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 evaluate whether liver metabolic zonation persists in human biliary cirrhosis, we used quantitative cytochemistry to measure activities of glucose 6 phosphatase (G6P) and NADPH dehydrogenase (ND) in hepatocytes situated in different zones of liver cirrhotic nodules. Liver specimens were obtained from 13 children with extrahepatic biliary atresia with compensated cirrhosis. Activity and distribution were compared with zonal activities measured in 17 control human liver specimens obtained during reduction hepatectomies for orthotopic liver transplantation. In normal human liver, G6P was 1.86 times more active in the periportal than in the perivenular zone. On the contrary, ND activity was lower in the periportal zone (63% of perivenular activity). A metabolic zonation persisted in extra-hepatic biliary atresia with compensated cirrhosis. G6P activity was 1.56 times greater at the nodule periphery than at the nodule center, whereas ND activity was lower at the periphery (75% of nodule center activity). This metabolic zonation is the opposite of that observed in animal toxic (CCl4) cirrhosis, in which greater G6P activity is observed at the nodule center and greater ND activity at its periphery. This confirms our previous hypothesis that the type of cirrhotic metabolic zonation may depend on the site of initial liver damage.
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PMID:Persistence of a liver metabolic zonation in extra-hepatic biliary atresia cirrhotic livers. 165 19

Among the worldwide accepted indications for liver transplantation, inherited metabolic disorders play an increasing role. In some paediatric centres this indication runs second after extrahepatic biliary atresia. The aim of liver transplantation in inherited metabolic disorders is twofold: the first is to save a patient's life, the second is to accomplish phenotypic and functional cure of his disease. These aims may be achieved in disorders presenting with cirrhosis, hepatoma, life-threatening progression or failure of other organs with preserved liver function. The timing of liver transplantation has become easier with development of surgical techniques of reduced-size donor livers. These techniques enable the performance of liver transplantation with ABO blood group compatible organs of almost any size if indicated either by deterioration of liver function or impending complications such as hepatoma or life-threatening progression. In comparison with other indications such as extrahepatic biliary atresia, postnecrotic liver cirrhosis or acute liver failure, the results of transplantation in patients with inherited metabolic disorders seem to be better, reaching up to 78-95% actuarial 1-year survival rates. However, lifelong immunosuppressive therapy is necessary. This seems to be acceptable even in disorders with only partial liver function defects.
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PMID:Treatment of inherited metabolic disorders by liver transplantation. 174 25

Forty eight needle biopsies of the liver, from children registered in the histopathological laboratory of Jos University Teaching Hospital (JUTH) were reviewed. Liver disease diagnosis was based on histopathological criteria without recourse to either clinical, biochemical or microbiological data. Hepatic Schistosomiasis (37.5%) and liver cirrhosis (25%) were the most frequently diagnosed lesions. There were only two cases of biliary cirrhosis secondary to biliary atrisia. Parasitic infestation of the liver was the most common cause of childhood hepatic dysfunction. Our results confirm the observations of workers in other tropical and subtropical regions, where infection is the commonest cause of childhood liver disease. This is in sharp contrast to the findings from European countries where neonatal hepatitis or biliary atresia are the most commonly diagnosed disorders. This retrospective study provides a good starting point for a prospective study, to determine the incidence and severity of childhood liver disease in Nigeria. This is a retrospective histopathological study aimed at establishing the pattern of liver disease in the paediatric age group in Jos. The indication for liver biopsy in all the cases was hepatosplenomegaly with or without biochemical abnormality.
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PMID:Childhood liver diseases in Jos, Nigeria: a retrospective histopathological study. 179 32

The long-term prognosis of extrahepatic biliary atresia after surgical restoration of bile flow is still controversial. An ongoing process of cirrhosis and the development of portal hypertension continue to create frequent and frustrating management problems. Clinical features, hepatic function, echotomography aspect, calcium-phosphorus metabolism and serum levels of 25-OH-D-3 were evaluated in 12 anicteric patients with extrahepatic biliary atresia successfully treated in a period from 1974 through 1987. Seven of these children had a total of 21 episodes of cholangitis. In five patients liver biopsy, obtained at the time of the external diversion closure, showed a biliary cirrhosis. Growth, development and hepatic function were normal in all children studied; one patient had esophageal varices. The serum levels of 25-OH-D3 in patients without oral supplementation of vitamin D are lower than normal. This deficit can be corrected by oral administration of vitamin D. Our study revealed that the children with successful portoenterostomy appeared to thrive normally and that they tolerated the relatively mild liver damage. We believe that Kasai operation should be done in all patients with extrahepatic biliary atresia and that the liver transplantation is to be reserved only in those with unsuccessful Kasai. In our experience external diversion was not useful to prevent cholangitis and moreover it complicates the hepatectomy in case of transplantation.
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PMID:[Long-term prognosis of patients with extrahepatic biliary atresia successfully treated with surgery. Our experience]. 194 2

Noninfectious obliterative cholangitis results from biliary tract inflammation in clinical conditions such as biliary atresia and sclerosing cholangitis. The purpose of this study was to develop an animal model of noninfectious biliary tract inflammation and fibrosis. An implantable osmotic pump was connected to a catheter placed into the gallbladder of hamsters. Phorbol myristate acetate (PMA) was infused into the biliary tract for periods of 6 hours to 28 days. After 7 days the animals developed neutrophil infiltration, cellular necrosis, and edema of the biliary ducts. After 14 days, the animals demonstrated intrahepatic cholestasis with bile duct fibrosis and acute and chronic inflammatory cell infiltration. By 28 days pronounced portal fibrosis was present, some of which created an early bridging cirrhosis pattern. In addition there was evidence of neocholangiogenesis. We conclude that long-term PMA infusion into the biliary tract generates an inflammatory response characterized by obliterative cholangitis and fibrosis, sharing many of the histologic features of human biliary atresia. This model may provide a relatively simple technique for investigating the process of nonpyogenic biliary tract inflammation.
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PMID:Experimental obliterative cholangitis. A model for the study of biliary atresia. 200 17

A young girl with biliary atresia leading to cirrhosis developed respiratory complications with hypoxemia. Intrapulmonary shunting was diagnosed with a 99mTc-MAA perfusion lung scan, which showed marked systemic activity. The shunting resolved after liver transplantation. The perfusion lung scan offers an efficient method to screen patients with cirrhosis in whom intrapulmonary shunts are suspected and to follow their progress.
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PMID:Reversal of intrapulmonary shunting in cirrhosis after liver transplantation demonstrated by perfusion lung scan. 202 96

Infants with idiopathic perinatal fibroinflammatory obliteration of the lumen of the extrahepatic biliary tree ("biliary atresia") invariably died of biliary cirrhosis before surgical techniques were devised to permit drainage of bile into the duodenum. Survival rates in operated patients now approach 75 percent at 10 years. While definitive diagnosis of biliary atresia without the use of cholangiography at laparotomy is difficult, because other disorders have similar clinical features, early diagnosis is important. The earlier surgery is undertaken, the more successful it is. With delay, irreversible changes occur in the liver that produce portal hypertension. This and liver failure eventually make liver transplantation necessary even in some operated patients. Hepatic disease associated with biliary atresia is in part due to delay in diagnosis, but complications of surgical therapy, such as ascending cholangitis, also play a role. With prolonged survival and as numbers of liver transplant recipients rise, new therapy-related complications, such as those associated with immunosuppression, will become more important in surgically treated biliary atresia.
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PMID:Biliary atresia and its complications. 218 2

Pathology of the inferior vena cava is not frequently encountered in the context of liver transplantation. Such a pathology was observed in 7.9% of our recipients, in the pre- intra- and post-operative period. Pre-existing anomalies of the IVC consisted the absence of the retrohepatic vena cava in 7 children with biliary atresia; technical adjustments were quite simple. During the operative period, dissection of the supra-hepatic vena cava was made very difficult in 2 patients with hepatic alveolar echinococcosis complicated by secondary biliary cirrhosis it was necessary to make a trans-diaphragmatic approach to the inferior vena cava. Post-operative inferior vena cava thrombosis occurred in four recipients, in three cases, it was caused by the inadequate size of the graft and major anastomotic discrepancy between the inferior vena cava of donor and recipient and in one case IVC thrombosis occurred in a context of allergy to heparin. Six of the 13 recipients with pathology of inferior vena cava died directly of indirectly because of these problems. Analysis of the causes of this pathology and their possible correction will perhaps allow better results in these patients who undergo liver transplantation.
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PMID:[Pathology of the inferior vena cava and liver transplantation]. 224 Oct 77

The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution was 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% had patency of the gallbladder and distal common duct, whereas only 8% had "correctable" biliary atresia (proximal duct patency). A variety of reconstructions were used, but the majority of patients had a Roux-en-Y portoenterostomy with or without exteriorization. Follow-up was available for 670 children (74%) with average length of follow-up of 5 years (range, 1 to 16 years). Five-year actuarial survival was 48% following Kasai's operation, but was less than 10% (at 3 years) if no operative correction was done. Survival was unaffected by sex, type of reconstruction, or cholangitis. Predictors of a bad outcome were (1) caucasian race; (2) operative age greater than 60 days; (3) presence of cirrhosis at initial biopsy; (4) totally nonpatent extrahepatic ducts; (5) absent ducts at the level of transection in the liver hilus; and (6) subsequent development of varices or ascites. Identification of factors predictive of the ultimate outcome provide a basis for either continued efforts with management of Kasai's operation or for early referral for liver transplantation.
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PMID:Biliary atresia registry, 1976 to 1989. 226 62

Between 4/1986 to 1/1989, 74 orthotopic liver transplantation were performed in 62 patients (62 first liver transplants, 10 as second graft and two as a third graft); 57 in adults and 17 in children. The main indication for the operation was liver cirrhosis (61.4%) (the most frequent etiology was alcoholic cirrhosis, 28.5%). Six cirrhotic patients had a hepatocarcinoma (9.6%). Two received a liver and kidney transplant due to terminal renal insufficiency and hemodialysis. The most frequent indication in children was biliary atresia (33.3%). Six patients had a fulminal liver failure (9.6%). AB0 blood group compatibility was identical in 87.5%, compatible in six and incompatible in three patients. Total orthotopic liver transplantation was performed in 67 patients, and size-reduced liver was indicated in 7 patients. Extracorporeal veno-venous bypass was used in adults but never in children. In 93.1% of the transplants a single hepatic artery was anastomosed to the recipient and in 6.9% a double anastomosis was performed. In 62.5% of the patients a end-to-end choledocho-choledochostomy was performed and in 34.8% hepatico-jejunostomy was indicated. Three months postoperative mortality rate was 12.9%. Arterial stenosis and thrombosis were the most frequent complication.
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PMID:[Early and late results of orthotopic liver transplantation]. 227


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