Cholangitis, Primary Sclerosing malady
Categories: Rare diseases, Liver diseases, Gastrointestinal diseases
Aliases & Descriptions for Cholangitis, Primary Sclerosing:
Orphanet epidemiological data:53
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
Global: Rare diseases
Anatomical: Liver diseases, Gastrointestinal diseases
ICD10: 30 29
Rare hepatic diseases
NIH Rare Diseases:47 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 secondary sclerosing cholangitis. An important gene associated with Cholangitis, Primary Sclerosing is PSC (Cholangitis, Primary Sclerosing). Affiliated tissues include liver, thyroid and spleen.
Disease Ontology:11 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:51 Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease characterized by... (613806) more...
Wikipedia:70 Primary sclerosing cholangitis (PSC) is a disease of the bile ducts that causes inflammation and... more...
Diseases in the Sclerosing Cholangitis family:
Diseases related to Cholangitis, Primary Sclerosing via text searches within MalaCards or GeneCards Suite gene sharing:(show all 11)
Graphical network of diseases related to Cholangitis, Primary Sclerosing:
Clinical features from OMIM:613806
Drugs for Cholangitis, Primary Sclerosing (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):(show top 50) (show all 80)
Interventional clinical trials:(show top 50) (show all 72)
Search NIH Clinical Center for Cholangitis, Primary Sclerosing
MalaCards organs/tissues related to Cholangitis, Primary Sclerosing:35
Liver, Thyroid, Spleen, Small intestine, T cells
Articles related to Cholangitis, Primary Sclerosing:
Search GEO for disease gene expression data for Cholangitis, Primary Sclerosing.
30ICD10 via Orphanet
39MESH via Orphanet
52OMIM via Orphanet
62SNOMED-CT via Orphanet
66Tumor Gene Family of Databases
68UMLS via Orphanet