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)

To assess the prevalence and significance of chronic ulcerative colitis in patients with severe autoimmune hepatitis and to determine the frequency of cholangiographic and histologic features of primary sclerosing cholangitis in those with colitis, 105 patients who had been screened by annual proctoscopic examination were studied. Patients with features of colitis were compared to counterparts without colitis who had been matched by age, sex, disease severity and treatment regimen. Seventeen patients (16%) had findings of chronic ulcerative colitis. Twelve of these underwent cholangiography and five (42%) had features of primary sclerosing cholangitis. Patients with and without cholangiographic abnormalities were indistinguishable by clinical, laboratory, immunoserologic, and histologic features. Fibrous obliterative cholangitis was present in only two patients, including one with normal cholangiography. Patients with colitis entered remission less frequently (59 vs. 94%, p less than 0.05), failed treatment more commonly (41 vs. 6%, p less than 0.05) and progressed to cirrhosis more frequently (75 vs. 25%, p less than 0.05) than counterparts without colitis. Patients with colitis but normal cholangiography, however, responded satisfactorily to therapy. We conclude that chronic ulcerative colitis can coexist with severe autoimmune hepatitis in the absence of primary sclerosing cholangitis or hepatitis C infection. Under such circumstances its presence does not adversely influence treatment outcome. Primary sclerosing cholangitis cannot be excluded by routine examinations and its presence is associated with a poor treatment response. Cholangiography should be considered in all patients with autoimmune hepatitis and colitis, especially in those recalcitrant to therapy.
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PMID:Frequency and significance of chronic ulcerative colitis in severe corticosteroid-treated autoimmune hepatitis. 150 Jun 96

We present data on 10 patients (5 men and 5 women, aged 21-56 yrs) with end-stage liver disease or tumour who underwent orthotopic liver transplantation at Groote Schuur Hospital between October 1988 and June 1991. Standard surgical techniques were used for procuring the donor liver, the recipient hepatectomy and the implantation of the liver. The venovenous bypass method was used in all but 2 patients. Postoperative immunosuppression was usually achieved with cyclosporin, azathioprine and low-dose steroids. Six patients were treated with prophylactic OKT3. Rejection episodes were treated with bolus doses of intravenous steroids. The indications for liver transplantation included chronic active hepatitis progressing to cirrhosis (5), biliary cirrhosis in association with inflammatory bowel disease (1), sclerosing cholangitis (2), alpha 1-antitrypsin deficiency (1), and tumour (1). All patients with chronic liver disease had experienced at least one complication, examples of which included encephalopathy, bacterial peritonitis, ascites, variceal bleeding and septicaemia. Serious postoperative complications included acute rejection of the transplanted liver, renal and liver failure that responded to intensive care support and medical management. One patient died on the 11th postoperative day with complications of bleeding oesophageal ulcer, shock and fungaemia. The remaining patients are alive and well 1-31 months after transplantation.
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PMID:Orthotopic liver transplantation at Groote Schuur Hospital. 150 34

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

In a 4-year review of 509 patients with chronic pancreatitis, the incidence of clinically manifest fixed common bile duct (CBD) stenosis was 9% (45 patients). In 76% this was alcohol related, and pancreatic calcification was present in 51%. All patients presented with unrelenting jaundice and five (11%) had cholangitis. The mean serum bilirubin (165 +/- 108, normal 0-17 mumol/l), alkaline phosphatase (1790 +/- 1143, normal 73-207 U/l) and gamma glutamyl transferase (798 +/- 660, normal 7-64 U/l) were markedly raised. Diabetes occurred in 8 (18%). A biliary drainage operation was performed in 43 patients and 11 had concomitant pancreaticojejunostomy. Endoscopic retrograde cholangiopancreatography (ECRP) provided valuable information preoperatively in outlining both biliary and pancreatic disease in selecting patients for dual ductal drainage. Minor complications not related to biliary anastomosis occurred in 14%. Four patients died (9%), two from pseudocyst-related haemorrhage. Jaundice was successfully relieved in all and did not recur during follow-up. No secondary biliary cirrhosis was encountered, but varying degrees of portal fibrosis were present in 75% of liver biopsies. The commonest biliary pathogen was E. coli. It is recommended that a biliary bypass operation be performed when the diagnosis is radiologically confirmed and no improvement occurs within 1 month.
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PMID:Chronic pancreatitis with biliary obstruction. 156 30

One hundred and four adult patients with cystic fibrosis were evaluated for the presence of liver disease as defined by abnormal liver function tests of six months' duration, histological evidence of fibrosis or cirrhosis, or the presence of portal hypertension, or both. Twenty patients fulfilled these criteria and were evaluated further for the presence of biliary tract abnormalities with biliary scintigraphy using 99Tc diisopropylphenyl-carboxymethyl iminodiacetic acid (DISIDA) and endoscopic retrograde cholangiography. Clearance of 99Tc DISIDA from the liver and biliary tree was diminished at 45 (E45) and 60 (E60) minutes in the patients with liver disease compared with those without liver disease; E45 = 37.8% and 65.8%, p less than 0.01; E60 = 48.2% and 77.5%, p less than 0.01 respectively. Serial analogue images of the extrahepatic biliary tree were consistent with common bile duct obstruction with retention of DISIDA and tapering of the common bile duct in seven of 18 patients with and two of 10 patients without liver disease. Endoscopic retrograde cholangiography showed changes consistent with sclerosing cholangitis, with beading and stricturing of the intrahepatic ducts in 12 of the 14 patients. In all 14 patients, including those in whom biliary scintigraphy had suggested obstruction, no abnormality of the common bile duct was identified. These results indicate that abnormalities of the bile ducts in patients with cystic fibrosis related liver disease are confined to the intrahepatic biliary tree and that common bile duct strictures do not contribute to either the progression or development of liver disease in these patients.
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PMID:Biliary complications of cystic fibrosis. 156 61

Hyperplastic changes of intrahepatic peribiliary glands have rarely been reported, with the exception of hepatolithiasis. To determine whether there are any hyperplastic changes in the glands in livers without hepatolithiasis, we examined 1,000 consecutive autopsy liver specimens that had no hepatolithiasis. The glands were divided into intramural mucous glands and extramural seromucous glands. The hyperplastic changes were found in "normal" livers and in livers with various hepatobiliary diseases, and they were classified into three categories: hyperplasia of intramural glands (49 cases; 4.9%), hyperplasia of extramural serous acini (35 cases; 3.5%), and hyperplasia of extramural mucous acini (92 cases; 9.2%). Two or more of these three hyperplastic changes occasionally coexisted in the same liver. Hyperplasia of intramural glands was seen rather evenly in normal livers and in livers with various hepatobiliary diseases. Prevalence of hyperplasia of extramural serous acini was high in intrahepatic cholangitis and submassive hepatic necrosis. Prevalence of hyperplasia of extramural mucous acini was high in cirrhosis, submassive hepatic necrosis, cholangitis, systemic infection, and extrahepatic biliary obstruction. The hyperplastic intramural glands and mucous acini of extramural glands contained more neutral, carboxylated, and sulfated mucin than normal glands. Although their pathogenesis is unclear, these hyperplastic changes may enhance seromucous secretion into biliary lumens and may lead to biliary dysfunctions such as retardation of bile flow and increased bile viscosity. These hyperplastic changes may be preexisting conditions predisposing to hepatolithiasis.
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PMID:Pathologic observations of intrahepatic peribiliary glands in 1,000 consecutive autopsy livers: IV. Hyperplasia of intramural and extramural glands. 156 45

Ultrasonic Doppler measurement of the blood flow in the portal vein and hepatic artery was conducted to evaluate the function and functional reserve of the liver in 146 patients with various forms of cholangitis combined with biliary cirrhosis and hepatic insufficiency. The functional reserve of the liver was judged by comparison of the basic blood flow on a fasting stomach with the blood flow after a functional histamine load. Five types of responses of the portal vein blood flow to the functional load according to the degree of disturbed hepatic function were revealed. Comparison of the flow of blood along the portal vein in healthy individuals with that in patients with diabetes mellitus and a formed splenorenal shunt showed that disconnection of the blood flow from the splenic vein has no effect on the flow of blood in the portal vein. The latter is regulated at the level of microcirculation in the liver, which is confirmed by the correlation between the blood flow in the portal vein and in the hepatic artery.
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PMID:[Doppler ultrasonic evaluation of hepatic functional reserve]. 157 35

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

Liver tumours frequently present at a late stage and only a minority of patients are likely to benefit from resection or transplantation. Inoperable tumours carry a grave prognosis. External beam irradiation of the liver is dose-limited by the radiosensitivity of hepatocytes, particularly in the presence of cirrhosis, but internal radiation using radio-isotope sources can achieve more selective irradiation of the chosen field. Sealed sources are dose-limited by their effects on surrounding tissues, whereas with unsealed sources the dose of radio-isotope administered is limited by bone marrow suppression. Iridium-192 wires are most frequently employed as a sealed intracavitary source. They may be inserted surgically, transhepatically or endoscopically. Doses of up to 60 Gy can be delivered to a malignant biliary stricture without damage to the surrounding parenchyma. The incidence of cholangitis is low if treatment is administered after insertion of an endoprosthesis. Unsealed radio-isotope sources may be injected directly into the tumour, administered embolically via the hepatic artery in the form of microspheres or lipid droplets, or given via parenteral infusion attached to tumour-specific antibodies. Of these vehicles, the lipid agent Lipiodol appears to be the most effective and can deliver a potentially lethal dose of radiation to small tumours. Host reaction to the injected antibody remains a major drawback to the use of monoclonal antibodies as targeting agents. Iodine-131 is a beta- and gamma-emitter, producing a local tumoricidal effect and allowing accurate dosimetry by means of external scintigraphy. Yttrium-90 is a pure beta-emitter with a greater maximum beta energy and cytotoxic range; however, it is retained in bony tissues, resulting in a dose-related risk of marrow suppression. Bone absorption cannot be measured by external imaging owing to the absence of gamma emission. This lack of accurate dosimetry, coupled with the toxic side-effects of yttrium treatment, make iodine-131 the current isotope of choice.
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PMID:Therapeutic aspects of radio-isotopes in hepatobiliary malignancy. 164 96

Two Rhesus monkeys infected with simian immunodeficiency virus for 15 and 24 months developed generalized oedema and one became jaundiced. At necropsy, the liver and pancreas were hard and irregular and the gall bladder was thickened. Histopathological examination showed extensive fibrosis of the pancreas, loss of exocrine acini and marked proliferation of ductules. Numerous cryptosporidia were present on the duct epithelium. The liver of both animals had widespread cirrhosis, bile duct proliferation and cholangitis. Cryptosporidia were found in many bile ducts and on the hyperplastic gall bladder epithelium. Lymph nodes and spleen of both animals showed depletion of cortical and paracortical elements characteristic of advanced immunodeficiency virus infection.
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PMID:Chronic pancreatitis and biliary fibrosis associated with cryptosporidiosis in simian AIDS. 177 Jan 78


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