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 sclerosing cholangitis (PSC) is a chronic cholestatic and progressive disease, of unknown etiology, characterized by inflammation and fibrosis of intra-hepatic and extra-hepatic biliary tree. Two thirds of the patients have, simultaneously, ulcerative colitis (UC); on the other hand, PSC is the most common chronic hepatic disease in patients with inflammatory bowel disease (IBD). Patients who have both diseases simultaneously are prone to develop colorectal carcinoma and cholangiocarcinoma. The disease predominantly affects young men, may be asymptomatic or presented as fluctuating jaundice, pruritus and increased levels of the cholestasis biochemical markers, or as secondary billiary cirrhosis. The diagnostic gold standard are the cholangiographic abnormalities, consisting of multifocal stenosis and dilatations, involving both the intrahepatic and the extrahepatic biliary tree. Liver biopsy is useful only for staging the disease and to confirm the diagnosis, in the atypical forms. Ursodeoxycholic acid is a promising drug, nowadays, mainly in the first stages of the disease, in spite of doubtful efficacy. Combination therapy, using ursodeoxicolic acid, methotrexate and alternating antibiotics monthly may be sucessfull in PSC before bile strictures occur. Liver transplantation is the only life-saving therapeutic alternative, able to improve significantly the survival and the life quality of the patients.
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PMID:[PRIMARY SCLEROSING CHOLANGITIS] 1214 May 88

Autoimmune hepatitis (AIH) is a rare autoimmune disease (incidence about 5% among all chronic liver disorders) that reflects a loss of tolerance to normal hepatic proteins. AIH is characterized by female preponderance, hypergammaglobulinemia, extrahepatic syndromes and a good response to immunosuppressive treatment. AIH may be subdivided into two or three subtypes. AIH type 1 is characterized by antinuclear autoantibodies (ANA) and/or smooth muscle antibodies (SMA). SMA are actin-specific, can occur without ANA and their presence relates strongly to AIH. AIH type 2 is defined by the presence of anti-liver-kidney microsomal antibodies (LKM-1). Patients with AIH type 2 are typically younger at the time of disease onset, exhibit higher inflammatory activity, suffer more frequent relapses under immunosuppressive treatment and are more likely to progress to cirrhosis. AIH type 3 is characterized by autoantibodies against the soluble liver antigen (SLA) and liver-pancreas antigen (LP), but ANA/SMA are frequently present and, therefore, some authors consider this autoantibody manifestation as belonging to AIH type 1. Antineutrophil cytoplasmic antibodies (ANCA) recognize cytoplasmic or nuclear components of neutrophilic granulocytes and are detected with high prevalence in patients with autoimmune liver diseases. They are associated with AIH type 1 but not with AIH type 2. However, 40-70% of patients with primary sclerosing cholangitis (PSC) also produce these autoantibodies. Autoimmune cholangitis is an idiopathic disorder with mixed hepatocellular and cholestatic findings that typically has antinuclear antibodies (ANA). It may be considered as an atypical form of primary biliary cirrhosis. It has been recognized that some forms of AIH may also occur with variable incidence and severity especially in patients with primary biliary cirrhosis (overlap AIH/PBC) or primary sclerosing cholangitis (AIH/PSC). On the basis of clinical, biochemical, serological, histological and radiological criteria a clear distinction between these conditions can be readily made in the majority of cases. An association of AIH-typical autoantibodies (anti-LKM-1, anti-SLA/LP) in association with antimitochondrial autoantibodies (AMA) almost confirm the overlap syndrome AIH/PBC. In PSC patients expressing typical ERCP findings and suffering from inflammatory bowel disease (IBD), the diagnosis of an overlap syndrome between PSC/AIH can be readily made in the presence of ANCA and AIH relevant autoantibodies. Apart from this kind of overlap syndrome involving different types of autoimmune disorders within the liver AIH can be also associated with other organspecific autoimmune disorders as documented in the autoimmune polyglandular syndrome type 1 (APS-1). In this disease homozygosity for a defect in a single gene (AIRE) leads to a broad spectrum of organ specific autoimmune diseases.
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PMID:[Autoimmune hepatitis and overlap syndrome: diagnosis]. 1223 64

Family planning practitioners should be aware of the possible effects of contraceptives on patients with gastrointestinal, liver, and gallbladder disorders. It is recommended that women with inflammatory bowel disease not become pregnant until their disease has been under control for 1 year. There is some evidence that oral contraceptive (OC) use is associated with an increased incidence of ulcerative colitis and Crohn's disease. Patients with stable, quiescent inflammatory disease can use a barrier method or, with reservations, a low-dose estrogen OC or a progestin-only preparation. No reports are available regarding the effect of the IUD on inflammatory bowel disease. Since ingestion of contraceptive steroids alters hepatocellular function, OCs are contraindicated during active liver disease or cirrhosis. The association between Oc use and cholelithiasis remains controversial. However, it has been determined that gallbladder bile is significantly more saturated with cholesterol during OC use than during normal menstrual functioning. OC use may precipitate clinically apparent gallbladder disease in women with subclinical cases. OC use should be avoided or pursued with great caution in women with a history of gallbladder disease or prior cholecystectomy.
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PMID:Colitis, hepatitis are disorders that pose counseling problems. 1233 12

Inflammation is overall a protective response, whose main goal is to liberate the human being of cellular lesions caused by micro-organisms, toxins, allergens, etc., as well as its consequences, and of death cells and necrotic tissues. Chronic inflammation, which is detrimental to tissues, is the basic pathogenic mechanism of hypersensitivity reactions against xenobiotics. Other frequent pathologies, for instance atherosclerosis, chronic hepatitis, inflammatory bowel disease (IBD), liver cirrhosis, lung fibrosis, psoriasis, and rheumatoid arthritis are also chronic inflammatory diseases. Chemical mediators of inflammation are derived from blood plasma or different cell-type activity. Biogenic amines, eicosanoids and cytokines are within the most important mediators of inflammatory processes. The different activities of eicosanoids derived from arachidonic acid (20:4 n-6) versus those derived from eicosapentaenoic acid (20:5 n-3) are one of the most important mechanisms to explain why n-3, or omega-3, polyunsaturated fatty acids (PUFA) exhibit anti-inflammatory properties in many inflammatory diseases. Dietary supplements ranging 1-8 g per day of n-3 PUFA have been reportedly beneficial in the treatment of IBD, eczema, psoriasis and rheumatoid arthritis. In addition, recent experimental studies in rats with experimental ulcerative colitis, induced by intrarectal injection of trinitrobenzene sulphonic acid, have documented that treatment with n-3 long-chain PUFA reduces mucosal damage as assessed by biochemical and histological markers of inflammation. Moreover, the defence antioxidant system in this model is enhanced in treated animals, provided that the n-3 PUFA supply is adequately preserved from oxidation.
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PMID:Polyunsaturated fatty acids and inflammatory diseases. 1244 11

Primary sclerosing cholangitis (PSC) is a chronic cholestatic hepatobiliary disease that usually progresses to biliary cirrhosis and liver failure; it also predisposes to cholangiocarcinoma. The cause of PSC is unknown, although evidence suggests that the tissue damage is mediated by the immune system. There is an unexplained close association between PSC and inflammatory bowel disease, particularly in ulcerative colitis, which coexists in the majority of patients with PSC. No medical therapy has been proven to halt or reverse disease progression; however, recent preliminary evidence suggests that ursodeoxycholic acid (UDCA) in a high dose of 20 to 25 mg/kg may slow the disease process. Evidence from a pilot study suggests that the combination of UDCA and immunosuppressive therapy, such as prednisolone or azathioprine, may also increase efficacy. For patients with end-stage PSC, liver transplantation remains the only effective therapy, although there is clear evidence that PSC may recur in the liver allograft.
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PMID:The management of primary sclerosing cholangitis. 1253 Sep 43

Gene therapy consists of the transfer of genetic material to cells to achieve a therapeutic goal. In the field of gastroenterology and hepatology gene therapy has produced considerable expectation as a potential tool in the management of conditions that lack effective therapy including non-resectable neoplasms of the liver, pancreas and gastrointestinal tract, chronic viral hepatitis unresponsive to interferon therapy, liver cirrhosis, and inflammatory bowel disease.
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PMID:The promise of gene therapy in gastrointestinal and liver diseases. 1265 82

Endoscopic therapy can be employed and may be useful in inflammatory bowel disease patients with dysplastic polyps, inflammatory strictures (enteric and biliary), bleeding, and for treatment of some complications of continent ileostomies. Dysplastic polyps can be removed endoscopically safely and effectively without resorting to colectomy, as long as there is no other detectable dysplasia in flat mucosa and complete removal can be assured (by biopsy of adjacent mucosa and close follow-up). Some colonic strictures in Crohn's disease can be dilated using endoscopes of graded caliber or with through-the-scope balloons, with or without stent placement. Endoscopy is most useful in dilating anastomotic strictures and less useful for long strictures in active inflammatory disease. Strictures in ulcerative colitis are suspicious for neoplasia and, if dilated at all, should be biopsied extensively and followed closely. We prefer colectomy to endoscopic dilatation for strictures in ulcerative colitis. Biliary endoscopy can be used to dilate strictures in primary sclerosing cholangitis and to sample these areas for malignancy. Although dilatation may improve morbidity and prolong survival, it may not prevent progression to cirrhosis. In cholangiocarcinomas, endoscopic dilatation with or without stents can offer palliation and perhaps be used to deliver photodynamic therapy. Injection and sclerotherapy can be employed in Crohn's disease bleeding from a discrete site. In obstructed continent ileostomies (both Kock pouches and pelvic ileoanal reservoirs), endoscopy can be employed effectively to both determine the cause of the obstruction and re-establish patency. We do not advocate endoscopic treatment of toxic megacolon because of the heightened risk of perforation. Endoscopic therapy of Crohn's fistulas is a possible emerging technology, but it has not been used in large cohorts of patients.
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PMID:Endoscopic Therapy for Inflammatory Bowel Disease. 1274 23

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin, characterized by inflammation, fibrosis, and obliteration of bile ducts, which ultimately results in biliary cirrhosis. The condition most commonly affects intrahepatic and extrahepatic bile ducts together, but sometimes only intrahepatic or extrahepatic ducts are involved. PSC is often associated with inflammatory bowel disease, especially ulcerative colitis. The majority of patients are initially asymptomatic, and identified on the basis of elevated serum levels of alkaline phosphatase or gamma-glutamyl transpeptidase, especially while screening patients with ulcerative colitis. Diagnosis is based on characteristic cholangiographic appearance with focal bile duct dilatations proximal to areas of stricturing that produce a beaded appearance. Ursodeoxycholic acid is most effective medical therapy, with other symptomatic measures, while liver transplantation is the treatment of choice for patients with advanced liver disease.
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PMID:[Primary sclerosing cholangitis--diagnosis and therapy]. 1458 67

Primary sclerosing cholangitis (PSC) is an idiopathic, chronic cholestatic liver disease of uncertain etiopathogenesis commonly associated with inflammatory bowel disease (IBD) and is characterized by patchy inflammation of the biliary tree progressing to fibrosis and strictures. The natural history of PSC is highly variable but characteristically follows a progressive clinical course leading to biliary tree strictures, cholestasis, and choledocholithiasis. The course of the disease may be complicated by cholangitis, secondary biliary cirrhosis, liver failure, and cholangiocarcinoma. The diagnosis of PSC is based on typical cholangiographic findings, supported by nonspecific clinical signs and symptoms, cholestatic liver biochemical tests, and liver biopsy. Uncommon and usually clinically obvious secondary causes of sclerosing cholangitis are excluded before establishing the diagnosis of PSC. Therapeutic approaches that show promise include endoscopic therapy and ursodeoxycholic acid. The only accepted therapy for end-stage PSC that can improve long-term outcome is liver transplantation. The diagnosis of cholangiocarcinoma--often difficult and elusive--usually precludes liver transplantation because its prognosis is very poor.
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PMID:Primary sclerosing cholangitis. 1471 69

Whilst the importance of mutations in a wide range of keratins in skin fragility disorders is now well established, there is much less evidence for simple epithelial keratin involvement in disease. Some simple epithelial keratin mutations have been reported in liver cirrhosis and pancreatitis patients, and recently mutations in the simple epithelial keratin K8 were identified in a group of patients with inflammatory bowel disease (Crohn disease or ulcerative colitis). In comparison with the mutations seen in epidermal keratins, these simple epithelial mutations would be predicted to have mild consequences, although analysis shows that they do have a distinct effect. This review article discusses the evidence that these mutations are a predisposing factor for inflammatory bowel disease.
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PMID:Keratin mutations and intestinal pathology. 1549 67


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