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)

As a part of a routine yellow fever surveillance program going on in the south of Bahia State, Brazil, liver fragments were obtained through postmortem viscerotomy from 702 individuals who died after presenting acute febrile illness from 1981 up till 1991. Instead of being only screened for the presence of yellow fever, the liver tissue was thoroughly evaluated by histopathology. More than a third of the cases exhibited marked and diffuse steatosis occurring in malnourished infants and young children. Hepatic fibrosis, granulomatous disease compatible with disseminated tuberculosis, advanced schistosomiasis, chronic alcoholic injury, chronic hepatitis and cirrhosis were also frequently observed. A miscellaneous group of hepatic pathological processes were also recognized, which included such diverse entities as Hodgkin's disease, glycogenosis, sickle-cell disease, hepatocarcinoma, etc. Only 124 (17.7%) cases showed normal hepatic histology. The wide possibility of histological diagnoses strongly indicates that the material obtained by viscerotomy can be further explored by an interested pathologist, to help in the understanding of nosology and epidemiology, concerning remote geographic areas where viscerotomy is being routinely performed.
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PMID:[Hepatic viscerotomy (its contribution to the study of regional nosology)]. 752 Oct 56

We describe an Arab girl with complete absence of phosphorylase b kinase activity in the liver, symptomatic hypoglycemia, and persistently elevated serum aminotransferase values whose symptoms did not lessen with age; sequential liver biopsies showed progression to cirrhosis. Cirrhosis could not be ascribed to any other known cause. We conclude that type IX glycogenosis is not always associated with a benign outcome.
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PMID:Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver. 756 85

The clinicopathological findings of eight children with hepatic adenoma in the absence of cirrhosis are presented. The lesions ranged in diameter from 0.1 to 14.5 cm. Associated disorders were Fanconi's anemia, type I glycogen storage disease. Hurler's disease, and severe combined immunodeficiency with ADA deficiency. The remaining three children had adenoma without known associated disorders. In the children with glycogenosis and Hurler's disease the adenomas were multiple. Significant dysplasia occurred in the two children with Fanconi's anemia; however, the lesions behaved in a benign fashion--one with regression of the tumor after cessation of androgen therapy and the other with nonrecurrence after complete resection. Proliferating cell nuclear antigen (PCNA) labeling index (LI) of the adenoma arising in patients with Fanconi's anemia was significantly greater than the PCNA-LI of adenoma in the other children (mean 4.1% versus 0.9% of nuclei), approaching the lower end of the spectrum for reported hepatocellular carcinoma cases. We emphasize that the worrisome pathology that may occur in hepatic adenoma in children, particularly with Fanconi's anemia, does not necessarily predict malignant behavior. The association of hepatic adenoma with Hurler's disease or severe combined immunodeficiency has not been reported previously.
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PMID:Hepatic adenoma in the pediatric age group. Clinicopathological observations and assessment of cell proliferative activity. 757 76

Reversibility of hepatocyte functional activity is shown by cytofluorometric and microbiochemical methods in human and rat liver during postcirrhosis rehabilitation. Contents of the total glycogen and its fractions in liver cells were defined on smears of isolated hepatocytes obtained from the live puncture liver biopsies. A double increase of glycogen level is shown, in average, in hepatocytes during experimental liver cirrhosis in rats. At the same time, a relative content of the hard soluble fraction of glycogen increases by 5-8 times. The glycogen level falls to reach the norm already within one month after shutting off the pathogenic influence. However, in some animals after 6 months this level becomes even lower than the normal one. Again, the ratio between the hard soluble fraction and light one remains. In men with cirrhosis glycogenosis of hepatocytes can be expressed in greater degree (increase by 4-5 times): it depends on the illness heaviness. Further changes in glycogen content depend on the pathological process development. Under experimental cirrhosis the activity of glucose-6-phosphatase decreases by 4.3 times. Within one month after stopping the pathogenic influence the activity of this enzyme increases by 3-4 times, but later, in 6 months, it decreases to reach 55-65% of the norm. No actual changes were observed in the activities of other enzymes.
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PMID:[A cytofluorimetric study of the glycogen content and of the enzymatic activity of its metabolism in human and animal hepatocytes in liver cirrhosis and during rehabilitation]. 780 69

The most early cirrhosis is observed in newborns with neonatal hemachromatosis. Early cirrhosis occurs in hereditary tyrosinemia type I, peroxisomal diseases and glycogen storage disease (type IV). In Wilson's disease, a case complicated with cirrhosis was reported in a 4-year-old patient. Slowly progressive cirrhosis is seen in patients with familial progressive intrahepatic cholestasis. Focal biliary cirrhosis is found in cystic fibrosis of the pancreas. Moreover, many other metabolic disorders, except for urea cycle disorders, are occasionally or rarely complicated with cirrhosis. Early diagnosis and proper management could prevent the development of cirrhosis in patients with galactosemia, hereditary fructose intolerance, etc. The occurrence of hepatoma must be monitored in these patients. Liver transplantation is indicated in a part of the patients with cirrhosis.
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PMID:[Liver cirrhosis in metabolic disorders]. 811 97

Type III glycogenosis, an inherited disorder of glycogen metabolism that results from reduced or absent activity of the enzyme amylo-1,6-glycosidase (debranching enzyme), has not been frequently associated with cirrhosis and portal hypertension in adults. An adult Caucasian man with well-document type IIIa glycogenosis, who presented with a variceal hemorrhage secondary to hepatic cirrhosis, is described here. No other cause of cirrhosis was found.
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PMID:A man with type III glycogenosis associated with cirrhosis and portal hypertension. 825 64

Liver transplantation is an effective therapy for end stage liver disease. Nevertheless in many areas of the world organ availability remains a major problem. We report here the success of the first living-related liver transplantation in Africa. The left lateral lobe of the mother was transplanted orthotopically to her 6 year old child suffering from liver cirrhosis complicating glycogen storage disease. The social and medical problems encountered are discussed. Living-related liver transplantation is a viable option in countries where cadaveric organ donation is either illegal or socially unacceptable.
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PMID:Living-related liver transplantation in Africa. 835 7

We present a 26-year-old woman with glycogen storage disease type III (debranching enzyme deficiency) complicated with liver cirrhosis and hypertrophic cardiomyopathy. Glycogen debranching enzyme has two catalytic sites, oligo-1,4,-1,4- glucantransferase (EC 2.4.1.25) and amylo-1,6-glucosidase (EC 3.2.1.33). Variability in the clinical phenotype could be a function of the involvement of one or other catalytic site, or differences in tissue expression of the defective enzyme, or both. We hypothesize that some subtypes of glycogen storage disease (GSD) type III may cause liver cirrhosis as seen in GSD type IV due to the accumulation of glycogen of abnormal structure.
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PMID:A case of glycogen storage disease type III (glycogen debranching enzyme deficiency) with liver cirrhosis and hypertrophic cardiomyopathy. 855 56

Oncogenic agents may hit at least four different types of target cells in the liver, namely the hepatocytes, the cholangiolar cells, the sinusoidal endothelial and the perisinusoidal cells. All of these cell types may give rise to neoplasms which develop from phenotypically altered preneoplastic cell populations via various intermediate stages to benign and/or malignant neoplasms. The manifestation of hepatocellular neoplasms induced by chemicals, radiation or viruses in different species including primates is regularly preceded by focal metabolic and morphological alterations which emerge in the liver parenchyma long before the neoplasms appear. The predominant sequence of metabolic changes leads from a focal excessive storage of glycogen (glycogenosis) through intermediate stages, in which the glycogenosis is frequently replaced by a lipidosis, to glycogen-poor hepatocellular carcinomas. The early hepatocellular glycogenosis is due to a disturbance in glycogen breakdown, which is associated with a dysfunction of signal transduction and glucose transport. During progression from the preneoplastic hepatocellular glycogenosis to glycogen-poor hepatocellular neoplasms a fundamental shift in carbohydrate metabolism takes place, gradually redirecting metabolites such as glucose-6-phosphate toward alternative metabolic pathways such as the pentose phosphate pathway and glycolysis. Studies on about 70 resected or explanted livers from patients bearing hepatocellular carcinomas or suffering from cirrhosis provided evidence for focal changes in glycogen metabolism similar to those observed in laboratory animals. An alternative sequence of cellular changes involving oncocytes and amphophilic cell populations rich in mitochondria and sometimes also peroxisomes has been observed in rats after administration of non-genotoxic hepatocarcinogens, particularly peroxisomal proliferators, and in woodchucks during hepadnaviral hepatocarcinogenesis. Our observations suggest fundamental changes in the cellular energy metabolism during hepatocarcinogenesis, which are most probably due to a disturbance in signal transduction pathways and may be causally linked to neoplastic cell conversion.
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PMID:[Sequential cellular and molecular changes during hepatocarcinogenesis]. 860 Jun 97

Type IV glycogenosis is usually a rapidly progressive disease of early childhood, causing death before 4 years of age. It is characterized by hepatosplenomegaly, cirrhosis, and chronic hepatic failure. Muscle involvement is generally overshadowed by liver disease. A mild non-infantile variant of type IV glycogenosis has been described in a few patients. In some of them, the patients suffered foremost from chronic progressive myopathy. We here report on a female patient aged 51 years who had experienced difficulties in climbing stairs for 2 years due to leg weakness. EMG revealed a myopathic pattern. The muscle biopsy findings revealed polyglycosan bodies. Biochemical investigation showed absence of branching enzyme in muscle but not in leukocytes and fibroblasts.
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PMID:A mild adult myopathic variant of type IV glycogenosis. 866 68


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