile salt transport GO:0015721 9.17 SLC51B SLC51A SLC10A1 NR1H4 ABCC3 ABCC2
15 canalicular bile acid transport GO:0015722 9.13 ABCC3 ABCC2 ABCB11

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

# Name GO ID Score Top Affiliating Genes
1 ATPase activity GO:0016887 9.77 ABCC3 ABCC2 ABCC1 ABCB4 ABCB11
2 transmembrane transporter activity GO:0022857 9.72 SLC51B SLC51A ABCC3 ABCC2 ABCC1
3 heme binding GO:0020037 9.65 MPO CYP8B1 CYP7A1
4 transporter activity GO:0005215 9.61 SLC51B SLC51A ABCB11
5 xenobiotic transmembrane transporting ATPase activity GO:0008559 9.46 ABCC2 ABCC1
6 xenobiotic transmembrane transporter activity GO:0042910 9.43 ABCC3 ABCC2 ABCC1
7 bile acid-exporting ATPase activity GO:0015432 9.4 ABCC3 ABCB11
8 bile acid transmembrane transporter activity GO:0015125 9.33 SLC51B SLC51A SLC10A1
9 organic anion transmembrane transporter activity GO:0008514 9.13 ABCC3 ABCC2 ABCC1
10 ATPase activity, coupled to transmembrane movement of substances GO:0042626 9.02 ABCC3 ABCC2 ABCC1 ABCB4 ABCB11

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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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