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

The histological and immunohistochemical characteristics of the liver in 44 children (28 boys, 16 girls) with extrahepatic biliary atresia at different stages of the clinical course were studied. Thirty-four wedge liver biopsy specimens taken during Kasai operations (25 specimens) and relaparotomy (9 specimens) and 20 hepatectomy explants taken at the time of transplantation were examined. Routine histological stains and monoclonal antibodies against different molecular weight cytokeratins and HLA-DR were used. The histopathological changes and the pattern of cytokeratin expression observed during the course of the disease were suggestive of persistent or recurrent extrahepatic biliary obstruction that occurred despite the Kasai operation and eventually led to cirrhosis and liver failure. Quantitative studies showed a progressive loss of intrahepatic bile ducts over the time course of the disease. This destruction of bile ducts had a geographic anatomical distribution in hepatectomy specimens, and in two livers it occurred predominantly in only one lobe. This geographic distribution of the vanishing bile ducts probably indicates an unpredictable and uneven obliteration of bile ducts in the porta hepatis during portoenterostomy wound healing and scarring.
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PMID:Intrahepatic bile duct loss in biliary atresia despite portoenterostomy: a consequence of ongoing obstruction? 137 80

Liver biopsy in the diagnosis of the results of a retrospective analysis of percutaneous liver biopsy in the differential diagnosis of prolonged cholestasis in infancy are reported. We compare the clinical features, serum bilirubin levels, hepatobiliary scintigraphy and histology in two groups of patients. One group of 56 patients had extrahepatic biliary obstruction (biliary atresia: 42; choledochal cyst: 9; extrinsic obstruction: 4; Caroli's disease: 1). Another group of 54 children had intrahepatic cholestasis with patent biliary tract (hepatitis: 38; non-specific cholestasis: 14; cirrhosis: 2). The percutaneous liver biopsy was better than the other procedures to differentiate biliary atresia from hepatitis. We conclude that percutaneous liver biopsy should be carried out in children with prolonged cholestasis when other non-invasive procedures have not ruled out extrahepatic biliary obstruction and before any surgical exploration of the biliary tract is performed.
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PMID:[Role of liver biopsy in the diagnosis of prolonged cholestasis in infants]. 143 8

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

Medical emergencies involving the liver and biliary tract are common clinical problems. If it is already known that the patient has cirrhosis it may be an easy matter to identify the cause of complications such as gastro-intestinal bleeding or coma, but it must be borne in mind that oesophageal varices are not the only cause of such bleeding in cirrhotics and that hepatic encephalopathy is not the only cause of coma. Bacterial infection should always be considered as a possible cause of deterioration in the clinical picture; it may be a complication of pre-existing acute or chronic liver or biliary tract disease or a cause of hepatobiliary disease; prompt administration of appropriate antibiotics may save the patient's life. If there is any suspicion of biliary obstruction in a patient with signs of bacteraemia the biliary tree should be drained without delay. The key to the management of hepatobiliary emergencies lies in prompt and appropriate supportive therapy, and then in a correct diagnosis which may allow specific treatment to be administered. However, it is often difficult to establish the cause, and the resources of a specialist centre may be needed. Prompt referral is indicated when a patient is clearly very ill and shows no signs of rapid improvement.
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PMID:Hepatobiliary disease: medical emergencies. 176 20

Twelve patients with portal hypertension caused by biliary cirrhosis were treated from January, 1983- June. 1990. There were 6 women and 6 men. Age ranged from 20 to 50 years with an average of 35.5 years. In all patients, a significant feature of this series was the long period of biliary obstruction caused by bile duct stones (N = 7), congenital atresia of biliary tract (N = 1), Caroli disease (N = 1), sclerosing cholangitis (N = 1), sclerosing carcinoma of biliary tract (N = 1), and benign postoperative stricture of biliary tract (N = 1). The incidence was 6.6% in portal hypertension; 2.2% in biliary tract disease and 18% in intrahepatic cholelithiasis. Esophagogastric varices were demonstrated in 6 cases. In 5 cases with or without esophageal varices, varices around the extrahepatic bile duct were detected before or during operation. Some complications including poor liver function, dilated collaterals around extrahepatic bile duct and biliary infection with underlying biliary stones existed simultaneously, therefore the management of these cases was very difficult. There was no single approach to patients with portal hypertension caused by biliary cirrhosis and it should be treated as an individual basis.
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PMID:Portal hypertension caused by biliary cirrhosis. 188 48

Recently it has been demonstrated that interaction of corticosteroids with extraadrenal target cells can effectively be modulated by metabolic transformation of the steroid hormone. As far as 11-hydroxylated glucocorticoids are concerned 11 beta-hydroxysteroid dehydrogenase (11 beta-HSD) is the most important enzyme charged with target cell metabolism. Inhibition of 11 beta-HSD function either by genetically transmitted deficiency or by exogenous enzyme inhibitors causes severe pathophysiological derangements, which result in a syndrome of "apparent mineralocorticoid excess". In the present paper we have tested whether or not endogenous inhibitors of this enzyme system might exist. The effects of the main naturally occurring mono-, di-, and trihydroxylated bile acids in man on 11 beta-HSD have been studied in in vitro experiments. Using rat renal microsomes it could be demonstrated that unconjugated bile acids of all three classes as well as the corresponding glycine and taurine amidates effectively inhibit oxidative as well as reductive activity of 11 beta-HSD, with lithocholic acid and chenodeoxycholic acid being the most potent compounds. It is concluded that bile acids are potent endogenous inhibitors of 11 beta-HSD and, therefore, could participate in abnormalities of cortisol metabolism observed in liver cirrhosis and extrahepatic biliary obstruction and, possibly, after ingestion of bile acids.
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PMID:Bile acids and their amidates inhibit 11 beta-hydroxysteroid dehydrogenase obtained from rat kidney. 194 47

We report the case of a 21-month-old child with a fibroinflammatory tumor of the liver causing biliary obstruction. The patient had fever, diarrhea, and jaundice. Abdominal ultrasonography, computerized tomography, and endoscopic retrograde cholangiopancreatography demonstrated the tumor to be in the porta hepatis. Growth of the mass resulted in destruction of the common hepatic duct, and chronic obstruction led to biliary cirrhosis over 4 months' time. Histologic study of the tumor revealed a fibrous stroma infiltrated by inflammatory cells. The patient was successfully treated with an orthotopic liver transplantation and is doing well 18 months following initial diagnosis without evidence of recurrence.
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PMID:Fibroinflammatory tumor of the liver causing biliary cirrhosis in an infant. 205 Dec 82

Increase in the production of hepatic lymph is a compensatory mechanism known to occur in cirrhosis, neoplasia of the liver, biliary obstruction, and right-sided cardiac decompensation with elevated hepatic venous pressure. In hepatic lymphostasis, dilated hepatic lymphatics are reflected on CT as low attenuation rims--perivascular lucencies (PVLs)--surrounding the portal veins and the intrahepatic vena cava. In this study, CT scans of the livers of normal controls and patients in each of the four disease categories mentioned above were evaluated for the presence of PVLs; the presence/absence of PVLs was correlated with other CT findings and various biochemical and pathophysiologic parameters. The PVLs were seen with some frequency in patients in each of the four disease categories (12-52%) but in none of the normal controls. In patients with tricuspid incompetence, the presence of PVLs correlated perfectly with the presence of mottled enhancement of the hepatic parenchyma (evidence of sinusoidal stasis). The presence of PVLs was associated with elevation of certain biochemical parameters, but no common biochemical marker was observed across the disease categories. Perivascular lucencies on CT reflect altered hepatic lymphatic dynamics; in the absence of impaired efferent lymph drainage, they reflect increased lymphatic flow. However, the sensitivity of the finding--the relationship of the presence/absence of PVLs on CT to the degree of alteration of the lymphatic dynamics--has yet to be assessed.
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PMID:Increased lymphatic flow from the liver in different intra- and extrahepatic diseases demonstrated by CT. 206 65

To clarify the morphology and pathogenesis of intrahepatic calculi in the incipient stage, we examined biliary sludge and microcalculi in intrahepatic bile ducts by morphologic and X-ray microanalytical methods in 18 (1.5%) among 1,179 autopsied livers. The hepatobiliary conditions of these 18 livers were intra- and extrahepatic biliary obstruction in 14 cases, hepatic fibrosis in three cases, and cirrhosis with hepatocellular carcinoma in the remaining one. Grossly, brown-pigmented microcalculi were observed floating in biliary sludge. Microscopically, the biliary sludge was composed of mucin, fibrinous materials, desquamated epithelial cells and a few bilirubin granules. The microcalculi were embedded in the sludge and consisted of mucin and precipitates of bilirubin with a granular, lamellar or amorphous appearance. Bacterial colonies were recognized in both the sludge and microcalculi in all but three cases. Intrahepatic bile ducts harboring sludge and microcalculi showed a minimal to moderate degree of glandular proliferation with mucin production. X-ray microanalysis disclosed that the sludge contained little calcium ion, whereas microcalculi were calcium-rich. These findings suggest that biliary obstruction, bacterial infection and mucin hypersecretion play an important role in the formation of intrahepatic biliary sludge and microcalculi, and that sludge is causally related to the formation of intrahepatic microcalculi. Intrahepatic microcalculi and biliary sludge may represent a pathogenetic sequence in the early stage of calcium bilirubinate hepatolithiasis.
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PMID:Biliary sludge and microcalculi in intrahepatic bile ducts. Morphologic and X-ray microanalytical observations in 18 among 1,179 consecutively autopsied livers. 209 92

Although prolonged survival after liver transplantation is now common, the condition of allografts after prolonged survival has not been widely discussed. We reviewed 86 biopsy samples from 38 patients. The samples were obtained between 366 and 1,622 days after transplant. Thirteen patients' biopsy samples were normal or showed minor changes. Six patients' samples showed rejection. Four patients, including two with rejection, demonstrated ischemic change. Three patients showed focal fibrosis, polymorphonuclear infiltration and bile duct proliferation simulating biliary obstruction, although biliary stones were found in only one patient. Three patients had acute hepatitis. Seven patients had a pattern of chronic persistent hepatitis; four had chronic active hepatitis. Follow-up biopsy samples were obtained in seven chronic hepatitis patients. Two of the patients with chronic hepatitis patients. Two of the patients with chronic active hepatitis have shown slight progression of the disease. None has progressed to cirrhosis, but neither has the chronic active hepatitis resolved. It is likely that some of these cases represent non-A, non-B hepatitis. Although histological abnormalities are common after successful transplantation, the clinical significance of many of the changes remains to be determined. Only patients with rejection or vascular thromboses required new transplants.
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PMID:The pathology of liver allografts surviving longer than one year. 222 29


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