Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary tumors of the liver that are of clinical significance are rare. Ninety-five percent of such lesions when encountered will be malignant and only 5% will be benign. Malignant primary hepatic lesions represent 2% to 3% of primary cancers encountered in the United States. Hepatocellular carcinoma constitutes 90% of malignant liver primaries in the adult. Seventy-five percent of cases are associated with cirrhosis of the liver and patients with hepatitis B infection have a 33- to 200-fold excess risk for this malignancy. Cholangiocarcinoma represents 5% to 10% of hepatic primary malignancies while hepatoblastoma is distinctly uncommon in adults. Treatment is primarily surgical, and resectability is limited by the presence of cirrhosis and spread of the tumor within and outside of the liver. Of the benign liver tumors, the liver cell adenoma seem to be associated with oral contraception and have a proclivity for intraperitoneal hemorrhage, especially during pregnancy. Focal nodular hyperplasia is a tumor-like condition that also may be associated with oral contraception. This article describes five cases, two of which had quite unique presentations.
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PMID:Primary tumors of the liver. 160 11

Cholangiocarcinomas occasionally arise in the nonbiliary cirrhotic liver, but their pathobiologic features remain unsettled. To characterize such cholangiocarcinomas, we examined immunohistochemically the expression of MUC3, MUC5AC, MUC6, and MUC7 apomucins in hepatic tissue specimens from 4 patients with cholangiocarcinoma and viral cirrhosis, 24 patients with cholangiocarcinoma without cirrhosis, and 16 patients with combined hepatocellular-cholangiocellular carcinoma. The cholangiocarcinomas associated with cirrhosis and the cholangiocarcinoma elements of combined hepatocellular-cholangiocellular carcinoma rarely expressed MUC3 and MUC5AC, but the cholangiocarcinomas not associated with cirrhosis frequently expressed these apomucins. MUC6 apomucin was widely expressed in cholangiocarcinomas, irrespective of the association with cirrhosis and also in the cholangiocarcinoma elements of the combined hepatocellular-cholangiocellular carcinomas. MUC7 apomucin was expressed frequently in cholangiocarcinomas associated with cirrhosis and combined hepatocellular-cholangiocellular carcinomas and infrequently in cholangiocarcinomas without cirrhosis, particularly those arising at the hepatic hilus. The similarity of the apomucin profiles between cholangiocarcinomas associated with cirrhosis and the cholangiocarcinoma elements of combined hepatocellular-cholangiocellular carcinoma suggests a similar or common histogenesis and that cholangiocarcinoma associated with cirrhosis might be derived from the combined type.
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PMID:Cholangiocarcinomas arising in cirrhosis and combined hepatocellular-cholangiocellular carcinomas share apomucin profiles. 949 2

Cholangiocarcinoma has a worldwide distribution which accounts for about 10-15% of all cases of primary hepatobiliary malignancy. Although, in the majority of cases, no aetiological factor can be identified, a number of risk factors have been shown to be important in the development of cholangiocarcinoma; most of these factors share long standing inflammation and chronic injury of the biliary epithelium. Primary sclerosing cholangitis is an uncommon disease, characterized by stricturing, fibrosis and inflammation of the biliary tree which is closely associated with chronic inflammatory bowel disease, particularly ulcerative colitis. It is commonly associated with cholangiocarcinoma and between 10-20% of patients with primary sclerosing cholangitis will go on to develop a cholangiocarcinoma. The rare congenital fibropolycystic diseases of the biliary system are associated with increased risks of cholangiocarcinoma, particularly choledochal cysts and Caroli's disease. Choledochal cysts are associated with a 10% overall incidence of cholangiocarcinoma: there is a 1% cumulative risk which plateaus after 15-20 years. However, the risk is diminished in children who present under the age of 10 years where the over all risk is 0.7%. This compares with the 14% over all risk of patients presenting over the age of 20 years. In the Far East, other forms of chronic inflammation associated with cholangiocarcinoma include infestation with liver flukes. Clonorchis sinensis and Opisthorchis viverinni. Cholangiocarcinoma is also rarely seen in association with cirrhosis and has been weakly linked to hepatitis C infection.
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PMID:Risk factors for biliary tract carcinogenesis. 1043 47

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology that is progressive in most symptomatic patients, advancing toward cirrhosis and liver failure. Liver transplantation is the only therapeutic option for patients with end stage liver disease resulting from this disorder. The results of transplantation for PSC are excellent with one-year survival rates of 90-97% and five-year survival rates of 80-85%, but are closely related to pre-transplant Child-Pugh stage. Recurrence of PSC after liver transplantation is common, occurring in up to 20% of patients, but it appears to have little effect on patient survival, as survival of patients with recurrent PSC is similar to that of those without evidence of recurrence. Cholangiocarcinoma is a catastrophic complication of PSC and as yet no reliable screening method exists. The results of liver transplantation for patients with clinically apparent cholangiocarcinoma are extremely poor, however in patients in whom a microscopic tumour is detected in the explanted liver, survival is similar to those transplanted with PSC without cholangiocarcinoma. Activity of inflammatory bowel disease (IBD) appears to be more severe after transplantation, especially in units where steroid immunosuppression is withdrawn early. Colon cancer appears within the first few years after transplantation in approximately 7% of patients with IBD who are transplanted for PSC. Annual colonoscopy in this population seems prudent.
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PMID:Liver transplantation for primary sclerosing cholangitis. 1084 76

Whether heterozygotes with alpha-1-antitrypsin (AAT) deficiency type PiZ bear an increased risk for chronic liver disease is controversial. On the basis of liver tissue from 1,030 autopsies (autopsy series), 1,847 biopsies (biopsy series) and 317 primary liver carcinomas (tumor series), we analysed the effect of heterozygous state PiZ for the development of liver diseases. The PiZ status was screened immunohistochemically and verified in selected cases by SSCP analysis and by sequencing DNA extracted from paraffin embedded tissue. The PiZ frequency in the biopsy series (3.4%) and tumor series (5.99%) was significantly higher than in the autopsy series (1.8%). Hepatic PiZ deposits in heterozygotes sometimes were as extensive as in homozygotes. The amount of PiZ deposits correlated positively with the inflammatory activity and stage of fibrosis, as well as with the age of patients. Patients with concurrent liver disease such as hepatitis and alcoholic liver disease showed significantly higher scores of inflammatory activity, stage of fibrosis and amount of PiZ deposits than those without additional liver disease. Cholangiocarcinomas and combined hepato-cholangiocarcinomas were seen significantly more frequently in patients with PiZ-associated liver carcinoma than in genetic healthy individuals (p = 0.004). Three out of 19 PiZ-associated liver carcinomas had developed in cirrhotic liver tissue. Heterozygotes of type PiZ have an enhanced risk for chronic liver disease including primary liver carcinoma. PiZ-associated liver diseases will become clinically manifest in middle or old aged adults. Rarely this genetic defect causes liver cirrhosis even without concurrent liver disease. PiZ-associated liver carcinomas are frequently characterized by cholangiocellular differentiation and may develop often in non-cirrhotic liver tissue. Immunohistochemistry is a specific method to detect hepatic PiZ deposits.
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PMID:[Liver changes in heterozygote alpha 1-antitrypsin deficiency PiZ]. 1114 23

Liver transplantation is an accepted form of treatment for patients with primary sclerosing cholangitis (PSC) and can provide long term survival. Cholangiocarcinoma occurs in 10% to 20% of patients with PSC, is difficult to diagnose and has a poor prognosis. It has been proposed that liver transplantation be undertaken early in the course of the PSC, before cancer develops. Such a proposal would have significant implications for the method of assigning priority to patients awaiting liver transplantation. Other patients on the waiting list would experience further delays, while there is no proven benefit for PSC patients. Few patients with this disease are removed from the waiting list because they developed cancer. If one were to state that PSC patients warrant special consideration because of the hypothetical risk of cholangiocarcinoma, the same argument could be applied to patients with hepatitis C and other causes of cirrhosis, who are at increased risk of hepatocellular carcinoma. The transplant allocation system is applied in an equitable fashion to patients with a large variety of liver diseases. Alteration of this system to benefit a small number of patients with PSC would violate the principles on which it was created, and cannot be justified.
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PMID:Motion - patients with primary sclerosing cholangitis should undergo early liver transplantation: arguments against the motion. 1242 31

Cholangiocarcinoma is a malignancy of the biliary duct system. Presently, these malignant biliary tract diseases are common among the people in Southeast Asia, with the world's highest prevalence in northeastern Thailand and Laos. This carcinoma tends to grow slowly and to infiltrate the walls of the ducts, dissecting along tissue planes and leading to biliary tract obstruction. Recently, hepatitis virus-associated chronic hepatitis or cirrhosis has been suggested to be involved in the pathogenesis of cholangiocarcinoma. Here, we report the prevalence of hepatitis B seropositivity among Thai patients with cholangiocarcinoma. A total of 55 patients with cholangiocarcinoma, who were admitted to King Chulalongkorn Memorial Hospital, Thailand, during 1992-2001, with known hepatitis B serology was selected for further prevalence study. Of the 55 cases (32 males and 22 females), hepatitis B seropositivity was found in five cases (three males and two females), giving a total prevalence rate equal to 9.1% (9.4% for male and 9.2% for females). Since hepatitis B infection is believed to be a risk factor in the development of cholangiocarcinoma, and both cholangiocarcinoma and hepatitis B infection are common in Thailand, screening and vaccination for hepatitis B may be a useful strategy to decrease the high rate of cholangiocarcinoma in Thailand. Further long-term study to study the relation of hepatitis B infection and cholangiocarcinoma is recommended.
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PMID:The prevalence of hepatitis B seropositivity among patients with cholangiocarcinoma. 1251 80

Cholangiocarcinoma (CC) is a malignant neoplasm deriving from intra- and extrahepatic bile ducts. It affects both sexes, and is most prevalent at the age 50 to 70. Chronic nonspecific ulcerative colitis, primary sclerosing cholangitis, hepatolithiasis, congenital hepatic fibrosis, and Caroli's disease may lead to the increased incidence of CC. Recently, hepatic cirrhosis in the course of virus-associated chronic hepatitis has been suggested to be involved in the pathogenesis CC. Histologically, 90-95% of CC are well differentiated adenocarcinomas. Usually the tumor grows slowly and metastazes late locally and even less frequently extrahepaticly. CC often causes symptoms by blocking the bile ducts, abdominal pain, weight loss, signs of portal hypertension, rare ascites and thrombophlebitis. Serum chemistry was compatible with obstructive jaundice. The increased expression of CEA, Ca19-9, as well as loss or reduction of sialomucin/sulfomucin concentration in the biliary lining epithelium may be indicative of malignant changes. CC as usually non-vascularized nonencapsulated tumor with a large amount of fibrosis. It is isochogenic in classical USG, CT or MRI. MRCP-magnetic resonance cholangiopancreatography and virtual endoscopy are more helpful methods on the diagnostics of CC. Recently, FDG positron emission tomography has been suggested to be a sensitive technique in identifying small bile duct cancers. Surgical excision of the lesion confirmed localized CC. The adjuvant radio- and chemotherapy and transplantation are not satisfactory. Palliative therapy includes surgical biliary-intestinal bypass procedures as well as operative and nonoperative techniques for biliary intestinal drainage. Recently, the local treatment of CC by photodynamic therapy as a palliative strategy is very promising. Ordinary CC is reported as a neoplasm with a poor prognosis. Post resection 5-year survival is affirmed in about 25% of CC, whereas after palliative treatment only 1 year.
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PMID:[Cholangiocarcinoma--bile ducts cancer]. 1290 Dec 70

Cholangiocarcinoma (CCA) is a lethal disease, afflicting many thousands the world over. Human CCA develops through a multi-step progression model, preceded by the onset of dysplasia in the cholangiolar ductal epithelium. An animal model of multi-step carcinogenesis in the biliary tree will enable the study of genetic changes in human CCA, and provide an avenue for chemoprevention strategies. We describe an oral thioacetamide (TAA)-induced model of rat CCA that recapitulates the histologic progression of human CCA. Male Sprague-Dawley (SD) rats (n = 170), weighing 350 +/- 20 g, were used in this study. Drinking water with TAA 300 mg/l was administered orally, and the liver was harvested and examined histologically at weekly intervals, beginning at 5 weeks after initiation of TAA. Harvested tissues were formalin-fixed and paraffin embedded for morphologic and immunohistochemical studies. Multifocal bile ductular proliferation with intestinal metaplasia (presence of goblet cells) and increasing histologic atypia (biliary dysplasia) was observed by the 9th week of TAA administration. Biliary cytokeratin (CK19)-expressing invasive intestinal-type CCA with stromal desmoplasia was evident at the 16th week, and by the 22nd week, the yield rate for CCAs had increased to 100%. Invasive CCAs preceded the development of hepatic cirrhosis by at least 4 weeks; the earliest incidence of hepatic fibrosis was observed beginning at 20 weeks post-TAA administration. The progression from normal cholangioles to biliary dysplasia to invasive CCA was accompanied by up-regulation of the proto-oncogenes c-met and c-erbB-2, tyrosine kinase receptors over-expressed in human CCAs. The study was terminated at 6 months, at which time no systemic metastases or deaths were observed. Oral administration of TAA in drinking water to male SD rats provides a reproducible animal model for development of CCA with a high yield rate. In particular, the presence of biliary dysplasia beginning at the 9th week, which progresses to invasive CCA, mimics the multi-step model of human CCA. The TAA rat model may serve as a powerful pre-clinical platform for therapeutic and chemoprevention strategies for human CCA.
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PMID:Thioacetamide-induced intestinal-type cholangiocarcinoma in rat: an animal model recapitulating the multi-stage progression of human cholangiocarcinoma. 1465 42

Cholangiocarcinomas are rare malignant tumors composed of cells that resemble those of the biliary tract. On the basis of their anatomic location, cholangiocarcinomas can be classified as intrahepatic, extrahepatic and hilar tumors. For reasons that are not clear, the incidence of cholangiocarcinoma is increasing globally. Established risk factors, including conditions associated with chronic biliary tract inflammation, account for a small proportion of cases. Additional risk factors such as cirrhosis, infection with hepatitis B virus and hepatitis C virus are now becoming recognized. The diagnosis of cholangiocarcinoma requires the integration of clinical information, imaging studies of the hepatic parenchyma and biliary tract, tumor markers, and histology. In terms of the treatment options for cholangiocarcinoma, surgery can be curative, although few patients are candidates for surgery. Palliative biliary decompression can provide symptomatic relief. Advances in MRI and positron-emission tomography scanning, identification of new tumor markers, improved utility of biliary cytology, and the use of photodynamic therapy for adjunct treatment are all expected to enhance the diagnosis, evaluation and management of cholangiocarcinoma.
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PMID:Cholangiocarcinoma. 1639 10


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