Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An Arab female child presented with rapidly progressive liver disease, with apparent onset in late infancy and death at 15 months. Microscopy showed panacinar hepatitis, portal and pericellular fibrosis, and diffuse Mallory bodies in the absence of steatosis or significant cholestasis. Hepatic copper concentration was moderately elevated. Known causes of early childhood cirrhosis were excluded. This case meets most of the established criteria of Indian childhood cirrhosis, yet is unusual in its occurrence in a child of Arab ancestry and in having a moderate degree of hepatocellular copper overload.
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PMID:Indian childhood cirrhosis--like liver disease in an Arab child. A brief report. 818 Jul 83

The concentration of biliary Cu was 0.12 +/- 0.01 microgram/ml in male LEC rats aged 14 weeks and 0.43 +/- 0.09 micrograms/ml in Fischer rats of the same age. When copper chloride (170 micrograms/kg b.w. as Cu) was infused intravenously (i.v.), the concentration of biliary Cu increased to only 0.21 +/- 0.06 microgram/ml 30 min after the infusion in LEC rats. In contrast, Fischer rats showed a concentration about 10 times higher (4.02 +/- 2.2 micrograms/ml) than that before the infusion. In Fischer rats pretreated with cadmium chloride, the biliary Cu concentration was 1.04 + 0.43 micrograms/ml 30 min after infusion of copper. Horseradish peroxidase (E.C.1.11.1.7) infused iv along with copper chloride was excreted into bile at a low level in LEC rats compared to Fischer rats. Our results suggest that the gross accumulation of hepatic Cu in the new, mutant LEC rats is due to a low excretion of Cu into bile and that the hepatobiliary dysfunction is related to spontaneous hepatitis.
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PMID:Decrease in biliary excretion of copper in Long-Evans cinnamon (LEC) rats causing spontaneous hepatitis due to a gross accumulation of hepatic copper. 821 Jun 89

The LEC rat is an inbred mutant strain with spontaneous hepatitis isolated from Long-Evans rats. Since approximately 40% of LEC rats die of fulminant hepatitis, the rat serves an animal model for studying the pathogenesis and treatment of human fulminant hepatitis. The remaining 60% of LEC rats survive and develop chronic (prolonged) hepatitis and subsequently develop liver cancer. Therefore, the LEC rat serves an important animal model for studying the significance of chronic hepatitis in the development of human liver cancer, which often develops in association with chronic hepatitis. The LEC rat can also be used as an animal model of Wilson's disease, since recent studies have disclosed high copper accumulation in the liver and low ceruloplasmin concentration in the serum of this mutant rat.
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PMID:The LEC rat: a model for human hepatitis, liver cancer, and much more. 829 9

Copper (Cu) accumulating bound to metallothionein (MT) in the liver of LEC (Long-Evans with cinnamon-like coat color) rats due to a hereditary metabolic disorder is assumed to lead to acute hepatitis with severe jaundice. The metal was shown to be present in the liver in a form not bound to MT at the beginning of hepatitis after first delivery and lactation. Following this change in the distribution of Cu from MT-bound to non-MT bound form in the liver, changes in the concentrations and distributions of Cu, zinc (Zn) and iron in the plasma and kidneys of LEC rats were also observed. Cu plasma distribution on a gel filtration column by HPLC-ICP revealed that the holo-form of ceruloplasmin (Cp) was present before hepatitis and increased with its development, indicating the availability of Cu for Cp by hepatitis. Cu-binding proteins migrating at the same retention times as those of hepatic Cu-MT and Cu,Zn-superoxide dismutase (SOD) were detected in plasma during hepatitis. Albumin was largely present in the form of nonmercaptoalbumin, reflecting that the bloodstream was under oxidative stress. A sudden increase in the concentration of Cu in the kidneys occurred with hepatitis, and the metal came to be distributed more to high molecular weight proteins with its development.
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PMID:Changes in copper distribution in the plasma and kidneys of LEC rats following acute hepatitis. 830 90

Long-Evans Cinnamon (LEC) rats, a mutant strain originating from Long-Evans rats, spontaneously develop hereditary hepatitis followed by hepatocellular carcinoma. The hepatic disorder in LEC rats is associated with their abnormal copper metabolism; metal-catalyzed reactions often give rise to oxygen radicals, which may be related to the carcinogenesis. By means of high-pressure liquid chromatography with electrochemical detection, cellular DNA damage caused by oxygen radicals can be assessed in terms of the amount of 8-hydroxydeoxyguanosine (oh8dG). We assayed the amount of oh8dG in DNA of liver, kidneys, and brain of LEC and Long-Evans Agouti (LEA) control rats in seven groups (n = 3 to 6) aged from 5 weeks to 24 months. Control rats, a healthy sibling line, were age-matched. The amount of oh8dG was correlated with the severity of the age-related clinical symptoms in LEC rats. The amount was higher in LEC rats than in the controls, especially in the liver at the acute stage of hepatitis. These findings suggest that oxygen radicals may be important in the carcinogenesis that occurs in LEC rats.
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PMID:Elevated level of 8-hydroxydeoxyguanosine in DNA of liver, kidneys, and brain of Long-Evans Cinnamon rats. 832 Jan 67

Distribution of metallothionein (MT) and copper ion (Cu) in the liver of LEC (Long-Evans Cinnamon) rats was investigated to examine the relationship between Cu-MT induction and the development of hepatitis followed by hepatocellular carcinomas. Immunohistochemical studies on MT in the liver of LEC rats indicated that MT is accumulated in nuclei and cytosols. Both MT and Cu, estimated by radioimmunoassay and flameless atomic absorption spectrometry, respectively, in subcellular fractions of the liver were found to be concentrated highest in cytosols, followed by nuclei, mitochondria and microsomal fractions. Gel-filtration (Sephadex G-75) studies demonstrated that MT is induced as the Cu-MT form. Furthermore, the Cu-MT fragment purified by the gel-filtration contains the Cu(I)-MT form, as demonstrated by ESR (electron spin resonance) measurements at 77K. These results will be important for understanding the development of hepatitis in LEC rats.
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PMID:Copper-metallothionein distribution in the liver of Long-Evans cinnamon rats: studies on immunohistochemical staining, metal determination, gel filtration and electron spin resonance spectroscopy. 838 94

The LEC (Long-Evans cinnamon) rat is a mutant strain displaying hereditary hepatitis and spontaneous hepatocellular carcinoma, and shows abnormal hepatic copper accumulation similar to that occurring in Wilson's disease. We evaluated the iron metabolism of LEC rats compared to LEA (Long-Evans agouti) rats. Hepatic iron and ferritin concentrations were remarkably increased depending on age in LEC rats but not in LEA rats. Increased hepatic iron is normally associated with decreased serum transferrin and total iron binding capacity in hepatic iron overload. In LEC rats, however, both serum transferrin and total iron binding capacity increased with increasing hepatic iron. This increase of serum transferrin and hepatic iron may be an additional important factor contributing to liver injury in LEC rats.
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PMID:Abnormal hepatic iron accumulation in LEC rats. 838 76

Certain features of Wilson's disease (WD) in Asia have been found to be different from those in other continents. The higher prevalence rate in Japan is presumably due to a higher consanguinity rate. In Chinese there is a tight linkage between WD and two gene loci for esterase D and retinoblastoma in the long arm of chromosome 13. The high proportion of patients with hepatic presentation accounts for early onset of WD in the Japanese and Chinese series. Skeletal involvement, leg hyperpigmentation, dark complexion, amenorrhea, epileptic seizures, and cerebral white matter degeneration are relatively more common among WD patients in Asia. Excessive copper in the liver appears to have a protective effect against hepatocellular carcinoma and type B hepatitis. Electrophysiological studies suggest widespread functional disturbances of the CNS in WD. Side-effects from penicillamine are rather frequent and often lead to interruption of the therapy. Trien is found to be effective without adverse reactions. Oral zinc therapy may be a suitable alternative for long-term management of WD patients in developing Asian countries.
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PMID:Geographic variations in Wilson's disease. 841 43

Long-Evans Cinnamon (LEC) rats are autosomal recessive mutants that develop hepatitis and hepatocellular carcinoma. Because copper accumulates in the livers of these rats, and some of their clinical and pathological features are similar to those of patients with Wilson's disease, LEC rats are proposed as an animal model of Wilson's disease. It has been thought that unbound copper generates free radicals, which act as hemolytic and hepatocytotoxic agents. To examine the effects of vitamin E as an antioxidant on hereditary hepatitis in LEC rats, we fed 3-week-old rats for 25 weeks either vitamin E-deficient, control, or vitamin E-supplemented diets which contained < 0.01 mg of total tocopherols, 2 mg of d,l-alpha-tocopheryl acetate (2 I.U.), and 58.5 mg of d,l-alpha-tocopheryl nicotinate (50 I.U.), respectively, per 100 mg of feed. In males, body weight loss was first observed in the vitamin E-deficient group, and mean ages at which jaundice occurred were in the order: deficient younger than control younger than supplemented groups. The ages when plasma glutamic oxaloacetic transaminase and glutamic pyruvic transaminase activities began to increase sharply and peaked followed the same order. Thus, it is likely that free radicals are involved in jaundice and hepatitis in LEC male rats, and they are a model for studying the relationship of copper, free radicals, and hepatitis. Conversely, in females, no apparent differences in clinical and biochemical changes were observed among the three groups. Causes for the discrepancy between the sexes remain to be clarified.
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PMID:Effects of dietary vitamin E on clinical course and plasma glutamic oxaloacetic transaminase and glutamic pyruvic transaminase activities in hereditary hepatitis of LEC rats. 845 79

The Long Evans Cinnamon (LEC) rat spontaneously develops fulminant hepatitis, which is usually lethal due to excess copper accumulation in the liver and is considered an animal model of Wilson's disease. LEC rats show a strong appetite for proline solution. Daily oral (p.o.) administration of proline resulted in significant delay of mortality. Feeding a copper-deficient diet greatly delayed the onset of jaundice and mortality and voluntary consumption or p.o. administration of proline further delayed jaundice and prevented mortality. LEC rats also consume ascorbic acid solutions, and p.o. administration of ascorbate also results in a significant delay in the appearance of jaundice and mortality. Combined treatment with ascorbic acid and proline is additive to delay further jaundice and mortality. An endogenous antioxidant protein, thioredoxin, when infused by minipump IP, could also inhibit the incidence of jaundice. These results indicate that antioxidant treatment combined with proline may be of benefit in Wilson's disease and possibly other forms of hepatic dysfunction.
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PMID:Proline, ascorbic acid, or thioredoxin affect jaundice and mortality in Long Evans cinnamon rats. 854 67


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