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

The chronic pancreatitis population of Wadsworth VA Hospital over the past five years was screened for two-fold or greater alkaline phosphatase elevation at any time during their course, as a marker for either distal common bile duct stenosis or other hepatobiliary disease. Forty-seven of 207 patients screened met this criterion and are reviewed in detail. Of the 16 patients with persistent alkaline phosphatase elevation (group B), 15 had proven common bile duct stenosis, demonstrating a clear pathophysiologic role of partial bile duct obstruction in their liver disease. Three had developed secondary biliary cirrhosis, marking this entity the commonest cause of secondary biliary cirrhosis at our hospital. Of the remaining 31 patients with transient alkaline phosphatase elevation (group A), only 4 had proven duct abnormalities which may resolve during recovery. Alcoholic liver disease was demonstrated with normal extrahepatic ducts in the remainder in group A adequately studies. Persistent greater than two-fold alkaline phosphatase elevation in pancreatitis thus represents a reliable marker of distal common bile duct stenosis, whose sequelae may include cholangitis and secondary biliary cirrhosis and which requires operative intervention in these cases. When a persistent alkaline phosphatase elevation greater than two-fold is encountered in a chronic pancreatitis patient, adequate cholangiography and liver histology are both necessary to confirm and grade this frequent and treatable complication.
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PMID:Common bile duct stenosis from chronic pancreatitis: a clinical and pathologic spectrum. 51 65

The authors report on their experience of 158 liver transplants performed on 135 patients. Nineteen underwent re-transplantation and four of these required a second re-transplantation; total number of re-transplantations: 23 (14.6%). Hepatic cirrhosis was the most common indication (50.6%), of which alcoholic cirrhosis was the most common type (32.5%). The authors briefly report on their operative techniques and the results of their procedures. The operative mortality (30 days) was 13.3% (18 out of 135 patients). Complications included nine cases of hepatic artery thrombosis (5.7%), four of arterial stenosis (2.5%), one case of portal venous stenosis (0.63%), four cases of post-operative portal venous thrombosis (2.5%), seven of biliary fistula (4.4%; five following choledochocholedochostomy and two following choledochojejunostomy), and two cases of common bile duct stenosis (1.3%). The actuarial survival rate at 48 months is 80%.
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PMID:Results of orthotopic liver transplantation: a personal experience. 145 19

The biliary complications of pancreatitis include cholestasis, secondary biliary cirrhosis, cholangitis, and pseudocyst or fistula affecting the hepatobiliary system. Of these, the most relevant for radiologists is cholestasis caused by biliary duct stenosis in an inflamed pancreatic head. Radiologic assessment of these complications is based on judicious use of ultrasound, computed tomography, and direct cholangiography. The typical imaging finding of common bile duct stenosis due to chronic pancreatitis is gradual tapered narrowing of the intrapancreatic common bile duct, which can be portrayed by carefully accomplished computed tomography, and ultrasound as well as cholangiography. When combined with clinical assessment, imaging tests can help determine strategies for treatment, which include traditional operations as well as transhepatic, endoscopic, or percutaneous interventions.
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PMID:Biliary complications of pancreatitis. 264 80

Thirty nine patients undergoing surgery for chronic pancreatitis were investigated for evidence of hepatobiliary disease. In addition to pre-operative assessment by liver function tests, ultrasound, ERCP (in 33) and percutaneous transhepatic cholangiography (in five), all had peroperative liver biopsy. Common bile duct stenosis was diagnosed in 16 (62%) of the 26 patients with successful cholangiography. Features of extrahepatic biliary obstruction were found on biopsy in 11 patients, three of whom showed features of secondary sclerosing cholangitis. No patients had secondary biliary cirrhosis. Three had parenchymal liver disease (cirrhosis, resolving hepatitis and alcoholic hepatitis respectively) and two others had features suggestive of previous alcohol-induced injury. Five (83%) of the patients with clinical jaundice had biopsy features of extrahepatic biliary obstruction, as did eight (67%) with alkaline phosphatase above twice normal and seven (44%) with radiological common bile duct stenosis. Neither alkaline phosphatase rise, nor common bile duct stenosis alone or in combination, were a reliable indication of the need for biliary enteric bypass surgery. Pre-operative liver biopsy may be a valuable adjunct in the assessment of such patients.
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PMID:Hepatobiliary complications in chronic pancreatitis. 271 85

Sixty patients with chronic alcohol-induced pancreatitis with endoscopic retrograde cholangiopancreatography evidence of common bile duct stenosis were studied to determine the clinical spectrum and natural history of this complication, as well as the indications for biliary bypass. In 17% of patients, common bile duct stenosis (CBDS) was an incidental finding at ERCP, while in the remaining cases pain and jaundice were the predominant symptoms in 35% and 48%, respectively. Biliary drainage was performed in 38% of patients for persistent or recurrent jaundice, cholangitis, and while undergoing pancreatic duct or cyst drainage procedures for pain. The benign nature of CBDS in chronic alcohol-induced pancreatitis (CAIP) in patients without persistent jaundice is emphasized. In particular, no histologically proved cases of secondary biliary cirrhosis were noted. The majority of patients with CBDS due to CAIP may be safely managed without biliary bypass but require close follow-up.
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PMID:The spectrum and natural history of common bile duct stenosis in chronic alcohol-induced pancreatitis. 281 30

Distal common bile duct stenosis was observed in 16 (9%) of 170 alcoholic patients admitted to a Veterans Administration Medical Center in the last five years. The following clinical and biochemical features were significantly more common (P less than 0.05) among the 16 patients with common bile duct stenosis than in 154 without: jaundice, cholangitis, hyperbilirubinemia, alkaline phosphatasemia, pancreatic calcification, and malabsorption. Surgical decompression of biliary tree was necessitated in 13 of 16 cases due to obstructive jaundice in seven, cholangitis in four, portal fibrosis in one, and persistent abdominal pain in one. The mean (+/- SE) time interval between initial serum alkaline phosphatase elevation and surgical intervention was 308 +/- 108 days. Liver histology in eight cases was remarkable for portal fibrosis in seven and biliary cirrhosis in one. These data suggest that distal common bile duct stenosis is a progressive lesion which is quite prevalent in patients with advanced pancreatic disease of alcoholic etiology.
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PMID:Prevalence and natural history of distal common bile duct stenosis in alcoholic pancreatitis. 647 79

The liver histopathology in 40 liver biopsies from 24 patients with verified chronic common bile duct stenosis due to chronic alcoholic pancreatitis has been reviewed code-blinded. This represents an 8% prevalence of this complication in approximately 300 patients with alcoholic pancreatitis screened biochemically for alkaline phosphatase greater than two-fold for less than 1 month. The majority were anicteric with no symptoms other than from acute exacerbations of chronic pancreatitis. Biliary obstructive liver histopathology of varying severity was diagnosed in 19 patients (79%), seven of whom (29%) had secondary biliary cirrhosis. In 3 of these 7 cases, progression to biliary cirrhosis was documented with sequential biopsies. The remainder demonstrated this histologic picture when first diagnosed, supporting this insidious nature of this process. Stromal edema of the portal tracts, increased portal connective tissue, and marked proliferation of interlobular bile ducts and ductules were the most striking histologic features. Histologic cholangitis, although frequent, was generally mild or absent, reflecting the incomplete nature of the duct obstruction. Features of alcoholic liver disease were observed in only two cases. The results indicate that (1) chronic alcoholic pancreatitis with incomplete duct obstruction frequently causes secondary biliary cirrhosis, (2) significant alcoholic liver disease very infrequently coexists with persistent common bile duct stricture from alcoholic pancreatitis, and (3) surgical biliary decompression should be considered in any patient with documented persistent common bile duct stenosis from alcoholic pancreatitis.
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PMID:Liver histopathology in chronic common bile duct stenosis due to chronic alcoholic pancreatitis. 728 90

In the course of alcoholic chronic pancreatitis, increased serum alkaline phosphatase level is usually caused by common bile duct stenosis but may also be due to alcoholic liver disease. The aims of this prospective study were to investigate whether clinical, biochemical and radiological factors could predict liver histopathological appearance. The study comprised 48 patients with chronic alcoholic pancreatitis, common bile duct stenosis and increased serum alkaline phosphatase levels; clinical, biochemical, radiological and histological data were recorded in all cases. Liver biopsy examination (surgical [n = 45] or intercostal [n = 3]) showed (a) biliary obstructive liver abnormalities (n = 33), which were severe in 20 cases (biliary fibrosis in 15, secondary biliary cirrhosis in 3, secondary sclerosing cholangitis in 2) and moderate in 13 cases; (b) alcoholic liver disease in 9; and (c) normal liver in 6. Clinical, biochemical and radiological data were not statistically different between patients with biliary obstructive liver disease and those with alcoholic liver disease. Forty-five patients underwent surgery; two patients with alcoholic hepatitis died after surgery, at the beginning of this study. We conclude that in chronic alcoholic pancreatitis with common bile duct stenosis and increased serum alkaline phosphatase levels, clinical, biochemical and radiological data cannot be used to predict the type of liver lesions. Therefore liver biopsy is warranted to identify (a) alcoholic hepatitis, which increases operative risk; and (b) biliary obstructive liver disease, frequent and often severe, in which surgical biliary decompression should be considered.
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PMID:Factors predictive of liver histopathological appearance in chronic alcoholic pancreatitis with common bile duct stenosis and increased serum alkaline phosphatase. 822 11

Proliferation of preexisting bile ducts, ductular metaplasia of hepatocytes and proliferation and differentiation of liver stem cells are discussed in the pathogenesis of neoductular structures in the liver. Under the condition of experimental bile duct obstruction and in extrahepatic bile duct stenosis neoductular structures are first the result of proliferation and sprouting of preexisting ducts and cholangioles. Especially in later stages of cholestasis but also in other chronic progredient liver diseases such as chronic alcoholic liver disease and chronic active hepatitis periportal hepatocytes may show a phenotypic shift towards ductular epithelia. In postnatal liver diseases hepatocytes first express keratin 7 and later keratin 19 during ductular transdifferentiation. This is in contrast to embryonal cholangiogenesis. In alpha-1-antitrypsin-deficiency, hemochromatosis, Wilson's disease, and chronic active hepatitis B cellular deposites typically located in hepatocytes such as alpha-1-AT, siderin, copper, HBs-Ag, and HBc-Ag can also be found in neoductular cells close to hepatocytes. These deposites seem to be retained during the ductular transdifferentiation of hepatocytes. Expression of bile duct-type integrin subtypes and TGF beta 1 in neoductular cells are involved in the changing parenchymal/mesenchymal interplay during neoductogenesis, resulting in periductular basal membrane and periductular fibrosis. In FNH the ductular transdifferentiation of hepatocytes is integrated in the histogenesis of micronodules and portal tract equivalents of these tumor-like lesions. Ductular structures in hepatoblastomas and especially in combined hepatocellular and cholangiocarcinomas (CHCC) may reflect the common embryologic derivation of hepatocytes and biliary epithelia. Non-neoplastic liver tissue in resection specimens of our CHCC showed a lower rate of cirrhosis, and a significantly higher Ki 67-LI of neoductular cells compared to liver tissue in resection specimens of HCC and liver metastases. 3 of 10 CHCC had developed in alpha-1-AT-deficiency, in which this protease-inhibitor was predominantly retained in periportal hepatocytes. These findings in non-neoplastic tumor-bearing liver tissue suggest that CHCC include a special histogenic type of primary liver carcinoma which in analogy to some experimental liver tumors might develop from periportal parenchymal cells.
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PMID:[Hepatic neoductules]. 860 Jun 93

Urea cycle defects (UCDs) typically present with hyperammonemia, the duration and peak levels of which are directly related to the neurologic outcome. Liver transplantation can cure the underlying defect for some conditions, but the preexisting neurologic status is a major factor in the final outcome. Multicenter data indicate that most of the children who receive transplants remain significantly neurologically impaired. We wanted to determine whether aggressive metabolic management of ammonia levels after early referral/transfer to a metabolism center and early liver transplantation would result in better neurologic outcomes. We report on 5 children with UCDs, ie, 2 male patients with X-linked ornithine transcarbamylase deficiency and 2 male patients with carbamoyl phosphate synthase deficiency, all of whom had neonatal presentations and underwent orthotopic liver transplantation before 1 year of age, and 1 female patient with partial X-linked ornithine transcarbamylase deficiency that was intractable to medical therapy, who underwent transplantation at 35 months of age. Developmental testing with the Griffiths scale was performed on 3 occasions each, 12 months apart, up to 45 months after transplantation. Full-scale indices for 3 children who underwent early transplantation showed average developmental quotients of 67. All 5 children had metabolic cures. There were no deaths (30-month survival rate: 100%). One child is currently listed for repeat transplantation because of bile duct stenosis and cirrhosis. We conclude that early liver transplantation and aggressive metabolic management improve early neurologic outcomes for children with UCDs, but longer follow-up monitoring is needed.
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PMID:Developmental outcomes with early orthotopic liver transplantation for infants with neonatal-onset urea cycle defects and a female patient with late-onset ornithine transcarbamylase deficiency. 1546 81


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