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)

PSC is the most common of the clinically significant hepatobiliary diseases seen in association with IBD, with an incidence that varies from 2.5% to 7.5%. Conversely, 50% to 75% of patients with PSC have IBD. This high degree of association suggests a common pathogenetic mechanism; however, no causal relationship has been established. The etiopathogenesis of PSC remains poorly understood, despite a large number of studies looking at differing hypotheses. The diagnosis is usually established by cholangiography. Liver biopsy can sometimes be helpful in diagnosing pericholangitis. There is a significant overlap of the histology with chronic hepatitis. Serum markers have been studied for diagnosing PSC, particularly for early diagnosis of cholangiocarcinoma, but none have shown the high sensitivity and specificity needed to use them clinically. PSC usually progresses insidiously and eventually leads to cirrhosis. Despite progress in early recognition, optimal management of patients with PSC remains a challenge requiring a multidisciplinary approach among hepatologists, endoscopists, surgeons, and interventional radiologists. Colectomy for ulcerative colitis does not alter the natural history of PSC. There is a high (10% to 15%) incidence of cholangiocarcinoma in patients with PSC. This incidence along with the risk of colon cancer in patients with ulcerative colitis makes it necessary to follow these patients closely. A number of pharmacologic therapies have been evaluated, but none has proven successful in slowing the progression of PSC or prolonging survival. Endoscopic therapy has a proven utility in treating complications of recurrent cholangitis or worsening jaundice in the setting of a dominant stricture, but endoscopy has not been shown to improve survival or decrease the need for liver transplantation. Liver transplantation is life-saving for patients with advanced PSC. Pericholangitis, gallstones, and chronic hepatitis are additional disorders noted in association with IBD, but they are much less common and easier to manage than PSC.
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PMID:Hepatobiliary manifestations of inflammatory bowel disease. 1037 79

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

Although the development of interventional radiology and biliary surgical techniques has prolonged the survival time of patients with primary sclerosing cholangitis, liver transplantation remains the only effective treatment for patients with primary sclerosing cholangitis with liver cirrhosis. Several prognostic survival models have been establised for this disease, and the efficacy of actual liver transplantations has been reported in comparison with these survival models. One- and 5-year actuarial patient survivals after liver transplantation for primary sclerosing cholangitis were shown to be greater than and approximately equal to 90%, respectively. An association with cholangiocarcinoma is the most adverse factor affecting survival after liver transplantation for primary sclerosing cholangitis, while the association of inflammatory bowel disease or previous bili-ary surgery does not adversely affect the outcome of the liver transplantation. Recurrent sclerosing cholangitis is an important issue for posttransplant patients with primary sclerosing cholangitis, and occurs in 10%-20% of such patients. Although our understanding of recurrent sclerosing cholangitis is still in the early stages, its potential occurrence indicates the need for a longer follow-up period after liver transplantation.
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PMID:Liver transplantation for primary sclerosing cholangitis. 1066 84

The clinical and pathological findings of idiopathic ductopenia were studied in a 30-year-old woman who initially manifested jaundice and pruritus. Serum biochemical tests of liver function indicated severe and progressive cholestasis. Viral hepatitis markers and circulating autoantibodies were absent. The patient had a normal cholangiogram and lacked evidence of inflammatory bowel disease. Histological examination of a liver specimen showed severe cholestasis and absence of interlobular bile ducts. Severe jaundice and intractable pruritus developed in the patient and served as the indications for liver transplantation 4 months after initial examination. Transplantation resulted in prompt and complete resolution of the jaundice and pruritus. Two types of idiopathic adulthood ductopenia associated with different prognoses are recognized. Patients with type 1 idiopathic adulthood ductopenia are asymptomatic or manifest symptoms of cholestatic liver disease. They tend to have less destruction of the intrahepatic bile ducts on liver biopsy specimens. Their clinical course ranges from spontaneous improvement to progression to biliary cirrhosis. In contrast, patients with type 2 idiopathic adulthood ductopenia generally manifest initial symptoms of decompensated biliary cirrhosis, have extensive destruction of the intrahepatic bile ducts on liver biopsy, and frequently require orthotopic liver transplantation.
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PMID:Idiopathic adulthood ductopenia: case report and review of the literature. 1076 70

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

Pattern recognition of the long-term disease course before, during, and after pregnancy can provide us with data about the influence of pregnancy on IBD, and vice versa. Determinants that predict an indolent versus an aggressive disease course are currently being sought. Our intention is to analyze the disease course during pregnancy in an EU-IBD inception cohort of 1200 patients diagnosed from 1991 to 1993 and followed up for 10 years. We also attempt to evaluate such factors as smoking and medication and to predict pregnancy course and fertility in IBD as well as in a cross-sectional study of members of the patient organization EFCCA. One of the questions that arose was: what factor is responsible for the observation that pregnancy decreases the incidence of relapses and the development of fibrostenotic lesions? Relaxin and the glycoprotein YKL-40 are validated in the cohort. The protein relaxin, produced by the corpus luteum during pregnancy, increases the laxity of fibrous tissue. Collagen fibers are dissolved and disorganized. As maternal rejection of the fetus does not occur, a protein from the fetal lymphocytes most likely decreases the maternal lymphocyte response. Multiparity may lead to subtle, acquired immune deficits. Glycoprotein YKL-40, which causes fibrosis in RA and cirrhosis, is speculated to be lower in multiparous women than in nonpregnant women due to the fetal lymphocytes that secrete a protein that is a potential immune modulator. Knowledge gained from future EC-IBD studies may result in new legislation (e.g. regarding adoption) that can benefit IBD patients throughout Europe.
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PMID:Pregnancy, fertility, and disease course in patients with Crohn's disease and ulcerative colitis. 1096 10

To investigate whether sclerosing cholangitis with an autoimmune serology characteristic of autoimmune hepatitis (AIH) and AIH are distinct entities, we studied 55 consecutive children with clinical and/or biochemical evidence of liver disease and circulating antinuclear (ANA), anti-smooth muscle (SMA), and/or liver-kidney-microsomal type 1 (LKM1) autoantibodies. They underwent liver biopsy, direct cholangiography, sigmoidoscopy, and rectal biopsy at presentation. Twenty-eight were diagnosed as AIH in the absence and 27 autoimmune sclerosing cholangitis (ASC) in the presence of radiological features of cholangiopathy. Twenty-six ASC and 20 AIH had ANA and/or SMA; 1 ASC and 8 AIH LKM1 autoantibody. Similarities between the 2 conditions included most clinical and biochemical parameters and a lower frequency of HLA DR4. Inflammatory bowel disease and histological biliary changes were more common in ASC; coagulopathy, hypoalbuminemia, lymphocytic periportal hepatitis, and HLA DR3 were more common in AIH. Histological biliary changes were observed in 65% of ASC and 31% of AIH patients. Eighty-nine percent responded to immunosuppression. Follow-up liver biopsies from 17 ASC and 18 AIH patients had similarly reduced inflammatory activity and no progression to cirrhosis. Sixteen follow-up cholangiograms from AIH patients and 9 from ASC patients were unchanged, while 8 ASC patients showed a progressive cholangiopathy. One child with AIH and ulcerative colitis developed sclerosing cholangitis 8 years after presentation. At 2 to 16 years (median, 7 years) from presentation, all patients are alive, including 4 ASC patients who underwent liver transplantation. In conclusion, ASC and AIH are similarly prevalent in childhood; cholangiography is often needed to distinguish between these 2 entities, which are likely to lie within the same disease process.
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PMID:Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. 1123 Jul 33

Since the aetiopathogenesis of primary sclerosing cholangitis (PSC) in humans remains undefined, investigators have studied a variety of animal models to gain insights into immunopathogenetic mechanisms associated with obliterative fibrous cholangitis of intra- and extra-hepatic bile ducts. To date, no animal model has been developed that exhibits all of the attributes of PSC. Rodent models instigated by bacterial cell components or colitis are promising because they may help to explain the strong association between PSC and inflammatory bowel disease (IBD). Other models of direct injury to biliary epithelia, peribiliary vascular endothelia or portal venous endothelia indicate that inflammation, chemokines and cytokines can produce diffuse sclerosis of bile ducts. Models of toxic, infectious or intra-luminal injury of the biliary tract also exhibit focal biliary sclerosis mediated by inflammation and cytokines. The histopathology of several models suggests a sequence of events beginning with secretion of proinflammatory cytokines by activated hepatic macrophages followed by peribiliary infiltration with CD4 and CD8 T cells with a T helper 1 phenotype. These results strongly suggest co-ordinated, pathogenetic roles for both the innate and adaptive immune responses. However, the stimuli that initiate and perpetuate peribiliary fibrosis remain unknown. Interestingly, several models are also associated with the development of anti-neutrophil cytoplasmic antibodies that react in a perinuclear and cytoplasmic pattern similar to that observed in patients with ulcerative colitis and/or PSC. Finally, models of extra-hepatic biliary obstruction continue to provide important information about the pathogenesis of portal fibrosis and secondary biliary cirrhosis that occurs in PSC and other diseases with obstruction of bile flow. Future studies in either existing or new animal models should advance our understanding of the pathogenesis of PSC, the major prerequisite for the development of effective therapies.
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PMID:Animal models for primary sclerosing cholangitis. 1149 70

Although the word synbiotics was coined to describe the combined action of pre- and probiotics, the ability to, like antibiotics, control infection, the term is now increasingly used in a wider sense, as a name for all the substances released by microbial fermentation in the lower gut. One obvious reason is that most of the substances released seem to influence the immune defense, increase resistance to disease, and, most important, prevent complications to surgery such as infections and thrombosis. Protection layer of lactobacillus does not exist only on the GI tract mucosa, it is important at all exterior body surfaces including those of the eye, the nose, the mouth, the respiratory tract, the vagina, not to forget the skin. It is clearly reduced at all sites when the patient is in the settings of ICU. Each human being has his/her own unique microbial collection, especially of strains of Bifidobacterium and Lactobacillus, and it should be possible to identify an individual on the basis of his/her personal intestinal microflora. The flora seems always to be significantly reduced in the sick, especially in connection with severe disease, care in ICU, and in patients with little food intake or on parenteral nutrition. Supply of both pre- and probiotics can modify functions such as appetite, sleep, mood and circadian rhythm, and this most likely through metabolites produced by microbial fermentation in the gut. Supply of lactic acid bacteria (LAB) can also significantly reduce serum levels of a variety of toxins such as endotoxin. An umbrella of supplemented probiotics could provide to the patients with liver cirrhosis a tool to reduce septic manifestations and the incidence of bleeding. LAB are effective in controlling diarrhea of both bacterial and viral origin. A series of experimental studies and several uncontrolled clinical studies support the idea of using probiotics in patients with IBD. Ecoimmunonutrition with pre- pro- and synbiotics offer to be suitable tools in the new millennium.
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PMID:Use of pro-, pre- and synbiotics in the ICU--future options. 1184 May 88

More and more patients are trying out herbal medicine. It is estimated that half of the population have used alternative products at least once in their live. Gastrointestinal diseases often require long-lasting treatments involving many side-effects that can impair the patient's motivation. The majority of persons with symptoms of the irritable bowel syndrome or chronic liver disease resort to non-conventional therapies. However, potential hepatotoxicity of herbal products should not be underestimated. In this article, we discuss herbal preparations in specific gastrointestinal and hepatological indications, concentrating on products that have been tested in randomized, controlled clinical trials. Effective symptomatic treatment of obstipation, irritable bowel and inflammatory bowel disease has been demonstrated with plant-derived preparations. On the other hand phytotherapeutic preparations can not be recommended at present for the treatment of cirrhosis or chronic viral hepatitis based on the available data.
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PMID:[Phytotherapeutic drugs in gastroenterology and hepatology]. 1212 81


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