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)

Spontaneous perforation of the biliary tract in infancy is rare; fewer than 100 cases have been reported. The authors report on two patients with spontaneous biliary perforation (SBP) who were treated during the past 5 years. One of the patients, a 10-month-old boy, presented with an extensive retroperitoneal mass, without jaundice. He was found to have a perforation in the pancreatic portion of the common bile duct (CBD). The second patient, a 6-week-old boy, had insidious onset of jaundice, abdominal distension, and acholic stools. He was found to have a perforation at the cystic duct/CBD junction and distal CBD stricture. Both patients initially underwent cholecystostomy and drainage. The second patient had a persistent external biliary fistula and high-grade stricture, and subsequently underwent CBD excision and Roux-en-Y hepaticojejunostomy, at age 12 weeks. The authors' findings do not support the widely held concepts proposing that (1) the diminished distal ductal caliber often associated with these lesions occurs secondary to the perforation rather than is a primary causative factor and (2) the distal biliary obstruction does not require treatment because it resolves with adequate drainage. In the absence of ductal abnormalities, the authors advocate external drainage. For SBP associated with an underlying stricture, prompt biliary intestinal bypass is necessary to avoid biliary cirrhosis and portal hypertension.
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PMID:Spontaneous biliary perforation: does external drainage constitute adequate therapy? 878 2

Serum alkaline phosphatase (sALP) can be separated into unbound liver type (L-ALP) and bound bone type (B-ALP) by means of WGA affinity chromatography. The L-ALP from the sera of normal adults and various liver diseases was found to show different chromatographic behaviours on DSA affinity column with multiple peaks of ALP activity after the L-ALP was treated with neuraminidase to remove the terminal sialic acids on the sugar chain of L-ALP. The L-ALP from normal sera contained no bound activity on DSA, whereas that from acute or chronic hepatisis, liver cirrhosis and biliary obstruction had a significant amount of bound fractions with weak and intermediate affinity. The strongly bound fraction(s) was only present in the L-ALP serum from primary liver cancer (PLC) and the positive rate of its appearance was 100% in 38 cases of PLC, including 8 alpha-fetoprotein (AFP) negative cases. The WGA chromatography can be skipped and similar results are obtained. The percentage of the strongly bound fraction in serum L-ALP was not related to the level of either sALP activity or AFP, and the appearance of the strongly bound fraction is attributed to the structural difference of sugar chains in L-ALP. Therefore, this L-ALP fraction may be assumed as a new index in the diagnosis of PLC, and the different profiles of sALP or L-ALP on DSA chromatography may be used in the differential diagnosis of benign and malignant liver diseases.
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PMID:Diagnosis of primary liver cancer using lectin affinity chromatography of serum alkaline phosphatase. 914 65

Quantitative analyses were performed on paediatric HIDA scans (EHIDA and DISIDA) to measure the flow rates of HIDA into and out of the liver. Analysis of the tracer outflow rate indicated that HIDA appeared to leave the liver even when there was complete biliary obstruction, implying 'leakage' from hepatocytes back into the blood. This potentially explains why a 'hepatogram' does not provide useful information about hepatic obstruction, whereas a renogram does yield useful information about renal outflow obstruction. In a small group of patients with no evidence of either hepatocellular disease or obstruction, the HIDA inflow rate into the liver was 0.003072 s(-1), which is similar to published colloid uptake rates in normal livers. This implies that although the two radiopharmaceuticals are taken up by different mechanisms, both mechanisms have a very similar extraction fraction. Patients with cirrhosis had a considerably reduced HIDA uptake rate (0.001072 s[-1]), and once again this was similar to colloid uptake from the blood in cirrhosis. Patients investigated for neonatal jaundice all showed reduced HIDA inflow, and this reduction was greatest (mean 0.000477 s[-1]) in those neonates whose jaundice was due to hepatocellular impairment. In biliary atresia, the HIDA rate was reduced to approximately 0.001040 s(-1), which was still considerably higher than the rate from patients with neonatal jaundice due to sufficient hepatocellular impairment to cause complete cholestasis.
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PMID:HIDA kinetics in children. 925 27

We determined the Guanine Deaminase (GD) activity of 200 patients with different diseases. It was found that GD activity of hepatic patients is higher than that of health adults, while the GD activity of other patients is in the normal range. There is a linear correlation between GD activity and ALT in patients with chronic hepatitis, billiary obstruction, and between GD activity and total bilirubin in patients with chronic active hepatitis, biliary obstruction and liver cirrhosis. Moreover, the GD activity of patients positive for anti-HCV is significantly increased. So GD activity in serum is a specific and sensitive index to estimating hepatic functions and can be used in the diagnosis of acute and chronic hepatitis, cirrhosis of liver, and C virus hepatitis.
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PMID:[The value of determining guanine deaminase in diagnosis of hepatic diseases]. 938 40

Liver injury due to bile duct ligation (BDL) is histologically characterized by cholestasis, bile ductular proliferation, hepatocellular damage, portal fibrosis, and ultimately biliary cirrhosis. Stem cells within the liver may act as progenitor cells for small epithelial cells termed oval cells that can differentiate into bile duct cells or hepatocytes, whereas myofibroblasts are the principal source of collagen production in fibrosis. The aims of this study were to determine 1) whether BDL induces oval cell proliferation and 2) whether blockade of Kupffer cells affects oval cell proliferation, bile duct proliferation, and myofibroblast transformation in experimental biliary obstruction. Male Sprague-Dawley rats were divided into two groups to receive either a single dose of gadolinium chloride (a selective Kupffer cell blocking agent) or vehicle. One day later, the gadolinium- and vehicle-treated groups were further subdivided to receive either BDL or sham operation. The rats were sacrificed on day 7 postoperatively. Serum was collected for measurement of aspartate aminotransferase, gamma-glutamyl transpeptidase, and bilirubin levels. Liver tissue was taken for evaluation of fibrosis, bile ductular cells, oval cells, and myofibroblasts. BDL resulted in elevated aspartate aminotransferase, gamma-glutamyl transpeptidase, and bilirubin in serum, and gadolinium pretreatment did not modify these effects. BDL induced marked oval cell proliferation, which was completely prevented by gadolinium pretreatment. Gadolinium did not affect the induction of bile duct expansion or myofibroblasts after BDL. We conclude that experimental biliary obstruction induces oval cell proliferation, which can be prevented by gadolinium pretreatment. This suggests that bile ductular proliferation and myofibroblast transformation are not mediated by Kupffer cells and that ductular proliferation can proceed in the absence of oval cells. Alternatively, gadolinium may directly affect oval cell proliferation after BDL.
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PMID:Gadolinium chloride suppresses hepatic oval cell proliferation in rats with biliary obstruction. 946 59

The authors report on an infant who had a multiloculated cystic lesion located in segment IV of the liver, consistent with Caroli's disease diagnosed, by routine prenatal ultrasound at 25 weeks' gestation, and confirmed by hepatobiliary HIDA scan and computed tomography soon after birth. Because there was no sign of biliary obstruction, the patient was observed initially, with gradual regression of the cysts noted by serial sonograms. Caroli's disease in older children and adults often is associated with recurrent cholangitis and cirrhosis, mandating resection when the disease is unilobar. However, the natural history of Caroli's disease diagnosed in utero is unclear, and a period of observation appears warranted in the asymptomatic patient.
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PMID:Regression of antenatally diagnosed localized Caroli's disease. 1099 10

Coagulopathy in patients with liver disease results from impairments in the clotting and fibrinolytic systems, as well as from reduced number and function of platelets. Parenteral vitamin K replacement corrects coagulopathy related to biliary obstruction, bacterial overgrowth, or malnutrition. Vitamin K is less effective for coagulopathy caused by severe parenchymal liver injury. Transfusion of fresh frozen plasma is the hallmark of treatment of significant coagulopathy in patients with liver disease and active bleeding. Transfusion of fresh frozen plasma also reverses moderate to severe coagulopathy of cirrhosis prior to invasive procedures. Cryoprecipitate is useful for severe coagulopathy with hypofibrinogenemia, especially when avoidance of volume overload is desired. Exchange plasmapheresis is useful in selected patients with coagulopathy due to liver disease, in whom fresh frozen plasma fails to correct coagulopathy or in patients who have coexistent severe fluid overload. Platelet transfusions, pooled or single donor, are useful in thrombocytopenic patients prior to performing invasive procedures or in the presence of significant bleeding, especially when the platelet count is below 50,000/mL. The use of recombinant factor VIIa and thrombopoietin therapy for correction of coagulopathy and thrombocytopenia, respectively, in patients with cirrhosis, is currently under investigation. Therapy with prothrombin complex concentrates, 1-deamino-8-d-arginine vasopressin and antithrombin III concentrates for the management of coagulopathy caused by liver disease can be hazardous and the use of these products is considered investigational at the present time.
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PMID:Coagulopathy of Liver Disease. 1109 2

Histologic cholestasis and clinical jaundice may be seen in all stages of alcoholic liver disease. In rare cases, isolated cholestasis without significant steatosis, hepatitis, or cirrhosis is identified in an alcoholic patient. The mechanisms of ethanol-induced cholestasis are not well studied but may involve compression of intrahepatic biliary radicals or interference with basolateral uptake and intracellular transport of bile acids. In the evaluation of the jaundiced alcoholic patient, clinical, biochemical, and radiologic data are usually sufficient to distinguish alcohol-induced liver disease from extrahepatic biliary obstruction. In cases where the diagnosis is not readily apparent, more invasive studies such as liver biopsy or ERCP may be necessary. The risk of these invasive studies is directly related to the degree of underlying hepatic dysfunction.
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PMID:Cholestasis and alcoholic liver disease. 1129 Dec 40

Since the aetiopathogenesis of primary sclerosing cholangitis (PSC) in humans remains undefined, investigators have studied a variety of animal models to gain insights into immunopathogenetic mechanisms associated with obliterative fibrous cholangitis of intra- and extra-hepatic bile ducts. To date, no animal model has been developed that exhibits all of the attributes of PSC. Rodent models instigated by bacterial cell components or colitis are promising because they may help to explain the strong association between PSC and inflammatory bowel disease (IBD). Other models of direct injury to biliary epithelia, peribiliary vascular endothelia or portal venous endothelia indicate that inflammation, chemokines and cytokines can produce diffuse sclerosis of bile ducts. Models of toxic, infectious or intra-luminal injury of the biliary tract also exhibit focal biliary sclerosis mediated by inflammation and cytokines. The histopathology of several models suggests a sequence of events beginning with secretion of proinflammatory cytokines by activated hepatic macrophages followed by peribiliary infiltration with CD4 and CD8 T cells with a T helper 1 phenotype. These results strongly suggest co-ordinated, pathogenetic roles for both the innate and adaptive immune responses. However, the stimuli that initiate and perpetuate peribiliary fibrosis remain unknown. Interestingly, several models are also associated with the development of anti-neutrophil cytoplasmic antibodies that react in a perinuclear and cytoplasmic pattern similar to that observed in patients with ulcerative colitis and/or PSC. Finally, models of extra-hepatic biliary obstruction continue to provide important information about the pathogenesis of portal fibrosis and secondary biliary cirrhosis that occurs in PSC and other diseases with obstruction of bile flow. Future studies in either existing or new animal models should advance our understanding of the pathogenesis of PSC, the major prerequisite for the development of effective therapies.
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PMID:Animal models for primary sclerosing cholangitis. 1149 70

Chronic biliary obstruction with repeated bouts of cholangitis adversely affects quality of life and may lead to secondary biliary cirrhosis with liver failure. We reviewed our experience with chronic biliary complications after surgical treatment of various diseases that at the end needed a liver transplantation. Twelve patients with previous biliary surgery developed secondary biliary cholangitis, secondary biliary cirrhosis, or both. Seven had surgery for liver hydatid disease by Echinococcus granulosus, another four had complicated biliary surgery unrelated to hydatid disease, and one had a history of a traffic accident with liver trauma and hepatectomy with chronic biliary fistula. The repeated cholangitis attacks and in two cases of hydatid disease the development of biliary-bronchial fistulas made these patients' lives miserable. All had had previous surgical procedures that made the transplantation procedure more difficult. Nevertheless, patient survival and graft actuarial survival after liver replacement were 75.0% and 69.2%, respectively, at 5 years.
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PMID:Long-term biliary complications after liver surgery leading to liver transplantation. 1159 86


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