Cholangitis, Primary Sclerosing malady
Rare diseases, Liver diseases, Gastrointestinal diseases categories
Aliases & Descriptions for Cholangitis, Primary Sclerosing:
MalaCards categories: See all MalaCards categories (disease lists)
Global: Rare diseases
Anatomical: Liver diseases, Gastrointestinal diseases
Rare hepatic diseases
Characteristics (Orphanet epidemiological data):51
primary sclerosing cholangitis:
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (Europe),1-5/10000 (Europe); Age of onset: Adult
NIH Rare Diseases:45 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 cystic fibrosis and cholecystitis. An important gene associated with Cholangitis, Primary Sclerosing is PSC (Cholangitis, Primary Sclerosing). Affiliated tissues include liver, thyroid and small intestine.
Genetics Home Reference:23 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:49 Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease characterized by... (613806) more...
Clinical features from OMIM:613806
Drugs for Cholangitis, Primary Sclerosing (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):(show all 22)
Interventional clinical trials:(show top 50) (show all 58)
Search NIH Clinical Center for Cholangitis, Primary Sclerosing
MalaCards organs/tissues related to Cholangitis, Primary Sclerosing:33
Liver, Thyroid, Small intestine, Spleen
Search GEO for disease gene expression data for Cholangitis, Primary Sclerosing.
Cellular components related to Cholangitis, Primary Sclerosing according to GeneCards Suite gene sharing:
Biological processes related to Cholangitis, Primary Sclerosing according to GeneCards Suite gene sharing:
28ICD10 via Orphanet
37MESH via Orphanet
50OMIM via Orphanet
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
66UMLS via Orphanet