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)

Results in 44 patients with esophageal bleeding who underwent a mesocaval shunt utilizing a prosthetic graft are presented. Portal hypertension was secondary to alcoholic cirrhosis in 30 patients, to chronic active hepatitis in eight, to primary biliary cirrhosis in four, to cirrhosis secondary to inflammatory bowel disease in one, and to portal vein thrombosis following splenectomy in one. Thirty-six shunts were performed during the emergent or semiemergent time period, and only eight were performed electively. Sixteen of the patients were Child's class A, 16 were class B, and 12 were class C. There were no hospital deaths in the emergency shunt group (of eight patients); there was a 12% mortality rate for patients undergoing semiemergency shunts (two of 17 patients) and a 42% mortality rate for patients who had emergency shunts (eight of 19 patients). Death was related more closely to hepatic reserve, however, than to timing of the shunt. Among the 32 class A and B patients, there were only three deaths in hospital (9%), as compared with seven deaths among the 12 class C patients (58%). Portal-systemic encephalopathy was high in the period immediately after operation (13 of 34 patients, 38%), but it was a chronic problem following discharge from the hospital in only three of 34 patients (9%). The mesocaval shunt is a safe, effective procedure for the control of variceal bleeding in class A and class B patients in any time period, but it carries a high operative mortality risk in the class C patient when it is performed as an emergency operation.
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PMID:Mesocaval shunts for the control of bleeding esophageal varices. 31 25

The spectrum and incidence of liver disease is described among a large series of patients with inflammatory bowel disease. The incidence of significant liver disease identified by the presence of serial biochemical abnormalities of liver function was 8.2 per cent. Transient peri-operative changes in liver function tests are common and usually relate to underlying intra-abdominal sepsis. Percholangitis, sometimes termed portal triaditis, is one of the commoner lesions, and is usually associated with extensive colitis and improves with resection of the underlying bowel disease. Cirrhosis of the liver is an important but uncommon complication and is usually associated with extensive long-standing disease. Stenosing cholangitis and biliary tract carcinoma are both important though rare associations. They are both associated with extensive disease of long-standing, but resection of the underlying inflammatory bowel disease does not necessarily protect the individual from these complications. Although stenosing cholangitis is a diffuse lesion of the biliary tree it is important to exclude strictures of the extra-hepatic biliary tree which may be amenable to surgical correction. Hepatic dysfunction is rarely the sole indication for advising surgery for the underlying bowel disease but the identification of the nature of the hepati- dysfunction provides a rational basis for such a decision and opportunities for the surgical correction of the hepatic lesion itself.
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PMID:The spectrum of hepatic dysfunction in inflammatory bowel disease. 48 86

In 45 patients with inflammatory bowel disease (9 with Crohn's disease and 36 with ulcerative colitis) and associated liver disorders, increased liver copper content (above 100 microgram/g dry weight) was found in 14 (31%). These patients represented about 50% of the patients with either biliary cirrhosis or pericholangitis. Four of the patients had levels regarded as compatible with hepatolenticular degeneration (greater than 250 microgram/g dry weight). In patients with chronic active hepatitis or non-specific changes in liver tissue, normal levels were found. The patients with Crohn's disease also had normal levels. Plasma ceruloplasmin was normal or increased in all. Determination of urinary copper output gave little diagnostic information. Alkaline phosphatases were markedly increased in most of the patients with increased liver copper concentration. In patients with ulcerative colitis and enhanced alkaline phosphatases, elevated liver copper content should be suspected and chelation therapy should be considered.
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PMID:Liver copper content in patients with inflammatory bowel disease and associated liver disorders. 53 3

Nine cases of primary sclerosing cholangitis were reviewed as to methods of diagnosis, association with other disease states and clinical course of the patients. There were four cases of primary sclerosing cholangitis occurring alone, four cases associated with inflammatory bowel disease (three with chronic ulcerative colitis and one case with proctosigmoiditis) and one case associated with porphyria cutanea tarda. All cases of primary sclerosing cholangitis occurring alone, progressed to secondary biliary cirrhosis, however, none of the cases associated with chronic ulcerative colitis progressed to secondary biliary cirrhosis. In all cases, the diagnosis was established by operative findings and biopsy results. The mode of clinical presentation was similar in all cases and was characterized by slowly progressive jaundice. Intravenous and oral cholangiography were not useful in establishing a diagnosis but endoscopic retrograde cholangiography offers preoperative diagnostic hope and use for follow-up evaluation. One case with ulcerative colitis had a Strongyloides infection and the organism was found in the fibrotic duct and pericholedochal lymph nodes. The etiological considerations are reviewed and the classification of sclerosing cholangitis associated with ulcerative colitis, as primary, is discussed. Therapeutic modalities are discussed, though therapy is mainly empirical at present.
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PMID:Primary sclerosing cholangitis. A report of nine cases and clinical review. 127 36

Primary sclerosing cholangitis (PSC) is a disease of the bile ducts which frequently is associated with inflammatory bowel disease. The disease is characterized by stenoses and saccular dilatations of the intra- and/or extra-hepatic bile ducts. Endoscopic retrograde cholangiography is the mainstay of diagnosis of this disease. A liver biopsy gives additional information and is particularly useful to rule out secondary cirrhosis. In a series of 50 patients the 5-year survival was 85%. The etiology is unknown, but in addition to inflammatory bowel disease the disease is associated with the autoimmune haplotype HLA-A1, B8, and DR3. Antibodies to perinuclear antigens in leukocytes are present in 65% of patients with PSC. Ursodeoxycholic acid therapy causes an improvement of symptoms and a decrease of elevated liver enzyme values. There are no drugs as yet with a proven effect on fibrosis or cirrhosis in this disease. Liver transplantation in this disease has a reported 4-year survival of 88%.
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PMID:Primary sclerosing cholangitis. An unresolved enigma. 129 52

Homozygous alpha 1-antitrypsin deficiency (PiZZ phenotype) is known to be associated with increased risk of cirrhosis and primary liver cancer. Although a relationship between heterozygous alpha 1-antitrypsin deficiency and chronic liver disease was suggested recently, it is still a matter of controversy whether such patients are at increased risk of liver cancer. The goal of this study was to determine the prevalence of heterozygous alpha 1-antitrypsin deficiency of different phenotypes among patients with primary hepatobiliary cancers. We studied 82 patients with primary hepatobiliary cancer; 59 had hepatocellular carcinoma and 23 had bile duct carcinoma. alpha 1-Antitrypsin quantitation and phenotyping were performed in each patient using standard methods. The distribution of the various Pi phenotypes was compared with that found in a normal population and reported elsewhere. Odds-ratio and chi 2 tests were used to measure the relative risk and the significance of association, respectively, between primary hepatobiliary cancers and heterozygous alpha 1-antitrypsin deficiency. Four patients in each of the cancer groups were heterozygous. Among the hepatocellular carcinoma patients, three had the PiMS phenotype and one had the PiMZ phenotype. Of these four heterozygous patients, only two had cirrhosis; one had cryptogenic cirrhosis and the other had hepatitis B virus-related cirrhosis. One noncirrhotic patient with a PiMZ phenotype had a fibrolamellar carcinoma. Of the four patients with bile duct carcinoma, three had the PiMS phenotype and one had the PiMZ phenotype. Of the four heterozygous patients, two had primary sclerosing cholangitis without associated inflammatory bowel disease and one patient had had previous biliary operations.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Lack of increase in heterozygous alpha 1-antitrypsin deficiency phenotypes among patients with hepatocellular and bile duct carcinoma. 131 55

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

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

Our aim was to analyze the predictive value of a variety of preoperative risk factors on operative outcomes. We reviewed all colorectal resections performed in a single hospital between January 1985 and May 1990. Nine hundred seventy-two resections were performed on 825 patients. We studied 17 preoperative risk factors generated from various medical risk categories. Using the multivariate discriminant function analysis, we calculated that 11 of the 17 risks were of significance in predicting outcomes (all with P less than or equal to 0.031). These factors included emergent operation, age greater than or equal to 75 years, congestive heart failure (CHF), prior abdominal or pelvic radiation therapy, corticosteroid use, albumin less than 2.7 g/dl, chronic obstructive pulmonary disease (COPD), previous myocardial infarction (MI), diabetes, cirrhosis, and renal insufficiency. The classification function generated by the discriminant analysis was used to categorize patients into one of four risk groups depending on their "risk score." The index used to develop each patient's "risk score" ranged from six points for an emergency operation to one point for diabetes. The mortality rates for the various risk groups were as follows: Group 1, zero to four points, 1 percent; Group 2, five to eight points, 10 percent; Group 3, 9 to 13 points, 19 percent; Group 4, greater than 13 points, 33 percent. In contrast to previous reports, we showed that age greater than or equal to 75 years alone is not a major preoperative risk factor but, rather, acts as a modifier for the other predictors of postoperative complications. We then assessed clinical questions concerning specific preoperative risks, such as steroid use, obesity, inflammatory bowel disease, COPD, and prior laparotomy, and their associated specific postoperative complications and have developed prevention strategies based on these findings. Through the use of the risk index, we also were able to assess an individual patient's operative risk more accurately.
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PMID:Multifactorial index of preoperative risk factors in colon resections. 173 12

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


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