PSC
MCID: CHL119
MIFTS: 44

Cholangitis, Primary Sclerosing (PSC) malady

Categories: Rare diseases, Liver diseases, Gastrointestinal diseases

Aliases & Classifications for Cholangitis, Primary Sclerosing

Aliases & Descriptions for Cholangitis, Primary Sclerosing:

Name: Cholangitis, Primary Sclerosing 54 50 13
Primary Sclerosing Cholangitis 12 50 25 56 14 69
Sclerosing Cholangitis 50 25
Psc 25 56
Cholangitis Primary Sclerosing 52
Cholangitis, Sclerosing 69

Characteristics:

Orphanet epidemiological data:

56
primary sclerosing cholangitis
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (Europe),1-5/10000 (Europe),1-9/100000 (Worldwide),1-9/1000000 (Worldwide),1-9/100000 (Norway),1-9/100000 (Sweden),1-5/10000 (Sweden),1-9/1000000 (Netherlands),1-9/100000 (Netherlands),1-9/1000000 (Canada),<1/1000000 (Spain),1-9/1000000 (Spain); Age of onset: Adult;

Classifications:

Orphanet: 56  
Rare hepatic diseases


External Ids:

OMIM 54 613806
Disease Ontology 12 DOID:0060643
ICD10 33 K83.0
Orphanet 56 ORPHA171
ICD10 via Orphanet 34 K83.0
MESH via Orphanet 43 C536419
UMLS via Orphanet 70 C0566602
UMLS 69 C0566602

Summaries for Cholangitis, Primary Sclerosing

NIH Rare Diseases : 50 primary sclerosing cholangitis (psc) is characterized by inflammation in the bile ducts (cholangitis) that leads to scarring (sclerosis), narrowing of the ducts, and a buildup of bile in the liver. early signs and symptoms include extreme tiredness, abdominal pain, and itchiness. as the condition worsens it may cause jaundice, an enlarged spleen, and eventually liver cirrhosis and failure. other complications may include weight loss, vitamin deficiency, and osteoporosis. many people with psc develop other autoimmune conditions such as inflammatory bowel disease, type 1 diabetes, celiac disease, or thyroid disease. psc is also a risk factor for cancer of the bile ducts (cholangiocarcinoma). last updated: 3/4/2013

MalaCards based summary : Cholangitis, Primary Sclerosing, also known as primary sclerosing cholangitis, is related to ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis and sclerosing cholangitis, and has symptoms including pruritus, fatigue and fever. An important gene associated with Cholangitis, Primary Sclerosing is PSC (Cholangitis, Primary Sclerosing), and among its related pathways/superpathways is Defensins. The drugs Vancomycin and Ceftriaxone have been mentioned in the context of this disorder. Affiliated tissues include liver, thyroid and spleen, and related phenotypes are Increased shRNA abundance (Z-score > 2) and hematopoietic system

Disease Ontology : 12 A sclerosing cholangitis characterized by fibroobliterative inflammation of the biliary tract, leading to cirrhosis and portal hypertension.

Genetics Home Reference : 25 Primary sclerosing cholangitis is a condition that affects the bile ducts. These ducts carry bile (a fluid that helps to digest fats) from the liver, where bile is produced, to the gallbladder, where it is stored, and to the small intestine, where it aids in digestion. Primary sclerosing cholangitis occurs because of inflammation in the bile ducts (cholangitis) that leads to scarring (sclerosis) and narrowing of the ducts. As a result, bile cannot be released to the gallbladder and small intestine, and it builds up in the liver.

OMIM : 54 Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease characterized by... (613806) more...

Wikipedia : 71 Primary sclerosing cholangitis (PSC) is a disease of the bile ducts that causes inflammation and... more...

Related Diseases for Cholangitis, Primary Sclerosing

Diseases in the Sclerosing Cholangitis family:

Cholangitis, Primary Sclerosing Secondary Sclerosing Cholangitis

Diseases related to Cholangitis, Primary Sclerosing via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 24)
id Related Disease Score Top Affiliating Genes
1 ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis 12.4
2 sclerosing cholangitis 10.5
3 cholangitis 10.5
4 bickerstaff brainstem encephalitis 10.2 CFTR HLA-A HLA-C
5 benign secondary hypertension 10.2 BPI CTSG MPO
6 campylobacteriosis 10.2 GGT1 GPT MPO
7 fasciitis 10.2 BPI CFTR CTSG MPO
8 pericholangitis 10.2 ABCB4 DLAT MPO
9 ringed hair 10.2 GGT1 GPT
10 b-cell lymphomas 10.2 DLAT GGT1 GPT HLA-A
11 epididymo-orchitis 10.2 HLA-A HLA-C
12 gastrointestinal adenoma 10.2 ABCB4 GGT1 GPT
13 eating disorder 10.2 BPI CTSG MPO PRTN3
14 major depressive disorder and accelerated response to antidepressant drug treatment 10.1 BPI CTSG MPO PRTN3
15 eclampsia 10.1 ABCB4 GGT1 GPT
16 subcorneal pustular dermatosis 10.1 CFTR CTSG MPO PRTN3
17 childhood malignant hemangiopericytoma 10.1 GGT1 LTF MADCAM1 MPO
18 lymphoid leukemia 10.1 ABCB4 DLAT GGT1 GPT
19 acute cholangitis 10.1 ABCB4 BPI CFTR CTSG HLA-C MST1
20 hyperlysinemia 10.1 BPI CFTR CTSG MPO PRTN3
21 bacterial meningitis 10.0 ABCB4 CFTR DLAT GGT1 GPT MPO
22 pseudoarthrosis 10.0 ABCB4 DLAT GGT1
23 primary biliary cholangitis 9.7
24 microcephaly, progressive, seizures, and cerebral and cerebellar atrophy 9.0 ABCB4 BPI CFTR CTSG DLAT DMBT1

Graphical network of the top 20 diseases related to Cholangitis, Primary Sclerosing:



Diseases related to Cholangitis, Primary Sclerosing

Symptoms & Phenotypes for Cholangitis, Primary Sclerosing

Clinical features from OMIM:

613806

Human phenotypes related to Cholangitis, Primary Sclerosing:

56 (show top 50) (show all 55)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 pruritus 56 Occasional (29-5%)
2 fatigue 56 Occasional (29-5%)
3 fever 56 Frequent (79-30%)
4 abdominal pain 56 Occasional (29-5%)
5 hepatosplenomegaly 56 Frequent (79-30%)
6 depression 56 Occasional (29-5%)
7 osteopenia 56 Occasional (29-5%)
8 splenomegaly 56 Frequent (79-30%)
9 hepatomegaly 56 Frequent (79-30%)
10 renal insufficiency 56 Occasional (29-5%)
11 portal hypertension 56 Frequent (79-30%)
12 type i diabetes mellitus 56 Occasional (29-5%)
13 osteoporosis 56 Occasional (29-5%)
14 ascites 56 Frequent (79-30%)
15 hepatitis 56 Occasional (29-5%)
16 weight loss 56 Frequent (79-30%)
17 congestive heart failure 56 Occasional (29-5%)
18 elevated hepatic transaminases 56 Frequent (79-30%)
19 hepatic fibrosis 56 Frequent (79-30%)
20 celiac disease 56 Occasional (29-5%)
21 autoimmunity 56 Very frequent (99-80%)
22 thyroiditis 56 Occasional (29-5%)
23 cirrhosis 56 Frequent (79-30%)
24 cholelithiasis 56 Occasional (29-5%)
25 jaundice 56 Occasional (29-5%)
26 pancreatitis 56 Occasional (29-5%)
27 cholestasis 56 Very frequent (99-80%)
28 vitamin d deficiency 56 Occasional (29-5%)
29 acute hepatic failure 56 Very rare (<4-1%)
30 abnormality of eosinophils 56 Excluded (0%)
31 encephalopathy 56 Very rare (<4-1%)
32 uveitis 56 Very rare (<4-1%)
33 pleural effusion 56 Occasional (29-5%)
34 prolonged prothrombin time 56 Occasional (29-5%)
35 abnormal biliary tract morphology 56 Very frequent (99-80%)
36 elevated alkaline phosphatase of hepatic origin 56 Frequent (79-30%)
37 spider hemangioma 56 Frequent (79-30%)
38 abnormal large intestine physiology 56 Frequent (79-30%)
39 dilated superficial abdominal veins 56 Frequent (79-30%)
40 ulcerative colitis 56 Frequent (79-30%)
41 palmar telangiectasia 56 Frequent (79-30%)
42 hepatocellular carcinoma 56 Occasional (29-5%)
43 hypoalbuminemia 56 Occasional (29-5%)
44 polyclonal elevation of igm 56 Occasional (29-5%)
45 generalized amyotrophy 56 Occasional (29-5%)
46 vitamin a deficiency 56 Occasional (29-5%)
47 vitamin k deficiency 56 Occasional (29-5%)
48 cholangiocarcinoma 56 Occasional (29-5%)
49 adenocarcinoma of the large intestine 56 Occasional (29-5%)
50 vitamin e deficiency 56 Occasional (29-5%)

UMLS symptoms related to Cholangitis, Primary Sclerosing:


icterus

GenomeRNAi Phenotypes related to Cholangitis, Primary Sclerosing according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 10.36 MPO
2 Increased shRNA abundance (Z-score > 2) GR00366-A-101 10.36 TCF4
3 Increased shRNA abundance (Z-score > 2) GR00366-A-103 10.36 HLA-C GPR35 HLA-A CTSG
4 Increased shRNA abundance (Z-score > 2) GR00366-A-105 10.36 GPR35
5 Increased shRNA abundance (Z-score > 2) GR00366-A-106 10.36 DLAT
6 Increased shRNA abundance (Z-score > 2) GR00366-A-110 10.36 HLA-A HLA-C
7 Increased shRNA abundance (Z-score > 2) GR00366-A-115 10.36 DLAT
8 Increased shRNA abundance (Z-score > 2) GR00366-A-118 10.36 HLA-C HLA-A
9 Increased shRNA abundance (Z-score > 2) GR00366-A-122 10.36 TCF4
10 Increased shRNA abundance (Z-score > 2) GR00366-A-124 10.36 HLA-C
11 Increased shRNA abundance (Z-score > 2) GR00366-A-126 10.36 MPO
12 Increased shRNA abundance (Z-score > 2) GR00366-A-129 10.36 GPR35
13 Increased shRNA abundance (Z-score > 2) GR00366-A-13 10.36 CTSG
14 Increased shRNA abundance (Z-score > 2) GR00366-A-130 10.36 GGT1 PRTN3
15 Increased shRNA abundance (Z-score > 2) GR00366-A-131 10.36 GPR35
16 Increased shRNA abundance (Z-score > 2) GR00366-A-133 10.36 PRTN3
17 Increased shRNA abundance (Z-score > 2) GR00366-A-143 10.36 GPR35 GGT1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-146 10.36 DLAT KRT19
19 Increased shRNA abundance (Z-score > 2) GR00366-A-147 10.36 TCF4
20 Increased shRNA abundance (Z-score > 2) GR00366-A-149 10.36 GPR35 HLA-C CTSG
21 Increased shRNA abundance (Z-score > 2) GR00366-A-152 10.36 PRTN3
22 Increased shRNA abundance (Z-score > 2) GR00366-A-156 10.36 HLA-A HLA-C
23 Increased shRNA abundance (Z-score > 2) GR00366-A-157 10.36 MPO
24 Increased shRNA abundance (Z-score > 2) GR00366-A-161 10.36 CTSG
25 Increased shRNA abundance (Z-score > 2) GR00366-A-162 10.36 HLA-C
26 Increased shRNA abundance (Z-score > 2) GR00366-A-163 10.36 DLAT
27 Increased shRNA abundance (Z-score > 2) GR00366-A-166 10.36 DLAT
28 Increased shRNA abundance (Z-score > 2) GR00366-A-169 10.36 GGT1 KRT19
29 Increased shRNA abundance (Z-score > 2) GR00366-A-176 10.36 MPO
30 Increased shRNA abundance (Z-score > 2) GR00366-A-177 10.36 TCF4 KRT19
31 Increased shRNA abundance (Z-score > 2) GR00366-A-178 10.36 HLA-A HLA-C
32 Increased shRNA abundance (Z-score > 2) GR00366-A-186 10.36 MPO
33 Increased shRNA abundance (Z-score > 2) GR00366-A-19 10.36 HLA-A HLA-C
34 Increased shRNA abundance (Z-score > 2) GR00366-A-198 10.36 GPR35
35 Increased shRNA abundance (Z-score > 2) GR00366-A-199 10.36 GPR35
36 Increased shRNA abundance (Z-score > 2) GR00366-A-202 10.36 HLA-C
37 Increased shRNA abundance (Z-score > 2) GR00366-A-21 10.36 CTSG
38 Increased shRNA abundance (Z-score > 2) GR00366-A-22 10.36 TCF4
39 Increased shRNA abundance (Z-score > 2) GR00366-A-29 10.36 MPO
40 Increased shRNA abundance (Z-score > 2) GR00366-A-31 10.36 HLA-A HLA-C
41 Increased shRNA abundance (Z-score > 2) GR00366-A-36 10.36 MPO
42 Increased shRNA abundance (Z-score > 2) GR00366-A-43 10.36 CTSG TCF4 DLAT KRT19 PRTN3
43 Increased shRNA abundance (Z-score > 2) GR00366-A-46 10.36 TCF4
44 Increased shRNA abundance (Z-score > 2) GR00366-A-47 10.36 HLA-C TCF4
45 Increased shRNA abundance (Z-score > 2) GR00366-A-5 10.36 DLAT
46 Increased shRNA abundance (Z-score > 2) GR00366-A-57 10.36 MPO
47 Increased shRNA abundance (Z-score > 2) GR00366-A-7 10.36 HLA-C GGT1 HLA-A MPO
48 Increased shRNA abundance (Z-score > 2) GR00366-A-70 10.36 MPO
49 Increased shRNA abundance (Z-score > 2) GR00366-A-74 10.36 GPR35 HLA-A HLA-C
50 Increased shRNA abundance (Z-score > 2) GR00366-A-76 10.36 TCF4
51 Increased shRNA abundance (Z-score > 2) GR00366-A-81 10.36 DLAT
52 Increased shRNA abundance (Z-score > 2) GR00366-A-82 10.36 PRTN3
53 Increased shRNA abundance (Z-score > 2) GR00366-A-93 10.36 HLA-C
54 Increased shRNA abundance (Z-score > 2) GR00366-A-99 10.36 PRTN3

MGI Mouse Phenotypes related to Cholangitis, Primary Sclerosing:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.65 LTF MPO MST1 PRTN3 TCF4 ABCB4
2 immune system MP:0005387 9.32 ABCB4 CFTR CTSG DMBT1 GGT1 LTF

Drugs & Therapeutics for Cholangitis, Primary Sclerosing

Drugs for Cholangitis, Primary Sclerosing (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 89)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vancomycin Approved Phase 4,Phase 3,Phase 1 1404-90-6 441141 14969
2
Ceftriaxone Approved Phase 4 73384-59-5 5479530 5361919
3
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
4
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
5
Ofloxacin Approved Phase 4 82419-36-1 4583
6
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
7
Levofloxacin Approved, Investigational Phase 4 100986-85-4 149096
8
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
9 Liver Extracts Phase 4,Phase 3,Phase 2,Phase 1
10 Bile Acids and Salts Phase 4,Phase 2,Phase 1
11 glucocorticoids Phase 4,Phase 1
12 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1
13 Hormone Antagonists Phase 4,Phase 1
14 Hormones Phase 4,Phase 1
15 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 1
16 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1
17 Immunoglobulin G Phase 4,Phase 1,Phase 2
18 Immunoglobulins Phase 4,Phase 2,Phase 1
19 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1
20 Immunosuppressive Agents Phase 4,Phase 2,Phase 1
21 Anti-Inflammatory Agents Phase 4,Phase 1,Phase 2
22 Antibiotics, Antitubercular Phase 4,Phase 2
23 Antibodies Phase 4,Phase 2,Phase 1
24 Antirheumatic Agents Phase 4,Phase 1,Phase 2
25 Prednisolone acetate Phase 4
26 Cytochrome P-450 CYP1A2 Inhibitors Phase 4
27 Cytochrome P-450 Enzyme Inhibitors Phase 4
28 Topoisomerase Inhibitors Phase 4
29 Prednisolone hemisuccinate Phase 4
30 Prednisolone phosphate Phase 4
31 Renal Agents Phase 4
32 Methylprednisolone acetate Phase 4
33 Methylprednisolone Hemisuccinate Phase 4
34 Antineoplastic Agents, Hormonal Phase 4
35 Antitubercular Agents Phase 4
36
Ursodeoxycholic acid Approved, Investigational Phase 2, Phase 3,Phase 1 128-13-2 31401
37
Bezafibrate Approved Phase 3 41859-67-0 39042
38 Antimetabolites Phase 3,Phase 1,Phase 2
39 Cholagogues and Choleretics Phase 2, Phase 3,Phase 1
40 Hypolipidemic Agents Phase 3,Phase 1,Phase 2
41 Lipid Regulating Agents Phase 3,Phase 1,Phase 2
42
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
43
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
44
Aldesleukin Approved Phase 2 85898-30-2, 110942-02-4
45
Fenofibrate Approved Phase 1, Phase 2 49562-28-9 3339
46 Nucleic Acid Synthesis Inhibitors Phase 2,Phase 1
47 Peripheral Nervous System Agents Phase 1, Phase 2
48 2-chloro-3'-deoxyadenosine Phase 2
49 Antibodies, Monoclonal Phase 2
50
Curcumin Phase 1, Phase 2 458-37-7 969516

Interventional clinical trials:

(show top 50) (show all 82)
id Name Status NCT ID Phase
1 Effect and Safety of Oral Vancomycin in Primary Sclerosing Cholangitis Patients Recruiting NCT02605213 Phase 4
2 Antibiotics to Decrease Post ERCP Cholangitis Recruiting NCT03087656 Phase 4
3 Leflunomide for Maintenance of Remission in IgG4 Related Disease Recruiting NCT02703194 Phase 4
4 A Pilot Study to Characterize Bile Acid Metabolism and Dysbiosis in Primary Sclerosing Cholangitis Active, not recruiting NCT02464020 Phase 4
5 Short-term Stenting Versus Balloon Dilatation for Dominant Strictures in Primary Sclerosing Cholangitis Unknown status NCT01398917 Phase 3
6 Probiotics in Patients With Primary Sclerosing Cholangitis Unknown status NCT00161148 Phase 3
7 Trial of High-dose Urso in Primary Sclerosing Cholangitis Completed NCT00059202 Phase 2, Phase 3
8 Digital SpyGlass Confirmed Common Bile Duct Stones Clearance Without Fluoroscopy Completed NCT02967926 Phase 3
9 The Effect of Bezafibrate on Cholestatic Itch Recruiting NCT02701166 Phase 3
10 Primary Sclerosing Cholangitis With Oral Vancomycin by the Study of Its Antimicrobial and Immunomodulating Effects Active, not recruiting NCT01802073 Phase 3
11 Efficacy and Safety of Vedolizumab Intravenous (IV) in the Treatment of Primary Sclerosing Cholangitis in Subjects With Underlying Inflammatory Bowel Disease Withdrawn NCT03035058 Phase 3
12 Norursodeoxycholic Acid in the Treatment of Primary Sclerosing Cholangitis Completed NCT01755507 Phase 2
13 Open Label Study to Evaluate Safety and Efficacy of LUM001 in Patients With Primary Sclerosing Cholangitis Completed NCT02061540 Phase 2
14 Simtuzumab (GS-6624) in the Prevention of Progression of Liver Fibrosis in Subjects With Primary Sclerosing Cholangitis (PSC) Completed NCT01672853 Phase 2
15 Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis Completed NCT00004762 Phase 2
16 Rituximab in IgG4-related Disease: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2
17 Mitomycin C Therapy for Patients With Primary Sclerosing Cholangitis Recruiting NCT01688024 Phase 2
18 PERSEUS: Preliminary Efficacy and Safety of Cenicriviroc in Adult Subjects With Primary Sclerosing Cholangitis Recruiting NCT02653625 Phase 2
19 A Trial of BTT1023 in Patients With Primary Sclerosing Cholangitis Recruiting NCT02239211 Phase 2
20 A Study Evaluating the Safety and Efficacy of Curcumin in Patients With Primary Sclerosing Cholangitis Recruiting NCT02978339 Phase 1, Phase 2
21 Safety, Tolerability, and Efficacy of GS-9674 in Adults With Primary Sclerosing Cholangitis Without Cirrhosis Recruiting NCT02943460 Phase 2
22 Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis. Recruiting NCT02424175 Phase 1, Phase 2
23 Induction of Regulatory t Cells by Low Dose il2 in Autoimmune and Inflammatory Diseases Recruiting NCT01988506 Phase 2
24 Obeticholic Acid (OCA) in Primary Sclerosing Cholangitis (PSC) Active, not recruiting NCT02177136 Phase 2
25 Phase 2 Study of NGM282 in Patients With Primary Sclerosing Cholangitis Active, not recruiting NCT02704364 Phase 2
26 A Study of Oral Hymecromone to Treat Adults With Primary Sclerosing Cholangitis Not yet recruiting NCT02780752 Phase 1, Phase 2
27 A Single-arm,Phase IIa,Safety and Efficacy Trial of Selected MSCs in the Treatment of Patients With PSC & AiH Not yet recruiting NCT02997878 Phase 1, Phase 2
28 Thalidomide for the Treatment of Primary Sclerosing Cholangitis (PSC) Terminated NCT00953615 Phase 2
29 Fenofibrate for PSC Terminated NCT01142323 Phase 1, Phase 2
30 The Value of CT Cholangiography in Primary Sclerosing Cholangitis Withdrawn NCT00588458 Phase 2
31 Erlotinib for Chemoprevention in Trisomy 7 Positive Primary Sclerosing Cholangitis (PSC) Completed NCT00955149 Phase 1
32 Gastrointestinal Microbiota in Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Completed NCT01322386 Phase 1
33 Safety and Efficacy Study of Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis Completed NCT01088607 Phase 1
34 Minocycline in Primary Sclerosing Cholangitis (PSC) Completed NCT00630942 Phase 1
35 A Pilot Study of Vancomycin or Metronidazole in Patients With Primary Sclerosing Cholangitis Completed NCT01085760 Phase 1
36 Pilot Study of Budesonide for Patients With Primary Sclerosing Cholangitis Completed NCT00004842 Phase 1
37 Evaluation of DHA for the Treatment of PSC Completed NCT00325013 Phase 1
38 A Pilot Study of Xifaxan to Treat Patients With PSC Completed NCT01695174 Phase 1
39 Combination Therapy With Ursodeoxycholic Acid (UDCA) and All-Trans Retinoic Acid (ATRA) for Treatment of Primary Sclerosing Cholangitis Completed NCT01456468 Phase 1
40 Biliary Excretion of Conjugated Bile Acids in Humans Measured by 11C-cholylsarcosine PET/CT Completed NCT01879735 Phase 1
41 Optical Frequency Domain Imaging (OFDI) for Biliary Stricture Imaging Completed NCT01439620 Phase 1
42 Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Recruiting NCT02137668 Phase 1
43 Genomics of Primary Sclerosing Cholangitis (PSC) Recruiting NCT01161992 Phase 1
44 A Study of HTD1801 in Healthy Subjects Recruiting NCT03099603 Phase 1
45 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1
46 NPD Measurements in PSC Patients Unknown status NCT00476814
47 Telomere Length and Other Parameters of Telomere Dysfunction in PSC and Colitis Unknown status NCT02247622
48 Liver Transplantation for Hilar Cholangiocarcinoma in Association With Neoadjuvant Radio- and Chemo-therapy Unknown status NCT01549795
49 Measurement of Fibrinogen in Patients With Systemic Inflammatory Response Syndrome (SIRS), Sepsis or Chronicle Liver Disease on Intensive Care Units (ICU) Unknown status NCT01169168
50 Sulfation of Bile Acids as a Biomarker for Hepatobiliary Diseases Unknown status NCT01200082

Search NIH Clinical Center for Cholangitis, Primary Sclerosing

Genetic Tests for Cholangitis, Primary Sclerosing

Anatomical Context for Cholangitis, Primary Sclerosing

MalaCards organs/tissues related to Cholangitis, Primary Sclerosing:

39
Liver, Thyroid, Spleen, Small Intestine, Heart

Publications for Cholangitis, Primary Sclerosing

Articles related to Cholangitis, Primary Sclerosing:

id Title Authors Year
1
Primary biliary cholangitis-primary sclerosing cholangitis in an evolving overlap syndrome: A case report. ( 28089354 )
2017

Variations for Cholangitis, Primary Sclerosing

Expression for Cholangitis, Primary Sclerosing

Search GEO for disease gene expression data for Cholangitis, Primary Sclerosing.

Pathways for Cholangitis, Primary Sclerosing

Pathways related to Cholangitis, Primary Sclerosing according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1
Show member pathways
11.29 BPI CTSG LTF PRTN3

GO Terms for Cholangitis, Primary Sclerosing

Cellular components related to Cholangitis, Primary Sclerosing according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 9.73 CFTR CTSG ENO1 HLA-A HLA-C LTF
2 extracellular space GO:0005615 9.65 BPI CTSG DMBT1 ENO1 GGT1 GPT
3 early endosome membrane GO:0031901 9.63 CFTR HLA-A HLA-C
4 secretory granule GO:0030141 9.61 CTSG LTF MPO
5 phagocytic vesicle membrane GO:0030670 9.54 DMBT1 HLA-A HLA-C
6 recycling endosome membrane GO:0055038 9.5 CFTR HLA-A HLA-C
7 extracellular exosome GO:0070062 9.5 ABCB4 BPI CFTR CTSG DMBT1 ENO1
8 azurophil granule lumen GO:0035578 9.46 BPI CTSG MPO PRTN3
9 MHC class I protein complex GO:0042612 9.4 HLA-A HLA-C

Biological processes related to Cholangitis, Primary Sclerosing according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.77 BPI CTSG HLA-A HLA-C MADCAM1
2 neutrophil degranulation GO:0043312 9.63 BPI CTSG HLA-C LTF MPO PRTN3
3 defense response to bacterium GO:0042742 9.62 BPI CTSG LTF MPO
4 negative regulation of growth of symbiont in host GO:0044130 9.4 CTSG MPO
5 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent GO:0002480 9.26 HLA-A HLA-C
6 antimicrobial humoral response GO:0019730 9.26 BPI CTSG LTF PRTN3
7 defense response to Gram-negative bacterium GO:0050829 8.92 BPI CTSG DMBT1 LTF

Molecular functions related to Cholangitis, Primary Sclerosing according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 8.92 CTSG LTF MST1 PRTN3

Sources for Cholangitis, Primary Sclerosing

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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