PedAM
Pediatric Disease Annotations & Medicines
| Disease | sclerosing cholangitis |
| Phenotype | C0566602|primary sclerosing cholangitis |
| Sentences | 51 |
| PubMedID- 26308055 | Cholangiopathies, such as primary sclerosing cholangitis (psc), primary biliary cirrhosis (pbc) and drug-induced bile duct damage, may result in cholestasis, which is characterized by the loss of cholangiocytes through necrosis by apoptosis [3]. |
| PubMedID- 24392145 | For example, primary sclerosing cholangitis (psc) has been diagnosed in 3.7% of patients with ulcerative colitis [2] and in 3.4% of those with crohn’s disease [3]. |
| PubMedID- 24765316 | Primary sclerosing cholangitis may represent an extreme alteration of maternal-fetal bile salt metabolism as in obstetric cholestasis. |
| PubMedID- 25646372 | We analysed patients with igg4-rd, patients with primary sclerosing cholangitis (psc) and elevated igg4 (a subset of patients with psc who have an elevated serum igg4 level but no histological or radiological evidence of igg4-rd), and healthy controls in a uk cohort. |
| PubMedID- 22087299 | [44] reported a higher recurrence rate of primary sclerosing cholangitis in patients undergoing ldlt. |
| PubMedID- 22087157 | Liver disorders such as autoimmune hepatitis, elevation of liver enzyme levels, primary biliary cirrhosis, nonspecific hepatitis, primary sclerosing cholangitis, and nonalcoholic fatty liver disease have been reported in patients with cd. |
| PubMedID- 26557943 | There are several established risk factors for cc, such as primary sclerosing cholangitis (psc), which is the commonest known predisposing factor for cc in the western world [5]. |
| PubMedID- 24552185 | Few cases have reported an association of polymyositis with cd, although the occurrence of polymyositis, alopecia universalis, and primary sclerosing cholangitis was observed in a male cd patient in germany [8], and hall documented a case of focal myositis involving the left gastrocnemius muscle [9]. |
| PubMedID- 23189226 | The progression of primary sclerosing cholangitis (psc), a chronic cholestatic syndrome characterized by fibrosing inflammation in the intra- and extra-hepatic bile ducts, appears to bear no relation to the presence or absence of the colon or to the degree of the inflammatory process within the diseased mucosa. |
| PubMedID- 22695995 | 10a coronal mip reformat shows early primary sclerosing cholangitis (psc) with irregular dilatation and strictures seen in the left sided intrahepatic ducts (arrows). |
| PubMedID- 26045700 | Primary sclerosing cholangitis (psc) is a chronic liver disease characterized by cholestasis, biliary inflammation and duct stricture that will lead to cirrhosis in approximately half of subjects (1-5). |
| PubMedID- 21286351 | It needs to be differentiated from primary sclerosing cholangitis (psc) and cholangiocarcinoma, as it results in completely different therapeutic strategy. |
| PubMedID- 24495412 | Primary sclerosing cholangitis (psc) can cause benign biliary stricturing and is the most common risk factor for cca in western populations, with rates of 8-30% reported in clinical follow-up or liver explant specimens after transplant. |
| PubMedID- 26297361 | This is supported by mrs studies which have shown that mitochondrial dysfunction is directly related to antimitochondrial antibody (ama) levels.14 unpublished data from the newcastle group have demonstrated significantly lower anaerobic threshold (at) values compared with patients with age-matched and sex-matched primary sclerosing cholangitis (psc) and sedentary controls, again supporting a muscle bioenergetic abnormality contributing to the fatigue seen in pbc. |
| PubMedID- PMC4070603 | Concomitant primary sclerosing cholangitis (psc) and disease extent are the only factors that influence the start and periodicity of colonoscopy surveillance in these patients. |
| PubMedID- 23408245 | Bile duct diseases (primary sclerosing cholangitis, primary biliary cirrhosis, choledochal cysts, choledocholithiasis, cholecystitis, and liver flukes), primarily affecting large intrahepatic bile ducts and/or extrahepatic bile ducts, contribute to both cc forms though there are large differences in odds ratios. |
| PubMedID- 21994888 | Primary biliary cirrhosis (pbc), primary sclerosing cholangitis (psc), and hepatolithiasis in adults and biliary atresia and choledochal cyst in infants are biliary diseases in which different anatomical levels of the biliary tree are specifically affected and characterized by cholangiopathy. |
| PubMedID- 22297845 | One example is the occurrence of primary sclerosing cholangitis (psc) in patients with inflammatory bowel disease, particularly those with inflammation along the length of the colon. |
| PubMedID- 22474447 | Primary sclerosing cholangitis (psc) is a chronic fibro-proliferative disorder of the biliary tree that frequently leads to hepatic failure and death in untreated patients [3, 4]. |
| PubMedID- 26000123 | Aip is associated with other autoimmune diseases, such as sjögren syndrome, primary biliary cirrhosis, primary sclerosing cholangitis, crohn disease and systemic lupus erythematosus in approximately 20–40% of patients [2, 10–14]. |
| PubMedID- 26501124 | Aid = autoimmune liver disease, ald = alcoholic liver disease, cd = cryptogenic disease, hbv = hepatitis b virus, hcc = hepatocellular cancer, hcv = hepatitis c virus, lf = liver failure, nafld = non-alcoholic fatty liver disease, nash = non-alcoholic steatohepatitis, pbc = primary biliary cirrhosis, psc = primary sclerosing cholangitis.supplementary table 2normalised counts per second (using area under the peak) (ncps) for the 40 measured ion peaks which were included in the statistical analysis for patients, controls and room air. |
| PubMedID- 21910861 | Primary sclerosing cholangitis (psc) is a rare entity of aild that has a cholestatic presentation and respond poorly to the treatment, with the ultimate progression to advance liver cirrhosis in most patients. |
| PubMedID- 23520144 | Primary sclerosing cholangitis (psc): benign inflammatory fibrosing disease of the biliary tree; associated with other inflammatory diseases such as colitis and tends to arise in the third decade of life. |
| PubMedID- 24634588 | Primary sclerosing cholangitis (psc) is a chronic fibroinflammatory syndrome linking chronic inflammation to carcinogenesis.33 there is an increased frequency of gallbladder mass lesions in patients with psc; the basis presumably relates to the ongoing inflammation, facilitating a metaplasia–dysplasia–carcinoma sequence.34 the strong correlation between gallbladder dysplasia/adenocarcinoma and bile duct dysplasia/cholangiocarcinoma supports the concept of a neoplastic “field effect” along the intrahepatic and extrahepatic biliary tract in psc patients.34 those with psc therefore should undergo annual gallbladder cancer surveillance, via abdominal ultrasound screening, for masses.35,36 cholecystectomy should be performed for lesions >0.8 cm. |
| PubMedID- 21209818 | The diagnosis of primary sclerosing cholangitis (psc) in this patient is established by the biochemical profile of chronic cholestasis, typical strictures and pruning of the biliary tree upon cholangiography, and ring fibrosis around the bile ducts in liver biopsy. |
| PubMedID- 24833928 | Primary sclerosing cholangitis (psc) is a chronic cholestatic liver disease and one of the most common indications for liver transplantation in adults. |
| PubMedID- 20531971 | Primary sclerosing cholangitis (psc) is a rare cholestatic liver disease with the histological features of chronic inflammation and fibrotic obliteration of the hepatic biliary tree, resulting in bile stasis and hepatic fibrosis.1 it commonly co-exists with autoimmune hepatitis (aih) as a part of hepatic overlap syndrome. |
| PubMedID- 22693517 | Primary sclerosing cholangitis (psc) was the single most common diagnosis in the non-igg4-rd group. |
| PubMedID- 21103219 | Diagnosis of primary sclerosing cholangitis is established by the detection of characteristic alterations in the biliary tree as seen with ercp or percutaneous transhepatic cholangiography or by the detection of periductal fibrosis on liver biopsy. |
| PubMedID- 22988518 | Primary sclerosing cholangitis (psc) is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterized by chronic periductal inflammation and sclerosing of the ducts, which results in segmental stenosis of bile ducts, cholestasis, and fibrosis [1]. |
| PubMedID- 26357612 | Autoimmune hepatitis (aih), primary biliary cirrhosis (pbc) and primary sclerosing cholangitis (psc) are three major types of autoimmune diseases localized in the liver or autoimmune liver diseases, whereas granulomatous diseases such as sarcoidosis and connective tissue diseases such as systemic lupus erythematosus, rheumatoid arthritis and primary sjögren syndrome (pss) are the major systemic autoimmune diseases with liver involvement. |
| PubMedID- 22701310 | A family history of crc, the presence of primary sclerosing cholangitis, backwash ileitis, and, in some studies, young age at onset of colitis increase further the risk of ibd-associated crc [5–8]. |
| PubMedID- 21767410 | Primary sclerosing cholangitis (psc) is a chronic cholestatic liver disease of unknown etiology [1] characterized by classic findings of multiple segmental strictures in the intra- and extra-hepatic bile ducts secondary to inflammation and obliterative fibrosis. |
| PubMedID- 24750423 | Primary sclerosing cholangitis (psc) is a chronic progressive disease of unknown aetiology characterized by fibrosclerotic destruction of the bile ducts resulting in multifocal stricturing of the biliary tree (mendes & lindor 2010; hirschfield et al. |
| PubMedID- 26425607 | One of the most dreaded complications of primary sclerosing cholangitis is cholangiocarcinoma, and this must always be considered in patients such as that presented here. |
| PubMedID- PMC3642475 | Due to a prevalence of primary sclerosing cholangitis (psc) of 5% in uc, patients were asked regarding previous diagnosis of psc. |
| PubMedID- 23681421 | Primary biliary cirrhosis (pbc) and primary sclerosing cholangitis (psc) are rarely found in pediatric celiac patients, with rates between 0.005 and 0.04 % [47, 53]. |
| PubMedID- 23226177 | Although the etiology of primary biliary cirrhosis (pbc), primary sclerosing cholangitis (psc) and autoimmune hepatitis (aih) remains unknown, there are several case reports of association of these hepatic autoimmune conditions in the same patient [1-4]. |
| PubMedID- 26431155 | Rna pools of 15 patients with primary sclerosing cholangitis (psc) and eight patients with cholangiocarcinoma (cc) identified several deregulated mirnas in bile. |
| PubMedID- 20975735 | Primary sclerosing cholangitis 44. this abnormal expression of pdc-e2 may depend on self-antigens being presented by cholangiocytes after binding to hla molecules, although hla class ii on cholangiocytes have more an intrahepatic basolateral than luminal surface expression 56–60 and anyway, are expressed only weakly and in the early stages of disease 60. this observation could be also secondary to immune complexes deposition rather than membrane protein expression. |
| PubMedID- 21059205 | Myasthenia gravis may occur together with primary sclerosing cholangitis in the same patient. |
| PubMedID- 22886419 | Chronic cholelithiasis, primary sclerosing cholangitis (psc), primary biliary cirrhosis (pbc), and biliary atresia are examples of cholestatic human liver diseases, as reviewed elsewhere.1, 2 cholestasis results in bile duct injury caused by the accumulation of toxic hydrophobic bile acids, which also damages the periportal hepatocytes.3 in response to injury, both biliary epithelial cells (becs) and hepatocytes will proliferate to compensate for their respective loss. |
| PubMedID- 24073337 | One of the best examples offered was represented by primary sclerosing cholangitis occurring in uc: in a subset of patients where the presence (in sera and colonic mucosa) of anticolonic mucosa autoantibodies that cross-react with biliary epithelium has been identified [21]. |
| PubMedID- 20740204 | Diseases such as myasthenia gravis, lupus erythematosis, rheumatoid arthritis, thyroiditis and sjögren's syndrome, dermatomyositis, primary sclerosing cholangitis, multiple sclerosis and pernicious anaemia can be seen in combination with thymomas [2]. |
| PubMedID- 24754367 | Autoimmune hepatitis and primary sclerosing cholangitis in children and adolescents. |
| PubMedID- 22927860 | Our objective was to describe a 14-year-old patient with bbs who presented with crohn disease (cd), primary sclerosing cholangitis (psc), and thyroiditis in the context of a cohort review at sainte-justine hospital and to alert clinicians to the increased risk of autoimmune disorders in these patients. |
| PubMedID- 21356211 | Pv hepatocytes, isolated from benign liver resections, normal donor tissue and biliary cirrhosis (primary biliary cirrhosis and primary sclerosing cholangitis) showed significant increase in ros accumulation during hypoxia (fig. |
| PubMedID- 20811628 | Primary sclerosing cholangitis (psc) is a chronic inflammatory condition of the intra- and extrahepatic bile ducts with a prevalence of approximately 10 per 100,000 in western countries [10], [11]. |
| PubMedID- 24949368 | Primary sclerosing cholangitis (psc), a rare condition in only 2%-9% of study populations, is associated with multiple strictures and benign lymphadenopathy. |
| PubMedID- 25788760 | One patient was staged as t0 and was transplanted for primary sclerosing cholangitis and ihc was identified in the explanted liver. |
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