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)

The activities of several glycosidases (beta-galactosidase, beta-glucosidase, N-acetyl-beta-glucosaminidase) were demonstrated in human bile. The enzyme activities are increased about 100 times after exclusion of bile salts and other small molecular compounds by Sephadex G-50 gel filtration. The use of 4-methylumbelliferyl derivatives as substrates was useful as measurement of the bile enzyme activities are not altered in the presence of bile pigments. Enzyme characteristics of bile glycosidases were determined: pH optimum and isoelectric point. The bile glycosidase activities were also measured in various hepatobiliary disorders (cholelithiasis, cancer of gallbladder, acute hepatitis, liver cirrhosis and fatty liver). The glycosidase activities in bile from patients with liver diseases, as well as with cholelithiasis, were generally decreased. Isoelectric focusing patterns of biliary glycosidases were similar for specimens from patients with hepatobiliary disorders as compared to normal.
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PMID:Bile lysosomal enzymes: characteristics and pathological significance for various hepatobiliary disorders. 1 80

Laparoscopy was technically successful in 73 of 75 patients in whom liver disease was suspected but not accurately diagnosed after medical investigation. In 46 patients (63%) the final histological diagnosis was either diffuse parenchymatous disease (cirrhosis/hepatitis) or metastatic malignant disease; 2 had a curable medical disease (tuberculous ascites); and 6 had a curable surgical disease (5 with gallstones and 1 with an ectopic adrenal tumour). There were no deaths and no serious complications. With increasingly accurate non-invasive techniques for localising lesions in the liver, laparoscopy, with directed biopsy, should help to provide correspondingly accurate histological confirmation.
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PMID:Laparoscopy in liver disease. 5 94

Percutaneous transphepatic cholangiography patterns of intrahepatic bile ducts in 134 patients were analysed. The majority of case material consisted of gallstone disease and complications of its surgery (44 cases) and secondary or primary neoplasms of the liver (40 cases). A satisfactory filling of the peripheral bile ducts was obtained after drainage of bile and with an injection of somewhat increased quantities of contrast medium into the biliary system. PTC was found to be highly effective in detecting space occupying lesions of neoplastic origin i.e. sensitivity-90 percent, specificity-95 percent, predictive value of a positive result-88 percent. PTC patterns of liver cirrhosis with concommitant extraheptic obstruction, chronic cholangitis, congenital ectasia of the bile ducts, liver abscesses are described. Although PTC is aimed primarily at the detection of nature and level of the extraheptic biliary obstruction there is no essential reason for neglecting its possibilities in recognizing intrahepatic disease.
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PMID:[Cholangiographic appearance of the intraphepatic bile ducts (author's transl)]. 14 73

This study is based on an analysis of 1839 consecutive necropsies. Liver diseases are common in Singapore. Of particular importance are cholelithiasis, cirrhosis and primary carcinoma of the liver. Gallstones are mainly of the pigment variety and a significant proportion are present in the bile ducts. The multiracial composition of the population is reflected in a difference in the pattern of liver diseases among the different ethnic groups. Indians are found to have more alcoholic hepatitis and cirrhosis which are often of the micronodular type, while the Chinese have significantly more macronodular cirrhosis and hepatocellular carcinoma. Clonorchiasis and schistosomiasis are confined to the immigrant Chinese. The pattern of liver diseases is changing. Cholangitis and cholangitic abscesses as a result of cholelithiasis are becoming more common while amoebic abscesses and hepatic ascariasis are definitely on the decline. This is attributable to improved sanitary conditions.
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PMID:The pattern of liver diseases in Singapore. An autopsy study. 22 47

Plasma and 24-h urinary adenosine 3':5'-monophosphate (cyclic AMP) and guanosine 3':5'-monophosphate (cyclic GMP) were measured by radioimmunoassay in 12 normal subjects, 33 patients with six types of non-neoplastic disease (cholelithiasis, peptic ulcer, coronary heart disease, hypertension, regional ileitis, and cirrhosis), and 34 patients with five types of disseminated neoplastic disease (acute myelocytic leukemia; Hodgkin's disease; and metastatic cancer of the lung, colon, and breast). In patients with non-neoplastic disease, cyclic nucleotide values in plasma and urine did not differ significantly (P greater than 0.05) from those in normal subjects. In patients with disseminated cancer, cyclic AMP values in plasma and urine likewise did not differ significantly from those in normal subjects. Plasma cyclic GMP, in contrast, was significantly elevated in all five types of cancer patients, and urinary cyclic GMP was significantly elevated (five times the normal mean) in patients with acute myelogenous leukemia and Hodgkin's disease.
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PMID:Plasma and urine cyclic guanosine 3':5'-monophosphate in disseminated cancer. 22 52

Exocrine pancreatic function was determined by oral administration of N-benzoyl-L-tyrosyl-p-aminobenzoic acid (peptic-PABA-test) in 120 controls, 74 patients with chronic pancreatitis, 35 patients with acute pancreatitis 2--6 weeks after recovery, 201 patients with a variety of gastro-intestinal diseases and in 10 patients with anorexia nervosa. In the control group, 70% +/- 18% of the oral administered dose of PABA was found within 6 hours in the urine. In contrast the group of chronic pancreatic patients excreted only 40% +/- 13% over the same period. "False negative" PABA excretion was found in 11 (9%) of the 120 persons with no pancreas disease. "False positive" PABA excretion was found in 13 (17,5%) of the 74 patients with chronic pancreatitis. The test was not influenced by age or sex. After stomach resection or cholecystectomy and in patients with ulcus duodeni, chronic hepatitis, functional diarrhea, Crohn's disease, colitis ulcerosa and acute pancreatitis 2--6 weeks after recovery the peptide-PABA-test was not distored. Diminished PABA excretion was encountered in some patients with toxic liver disease, inflammatory disease of the small intensine like M. Whipple, celiac disease and unspecific enteritis and in a few patients with cholelithiasis. Low PABA excretion was found in early all patients with partial small intestinal resection, terminal liver cirrhosis or liver metastasis with ascites and in all patients with anorexia nervosa.
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PMID:[The specificity of peptide-PABA-test (author's transl)]. 31 33

Pigment gallstones are defined as any dark brown-to-black stone, consisting of calcium salts of bilirubin, phosphate, carbonate and other anions, and can be separated into carbonate- and noncarbonate-containing groups. Pigment stones predominate in the rural Orient, in cirrhosis, and in elderly United States patients undergoing cholecystectomy. Clinical associations include bile duct obstruction, stasis, and possibly hemolysis. Of pigment stones, 50% are radioopaque and account for two-thirds of all opaque stones. The concentrations of bile salts, phospholipids,, cholesterol, and total bilirubin in bile are similar to normal levels, but the concentration of unconjugated bilirubin is increased in the bile of some patients. Increased unconjugated bilirubin in bile may be caused by increased hydrolysis of excreted conjugated bilirubin. Unconjugated bilirubin is solubilized by bile salts, but the interaction is primarily nonmicellar. Ionized calcium and pH are important determinants of solubility. Sulfated glycoproteins, excreted in increased amounts in patients with cholelithiasis, may be the site of pigment stone precipitation because these compounds bind calcium salts tightly. E coli is frequently cultured from pigment stones in Japan but not in the United States; thus, bacterial beta-glucuronidase may be important in stone formation in Japan but probably not in the West. Stasis leads to increased calcium secretion and to increases in the concentration of sparingly soluble compounds that may then precipitate. Incomplete emptying of the gallbladder may result in the same concentration process. Unsaturated fats and chronic vagal stimulation cause pigment stone formation in animals. At present, surgery is the only treatment for pigment lithiasis.
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PMID:Pigment gallstones. 31 81

It is generally accepted that the bile acids are responsible for pathologies as a result of deficiency or by toxic action. Quantitative deficiency is difficult to evaluate but the normal pool of bile acids is generally considered to be between 2 and4 grams. Daily loss and replacement by synthesis is thought to be between 500 and 700 mg. There is experimental evidence to demonstrate the toxic action of certain bile acids on metabolic structures and processes. There is no doubt that alterations in the metabolism of bile acids give rise to certain pathologic aspects in some diseases of the gastrointestinal tract or the hepatobiliary system. There are other conditions, on the other hand, in which the study of these acids may reveal significant physiopathologic implications. The first group includes terminal ileopathy, blind loop syndrome, gastric ulcer, gastritis, cholestasis, cirrhosis of the liver, and cholelithiasis. In the second group are such diverse conditions as acute pancreatitis, cancer of the colon, endocrine disturbances, some hyperlipidemias, and others. Much of the present day understanding of the physiopathology of the bile acids will probably have to be revised in the nex few years, in view of the rapid advances being made in this field.
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PMID:[Bile acids II. Physiopathologic and clinical aspects (author's transl)]. 47 Apr 97

Gallstone formation is a heterogeneous disease for which supersaturation of bile with cholesterol and hemolysis of RBCs are major driving forces associated with initial formation and growth. Specific risk factors are superimposed on the gradually increasing prevalence of gallstones with age in most populations. Major risk factors associated with cholesterol gallstone formation are American Indian ancestry, female sex, obesity, and ingestion of lithogenic drugs, such as estrogen-containing preparations and clofibrate. Hemolysis and cirrhosis are risk factors for pigment stones.
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PMID:Who gets gallstones and why. 47 49

Erythropoietic protoporphyria (EEP) is the most frequently found erythropoietic porphyria in men. This inborn error of heme metabolism with autosomal dominant mode of inheritance is based on a deficiency of ferrochelatase. This defect leads to an increase for protoporphyrins predominantly in the red cells. Under the influence of sun light, especially UV-A, photohemolysis occurs and the content of protoporphyrin in the tissue increases. This is associated with acute sun burn like skin lesions, which usually clear up completely. Persistent skin lesions are seen in form of hyalinosis like infiltrations of the most intensively light exposed skin areas in some patients. Associated symptoms might be: gallstones or rarely a cirrhosis of the liver. Treatment with photoprotective ointments and especially carotinoids (beta-carotene, canthaxanthine) is very effective and only in some rare cases this symptomatic therapy fails. Prognosis of the disease is good. Only a few patients died of the associated hepatic failure.
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PMID:[Erythropoietic protoporphyria]. 49 71


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