Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085584 (encephalopathy)
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To determine the frequency and prognosis of histologic cirrhosis developing during or after corticosteroid therapy of hepatitis B surface antigen-negative chronic active hepatitis, we followed 83 patients for 90 +/- 5 mo after administration of corticosteroids. Thirty-three patients satisfied histologic criteria for cirrhosis after 30 +/- 5 mo. In 25 patients, cirrhosis developed during treatment; in 8 patients, cirrhosis eventuated after remission and cessation of therapy. The probability of developing histologic findings of cirrhosis was 59% if remission had not been achieved after 3 yr of continuous therapy. Longer requirements for treatment and deterioration during therapy characterized these patients. Once remission was achieved, the mean annual incidence of cirrhosis was only 2.6%. Patients who manifested evidence of cirrhosis in their biopsy specimens could not be distinguished by initial clinical, biochemical, or histologic findings. Ascites, encephalopathy, and esophageal varices developed infrequently; 5-yr survival after documentation of cirrhosis was 93%. We conclude that histologic features of cirrhosis develop commonly during therapy, especially if remission is not achieved quickly. After remission, cirrhosis develops infrequently. The development of histologic cirrhosis does not influence immediate morbidity and 5-yr life expectancy.
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PMID:Development and prognosis of histologic cirrhosis in corticosteroid-treated hepatitis B surface antigen-negative chronic active hepatitis. 648 94

The 15N-tracer technique gives new opportunities to medical research for investigation of normal and pathological nitrogen metabolism. Analysis of 15N kinetic data derived from total nitrogen excretion in the urine allows to calculate quantitatively certain parameters of protein synthesis and protein metabolism. At the same time analysis of serum samples will allow measurement of 15N-nitrogen incorporation into particular protein or non-protein fractions of the plasma. In the study reported here patients with different liver parenchyme lesions (acute hepatitis, chronic active hepatitis, biliary obstruction, cirrhosis of the liver, hepatic coma) were investigated with a standardized 15N-tracer method developed by us. It could be shown, that protein turnover in patients with liver insufficiency is significantly decreased as compared to healthy persons. Decrease of protein synthesis is associated with a still more pronounced decrease of protein catabolism. There are significant differences in dynamics of 15N in patients with hepatic coma as compared to other patients with liver parenchyme disease without portal encephalopathy. Nevertheless, even in hepatic coma 15N incorporation into plasma proteins can be shown. Differences in the 15N elimination kinetics of the non-protein pool between patients with chronic active hepatitis, biliary obstruction and hepatic coma could be found.
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PMID:[Protein turnover in liver failure--determination using the stable isotope 15N]. 650 88

Objections to portal systemic shunting in children with life-threatening hemorrhage from esophageal varices include the high incidence of postshunt encephalopathy with neurologic and psychiatric sequelae and the inability to provide an adequate shunt in very young children. We have operated on eight children in the past 4 years for bleeding varices. The causes were: portal vein thrombosis (3), congenital hepatic fibrosis (2), chronic active hepatitis (2), and cystic fibrosis (1). The ages at operation were between 2 and 17 years. These children underwent various modifications of an operation described by Sugiura. The operation we have developed is done through a single thoracoabdominal incision, dividing and anastomosing the esophagus with a stapler, preserving the vagal innervation to the pylorus and antrum, and wrapping the fundus around the distal esophagus at the site of the anastomosis. The venous drainage of the lower esophagus and of the upper stomach is divided. The operation is therefore shorter and simpler, but adheres to the principles enunciated by Sugiura. Complications include one significant postoperative anastomotic leak and one symptomatic esophageal stricture. Longterm results have been gratifying with no evidence of rebleeding from esophageal varices. We believe that our modification of the original Sugiura operation is the preferred therapy of bleeding esophageal varices when surgical intervention is indicated because it preserves the normal structure and function of the upper gastrointestinal tract as well as the portal venous drainage to the liver.
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PMID:A modified Sugiura operation for bleeding varices in children. 660 31

Plasma amino-acid concentrations were measured in 167 patients with liver disease of varying aetiology and severity, all free of encephalopathy, and the results compared with those in 57 control subjects matched for age and sex. In the four groups of patients with chronic liver disease (26 patients with chronic active hepatitis, 23 with primary biliary cirrhosis, 11 with cryptogenic cirrhosis, and 48 with alcoholic hepatitis +/- cirrhosis) plasma concentrations of methionine were significantly increased, while concentrations of the three branched chain amino-acids were significantly reduced. In the first three groups of patients plasma concentrations of aspartate, serine, and one or both of the aromatic amino-acids tyrosine and phenylalanine were also significantly increased, while in the patients with alcoholic hepatitis +/- cirrhosis plasma concentrations of glycine, alanine, and phenylalanine were significantly reduced. In the three groups of patients with minimal, potentially reversible liver disease (31 patients with alcoholic fatty liver, 10 with viral hepatitis, and 18 with biliary disease) plasma concentrations of proline and the three branched chain amino-acids were significantly reduced. Patients with alcoholic fatty liver also showed significantly reduced plasma phenylalanine values. Most changes in plasma amino-acid concentrations in patients with chronic liver disease may be explained on the basis of impaired hepatic function, portal-systemic shunting of blood, and hyperinsulinaemia and hyperglucagonaemia. The changes in patients with minimal liver disease are less easily explained.
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PMID:Plasma amino-acid patterns in liver disease. 707 13

We prospectively studied patients with subacute hepatic failure due to subacute hepatitis to find out 1) its relative prevalence compared to acute liver failure due to fulminant hepatitis and chronic liver failure due to chronic active hepatitis; 2) its clinical, biochemical, and morphological hepatitis; 3) the role of virus B in its etiology; and 4) its prognosis and whether there were any predictors of bad prognosis. Thirty-three patients with subacute hepatic failure were registered during a 3-year period. Persistent or progressively deepening jaundice of 8 weeks duration and development of moderate to sever ascites in patients starting otherwise typical features of acute viral hepatitis, defined the subacute hepatic failure group. The characteristic features included moderate to deep icterus, ascites, and peripheral edema; encephalopathy and gastrointestinal bleeding were infrequent. Liver function tests were abnormal but not diagnostic. Submassive and bridging necrosis of the liver were the main histological findings. Virus B etiology was recorded in 42% of the patients. Mortality was 66%. This condition is highly fatal and not infrequent in India.
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PMID:Subacute hepatic failure; is it a distinct entity? 711 11

Distal splenorenal shunt (DSRS) is a once-only form of treatment. It is suitable for many black South Africans with non-cirrhotic variceal bleeding who cannot attend repeated follow-up sclerotherapy sessions. However, persistent hyperbilirubinaemia and encephalopathy may occur following DSRS in schistosomiasis. Forty-one consecutive patients with DSRS have been treated over a 7-year period. The causes of portal hypertension were schistosomiasis (32), portal vein thrombosis (8) and diffuse nodular hyperplasia (1). Operative mortality was 6%. Encephalopathy was observed in 1 patient. Galactose elimination capacity (GEC) and technetium-diethylenetriamine penta-acetic acid hepatic perfusion index (HPI) were used to assess liver function and hepatic perfusion pre- and postoperatively, respectively, in schistosomiasis. GEC was 348 +/- 37 (M +/- SD) before, compared with 343 +/- 67 postoperatively (P = 0.78). HPI showed long-term preservation of hepatopetal portal venous flow following DSRS. Morbidity and mortality were observed only in patients with schistosomiasis associated with hepatitis B chronic active hepatitis. DSRS is ideal treatment in selected patients with non-cirrhotic variceal bleeding.
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PMID:Distal splenorenal shunt for non-cirrhotic variceal bleeding in black South Africans. 759 1

To determine the importance of hepatitis C virus (HCV) infection in the aetiology of chronic liver disease in southern India, the prevalence of HCV antibodies and HBV markers was estimated in 100 patients with chronic liver disease and in 56 patients with a variety of other gastrointestinal and liver diseases who served as controls. HCV antibody was measured by a second-generation ELISA. HBsAg, anti-HBc, anti-HBs and anti-D were also estimated. HCV antibodies were detected in 26/100 patients with chronic liver disease compared to 0/56 controls. HBV markers were present in 72 of 100 patients with chronic liver disease compared to 21/56 (37.5%) controls. Anti-D was noted in 4/100 patients with chronic liver disease and in none of the controls. Many patients had serological evidence of both B and C infection; 73% of those with anti-HCV also tested positive for HBV markers. HCV related disease presented at a median age of 60 years compared to HBV related disease which presented at a median age of 40. There was no significant difference between HCV and HBV positive patients in symptomatology, but encephalopathy was uncommon and cirrhosis the usual finding at histology in HCV positive individuals, while chronic active hepatitis was found in 30% of biopsied HBV related disease. HCV is a significant cause of chronic liver disease in this geographic region, although HBV infection continues to account for the largest proportion of cases.
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PMID:Prevalence and presentation of hepatitis C related chronic liver disease in southern India. 778 72

Dogs with portosystemic encephalopathy (PSE) are known to develop pituitary-dependent hyperadrenocorticism, but there have been no reports on the plasma protein binding of cortisol in these dogs. Since the liver is involved in the synthesis of corticosteroid-binding globulin (CBG) and other transport proteins for cortisol, the binding characteristics of these proteins and thus the biologically-active free fraction of cortisol might be altered in dogs with PSE. We investigated the total concentration of cortisol and the free fraction and the free cortisol concentration in plasma of thirty-two dogs with PSE due to inherited portosystemic shunts or chronic active hepatitis with cirrhosis. We found a significantly higher free fraction (14.7 +/- 5.8%, P < 0.0001) and free cortisol concentration (26.3 +/- 23.1 nM, P < 0.001) in these dogs than in healthy controls (8.2 +/- 2.3% and 9.2 +/- 7.2 nM, respectively). Moreover, basal concentrations of total cortisol in the dogs with PSE were higher than in the healthy controls (190 +/- 146 nM v. 107 +/- 65, P < 0.01). The per cent free cortisol in plasma was not significantly correlated with the concentration of albumin or the total cortisol in plasma. We conclude that there is decreased binding of cortisol in plasma of dogs with PSE due to decreased hepatic synthesis of cortisol binding proteins. The presence of increased concentrations of free cortisol in these dogs indicates that their basal pituitary-adrenocortical activity was increased, probably due to aberrant neurotransmission in brain centers associated with pituitary function, as a result of hepatic encephalopathy.
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PMID:Increased free cortisol in plasma of dogs with portosystemic encephalopathy (PSE). 782 25

Chronic forms of viral B,C and D hepatitis and fulminant hepatitis represent a serious healthcare problem. The study deals with the changes in the strategy in treating these diseases. During the chronic active hepatitis caused by the B hepatitis virus, the main aim of treatment is to cease multiplication of viruses, eliminate the clinical symptoms, prevent the development of cirrhosis, or the origin of hepatocellular carcinoma. The authors analyze the possibilities of the application of corticosteroids, viricidal drugs (vidarabin and interferons) and other medicaments (acyclovir, zidovudin, duramin, gancyclovir, chinacrin, and others) besides corticosteroids, interleukin 2 and tymozin from the group of immunomodulators were tested. The testing included the factor stimulating the colonies of granulocytes and myeloblasts and other substances. The therapy of acute protracted B hepatitis by means of interferon still requires controlled studies. Superinfection by D virus in chronic carriers of HBsAG causes chronic hepatitis which quickly leads to the development of cirrhosis. The therapy on basis of alpha interferon decreases the RNA virus D hepatitis serum level and leads to an improvement in the development of chronic hepatitis in half of the patients. Therapy of chronic C hepatitis on basis of corticosteroids is ineffective, and can be dangerous. Acyclovir is proved to be ineffective as well. The open study indicated certain positive results in application of interferon. The fulminant hepatitis can be defined as a development of encephalopathy and a decrease of the prothrombin time to less than 50% in the course of acute hepatitis. The break-point in the therapy of fulminant hepatitis took place in association with the performance of the transplantation of the liver. Impossibility to transplant the liver means that the effect of therapy of fulminant hepatitis is merely of supportive value. Majority of patients die due to neurologic complications, namely unmanageable oedema of the brain. But still, neither the antioedema therapy, e.g. on basis of manitol, as well as by means of corticosteroids, hemodialysis, hemofiltration, plasmapheresis and hemoperfusion, nor the treatment on basis of E1 prostaglandine improved the survival of patients. (Tab. 2, Ref. 82).
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PMID:[Treatment of viral hepatitis]. 855 59


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