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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Complications of endoscopic retrograde cholangiography specific to patients with primary sclerosing cholangitis have not yet been reported. We observed transient rises of serum bilirubin after diagnostic endoscopic retrograde cholangiography in five of 15 patients and persistent rises in three of 15 patients with primary sclerosing cholangitis examined consecutively by endoscopic retrograde cholangiography from 1985 to 1990. Deterioration of cholestasis was particularly associated with advanced disease. Seven of eight patients with deterioration after endoscopic retrograde cholangiography had septal fibrosis (stage III) or cirrhosis (stage IV) and a priori elevated serum bilirubin levels. In contrast, all patients with no deterioration of cholestasis following endoscopic retrograde cholangiography had early histological changes (stage I-II), and all but one patient had normal serum bilirubin levels. We conclude that the potentially harmful effects on biliary excretion must be taken into account when the use of endoscopic retrograde cholangiography is being considered in patients with advanced primary sclerosing cholangitis.
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PMID:Deterioration of cholestasis after endoscopic retrograde cholangiography in advanced primary sclerosing cholangitis. 150 31

The responses of serum IRI, serum IRG, and blood sugar levels to 75 g oral glucose and serum IRI to glucagon injection were investigated in 26 chronic hepatitis, 20 liver cirrhosis, 5 primary sclerosing cholangitis (PSC) and 8 healthy volunteers served as controls. The results obtained herein were as follow: 1) The frequency of the glucose intolerance in PSC was higher than the other liver diseases. The mean values of the insulinogenic index (delta IRI/delta BS 30 min) in PSC was lower than control subjects. No suppression of IRG by glucose was observed in PSC. 2) The maximum IRI value (max delta IRI) in PSC during glucagon test was lower than that in control subjects. 3) In one case of 5 PSC ICSA was founded to be positive. These data suggest that we should pay much attention to suffering from diabetes mellitus in natural history of PSC.
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PMID:[Studies on the glucose tolerance and the endocrine function of the pancreas in primary sclerosing cholangitis]. 151 43

Benign bile duct strictures remain one of the most difficult problems encountered by the hepatobiliary surgeon. The vast majority of bile duct strictures occur as a complication of cholecystectomy. The patients may present early in the postoperative period with evidence of a biliary leak or months to years later with the development of jaundice or cholangitis. The essential first step of management consists of delineation of the proximal biliary anatomy. Current management techniques include either operative biliary reconstruction or nonoperative balloon dilatation by either the percutaneous transhepatic or endoscopic routes. The best form of surgical reconstruction of the biliary tree is a biliary-enteric anastomosis from the proximal bile duct to a Roux-en-Y limb of jejunum. In these cases, we favor the use of long-term postoperative biliary stenting using Silastic stents. Recent retrospective, nonrandomized results from our institution favor this surgical technique over nonoperative dilatation. Primary sclerosing cholangitis is a rare cause of biliary strictures. The etiology of sclerosing is unknown, but its association with ulcerative colitis and other diseases suggest an autoimmune condition. The diagnosis is confirmed by typical cholangiographic findings of multiple areas of stricture and dilatation. No medical management has proven to be successful. Surgical management for symptomatic patients includes resection of the hepatic bifurcation with long-term transhepatic stenting of the biliary tree for patients with primarily extrahepatic and/or hilar disease and with no evidence of cirrhosis. In patients with primarily intrahepatic strictures or advanced cirrhosis, liver transplantation is the treatment of choice. Benign strictures due to other causes, such as chronic pancreatitis, calculous biliary disease, sphincter of Oddi stenosis, duodenal Crohn's disease, peptic ulcer, or perivaterian duodenal diverticula usually can be managed by choledochoduodenostomy or choledochojejunostomy without long-term stenting. The management of other rare benign biliary strictures is dependent upon their extent and underlying etiology.
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PMID:Current management of benign bile duct strictures. 153 94

Studies in vitro and in vivo show that hydrophobic bile acids tend to accumulate in the liver tissue in chronic liver disease, thus damaging hepatocyte membranes. Ursodeoxycholic acid (UDCA) is a hydrophilic bile acid which counteracts hepatotoxicity of more hydrophobic bile acids by partially replacing the pool of bile acids in the liver and/or by inhibiting the intestinal absorption of toxic bile acids. UDCA seems safe and effective in the early stages of primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and possibly in other severe cholestatic syndromes of infancy. Moreover, there is increasing evidence that UDCA improves the common indices of liver function in chronic non-cholestatic hepatic disorders including active cirrhosis, though maintaining the residual functional liver mass. This article reviews the cytoprotective effect of UDCA in chronic cholestatic and non-cholestatic liver disease based on the results of major clinical trials on this topic.
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PMID:Cytoprotection with ursodeoxycholic acid: effect in chronic non-cholestatic and chronic cholestatic liver disease. 157 77

Primary sclerosing cholangitis is a condition of unknown cause. It is recognized by liver dysfunction and its characteristic radiologic appearance, which is related to portal tract inflammation, bile duct proliferation, and periductal fibroses involving small intrahepatic and large extrahepatic ducts. The disease lasts about 10 years from the time of diagnosis. Primary sclerosing cholangitis is recognized by abnormal results on routine liver function tests or by the development of clinical jaundice. An autoimmune cause has been suggested because of its strong association with inflammatory bowel disease, certain antigens, AIDS, and immunoregulatory abnormalities. Results of medical management of sclerosing cholangitis have been disappointing. Immunosuppressive drugs, copper chelating agents, and antibiotics have failed to alter progression of the disease. Colectomy in patients with inflammatory bowel disease also has no influence. The judicious use of dilations of strictures, bypass procedures, or resection can palliate jaundice in patients with primary sclerosing cholangitis, but liver transplantation is the definitive treatment. Because palliative operations increase the hazards of liver transplantation, percutaneous dilations and stentings are preferred initially. Cirrhosis and portal hypertension are indications for transplantation. In the future, transplantation may be indicated earlier in the course of the disease.
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PMID:Primary sclerosing cholangitis. 158 51

Evaluation of chronic liver disease begins with a carefully taken history, thorough physical examination, and standard laboratory tests. Often, however, other studies are required, such as a viral hepatitis panel, serologic tests for autoimmune markers, tests for antimitochondrial antibodies, measurement of serum iron and ceruloplasmin levels, liver biopsy, and imaging studies of the extra-hepatic bile ducts. Medical treatment of chronic active hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis remains unsatisfactory. Early treatment of hemochromatosis and Wilson's disease can prevent cirrhosis and liver failure. Liver transplantation is now a viable procedure for patients with end-stage chronic liver disease.
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PMID:Chronic liver disease. The scope of causes and treatments. 158 71

It has been suggested that immunological mechanisms involving lymphocyte-mediated damage are important in the characteristic bile-duct damage that occurs in primary biliary cirrhosis and primary sclerosing cholangitis. Because adhesion is necessary for the interaction of lymphocytes with their target structures, we have studied the expression of intercellular adhesion molecule 1, a ligand for the leukocyte adhesion receptor lymphocyte function-associated antigen 1 in the liver of patients with primary biliary cirrhosis and primary sclerosing cholangitis. Strong expression of intercellular adhesion molecule 1 was seen on interlobular bile ducts and proliferating bile ductules in both conditions. In primary biliary cirrhosis, medium-sized ducts, which are spared by the disease, were negative. Minimal bile-duct staining was seen in conditions in which bile-duct damage is not a major feature, such as nonbiliary cirrhosis and acute liver diseases. In patients with cirrhosis from any cause, strong expression of intercellular adhesion molecule 1 was detected on the periseptal hepatocytes adjacent to new connective tissue. The intensity of immunohistochemical staining was recorded using a semiquantitative visual scoring system that was subsequently validated quantitatively by confocal laser scanning microscopy. The expression/induction of intercellular adhesion molecule 1 on bile ducts may be important in the pathogenesis of bile-duct damage in primary biliary cirrhosis and primary sclerosing cholangitis and is further evidence to support an immune pathogenesis in these two conditions. Furthermore, the induction of intercellular adhesion molecule 1 on hepatocytes may be an important factor in the liver-cell damage and fibrosis that occur during the development of cirrhosis.
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PMID:Increased expression of intercellular adhesion molecule 1 on bile ducts in primary biliary cirrhosis and primary sclerosing cholangitis. 171 72

From 1975 to 1989, primary sclerosing cholangitis (PSC) was diagnosed in 15 patients especially based on cholangiographic features associated with clinical, biological and histological findings. 93 p. cent of patients had altered liver enzymes at the diagnosis time but only 26% was asymptomatic. 33 p. cent had a concomittant inflammatory bowel disease. Retrograde cholangiography showed injuries of the intrahepatic ducts in 93 p. cent; the more frequently observed lesions were short and multifocal strictures, associated with irregularities of the biliary wall or decreased arborization of intrahepatic bile ducts, but without marked dilatation. Mean follow-up was 44 months (3 to 120). Actuarial survival was 48 p. cent at 5 years. In 4 patients, because of poor clinical and biological (serum bilirubin greater than or equal to 4 mg/dl) evolution, a second retrograde cholangiography was performed showing in all cases apparition of marked ductal dilatation in extra-hepatic (one case) or in intra-hepatic bile ducts (three cases). We diagnosed one benign extra-hepatic stricture, two cholangiocarcinoma and one biliary cirrhosis complicating PSC. We concluded that retrograde cholangiography is necessary if jaundice appears to diagnose every complications of PSC. Apparition of marked ductal dilatation should be the witness of pejorative evolution, especially apparition of cholangiocarcinoma that must be excluded.
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PMID:[Primary sclerosing cholangitis: contribution of retrograde cholangiography in follow-up: apropos of 15 case reports]. 180 32

The preceding discussions outline the various forms of cirrhosis that may be encountered in the elderly population. Cirrhosis is not uncommon in older patients. Although it has been stated that most cirrhosis in the elderly is due to alcohol, these assumptions are perhaps overestimations. In the authors' experience, many older patients are inappropriately labeled with alcoholic liver disease--presumed guilty until proven otherwise--and have subsequently been shown to have nonalcoholic liver disease. Careful investigation is required. Hepatotoxic drug exposure (e.g., to alpha methyldopa, nitrofurantoin, or isoniazid) should be ruled out, and hepatitis B and hepatitis C serology obtained. Primary biliary cirrhosis may occur in both sexes, and thus antimitochondrial antibody should be assayed. Severe heart disease may result in cardiac cirrhosis in the elderly, with ascites and hepatomegaly. Alpha 1-antitrypsin deficiency, primary sclerosing cholangitis, idiopathic hemochromatosis, and chronic autoimmune hepatitis may result in advanced cirrhosis in the elderly; appropriate serum studies should be obtained. If questions remain and if therapy may be changed, liver biopsy can be performed. A recent study suggested, however, that the risk of hemorrhage from liver biopsy in the elderly may be increased, especially if malignancy is present. The era of treatment for liver diseases has arrived. Colchicine, methotrexate, ursodeoxycholic acid, and others have shown promise in the treatment of PBC, primary sclerosing cholangitis, and alcoholic liver disease. Corticosteroids may be lifesaving in autoimmune liver disease. Phlebotomy remains the treatment of choice for hemochromatosis in any age group. Interferons and other antiviral agents are being used in chronic type B and type C hepatitis. Treatment of the complications of cirrhosis in the elderly may be safely accomplished. Advanced age is not a contraindication to variceal sclerotherapy. Vasopressin, however, may be contraindicated in the elderly patient if there is an underlying history of atherosclerotic coronary or peripheral vascular disease. Large-volume paracentesis and peritoneal venous shunting can afford symptomatic relief of ascites, even in the geriatric population. Finally, as noted previously, advanced age is no longer to be considered an absolute contraindication for liver transplantation. The evaluation of liver disease in the elderly may be diagnostically challenging, and its treatment rewarding.
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PMID:Liver diseases in the elderly. 185 64

Tissue from 15 livers with primary sclerosing cholangitis, obtained at transplantation, was examined histologically with respect to: small and medium sized bile duct lesions; large bile duct lesions; fibrosis/cirrhosis; and parenchymal changes. Lesions affecting small and medium-sized bile ducts were quantified by determining the percentage of 20 portal tracts involved. The two characteristic bile duct lesions of primary sclerosing cholangitis, periductal fibrosis and fibro-obliterative scars, were largely confined to medium-sized portal areas. Although present in each case, the number of such lesions varied considerably. Loss of bile ducts was the most conspicuous feature in small portal tracts where the diagnostic duct lesions of primary sclerosing cholangitis were rarely observed. Inflammation, ulceration and cholangiectases of large intrahepatic ducts were common, and appear to be useful additional diagnostic features.
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PMID:The spectrum of bile duct lesions in end-stage primary sclerosing cholangitis. 193 11


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