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)

Cholesterol ester storage disease (CESD) is a rare congenital disorder of lipid metabolism, with mutation of the lysosomal acid lipase gene, causing chronic liver disease, usually before adolescence. We here describe three adult siblings with CESD diagnosed by light microscopic demonstration of excessive lysosomal storage of lipids with accumulation of foamy cells in liver biopsies and by a decrease in acid lipase activity (2-3% of controls). One patient (male, 46a) had extensive liver fibrosis, another (female, 58a) had cirrhosis of the liver. The third patient had died from variceal haemorrhage (female, 56a). Using sequence analysis of RT-PCR products of LAL mRNA, the patients were identified as compound heterozygotes for a G-->A substitution at position -1 of the exon 8 splice donor site and a point mutation at the second allele, resulting in a His108-->Pro shift. In two patients, therapy with lovastatin was initiated, which led to normalisation of serum cholesterol and triglyceride levels. After 12 months, liver biopsy demonstrated a significant decrease in vacuolisation of hepatocytes, with fewer and smaller droplets. Semi-automated computer-assisted image analysis of electron microscopic sections demonstrated a decrease in the hepatocellular lysosomal area from 20.5+/-7.1% to 11.7+/-6.5% (p<0.05) and 41.7+/-5.1% to 33.4+/-4.4% (p<0.01). We conclude that in two siblings with a novel LAL variant and mild phenotype of CESD, lovastatin decreased both serum lipid concentrations and hepatocellular lysosomal content.
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PMID:A novel variant of lysosomal acid lipase in cholesteryl ester storage disease associated with mild phenotype and improvement on lovastatin. 936 51

The present study was done to determine the additional influence of daily ethanol intake (15% in drinking water ad libitum) on long-term toxic effects of a single administration of dibutyltin dichloride (DBTC, 8 mg/kg b.w. i.v.) in pancreas and liver of rats. Pathohistological changes in pancreas, bile duct and liver as well as pathobiochemical parameters of pancreatitis (amylase and lipase activity), liver lesions (alkaline phosphatase activity and bilirubin) and fibrosis (hydroxyproline and hyaluronic acid) were measured 1 day and 1 to 24 weeks after DBTC- and DBTC/ethanol administration. DBTC alone induced in rats an acute interstitial pancreatitis as well as acute bile duct and liver lesions in the early experimental phase. Later on, the acute inflammatory processes in pancreas and liver took a chronic course resulting in pancreatic fibrosis and liver cirrhosis. Ethanol increased the toxic effects of DBTC on pancreas and liver during the acute and chronic course. In the acute phase lasting 1 day to 2 weeks, ethanol enhanced the DBTC toxicity on acinar cell and bilio-pancreatic duct epithelium as well as the formation of obstructive ductal plugs by necrotic cell debris. The obstruction and cholestasis in the DBTC/ethanol-group were significantly stronger as in the DBTC-group. The significant increase of hydroxyproline in urine and hyaluronic acid in serum of the DBTC/ethanol treated rats after 12 to 24 weeks was connected with a more severe chronic inflammatory fibrosis in pancreas and liver in comparison to the DBTC-treated group.
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PMID:The influence of ethanol on long-term effects of dibutyltin dichloride (DBTC) in pancreas and liver of rats. 958 82

Serum amylase and lipase concentrations were determined in 78 patients with chronic liver diseases [26 chronic active hepatitis (CAH) and 52 liver cirrhosis] and in 15 healthy subjects. Pancreatic isoamylase concentrations and macroamylase complexes were assayed in hyperamylasemic sera. Serum amylase levels were abnormally elevated in 27 patients (35%; 22 liver cirrhosis, 5 CAH), whereas serum lipase levels were elevated in 16 patients (21%; 15 liver cirrhosis, 1 CAH). In 9 of the 27 hyperamylasemic patients, the hyperamylasemia was of pancreatic type. Macroamylasemic complexes were not detected in hyperamylasemic sera. Patients with liver cirrhosis had serum levels of amylase and lipase significantly higher than both the healthy subjects and the patients with CAH, while no significant differences were found in serum levels of these enzymes in patients with CAH as compared to the healthy subjects. A decreased liver metabolism of serum amylase and lipase in patients with chronic infective liver disease, especially in those having liver cirrhosis, may lead to an accumulation of these enzymes in the blood.
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PMID:Serum pancreatic enzyme concentrations in chronic viral liver diseases. 1006 22

Chronic pancreatitis is characterized by progressive and irreversible loss of pancreatic exocrine and endocrine function. In the majority of cases, particularly in Western populations, the disease is associated with alcohol abuse. The major complications of chronic pancreatitis include abdominal pain, malabsorption, and diabetes. Of these, pain is the most difficult to treat and is therefore the most frustrating symptom for both the patient and the physician. While analgesics form the cornerstone of pain therapy, a number of other treatment modalities (inhibition of pancreatic secretion, antioxidants, and surgery) have also been described. Unfortunately, the efficacy of these modalities is difficult to assess, principally because of the lack of properly controlled clinical trials. Replacement of pancreatic enzymes (particularly lipase) in the gut is the mainstay of treatment for malabsorption; the recent discovery of a bacterial lipase (with high lipolytic activity and resistance to degradation in gastric and duodenal juice) represents an important advance that may significantly increase the efficacy of enzyme replacement therapy by replacing the easily degradable porcine lipase found in existing enzyme preparations. Diabetes secondary to chronic pancreatitis is difficult to control and its course is often complicated by hypoglycaemic attacks. Therefore, it is essential that caution is exercised when treating this condition with insulin. This paper reviews recent research and prevailing concepts regarding the three major complications of chronic pancreatitis noted above. A comprehensive discussion of current opinion on clinical issues relating to the other known complications of chronic pancreatitis such as pseudocysts, venous thromboses, biliary and duodenal obstruction, biliary cirrhosis, and pancreatic cancer is also presented.
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PMID:Chronic pancreatitis: complications and management. 1050 49

1. Dialkyltin compounds have been widely used in industry and agriculture, mainly as biocides, catalysts and plast stabilizer. In dependence on the length of the alkyl chains these organotins exert toxic effects on the immune system, the bile duct, liver and pancreas. It has been supposed that similar to organoarsenic the toxicity of the dialkyltin compounds is related to reactions with biological dithiol groups. Therefore, in the present study, the antidotal effects of 2,3-dimercapto-propane-1-sulfonic acid (DMPS) and meso-2,3-dimercaptosuccinic acid (DMSA) on the organotoxic effects of dibutyltin dichloride (DBTC, single administration of 27 micromol kg(-1) b.w. i.v.) in rats were studied using different doses (100 and 500 micromol kg(-1) b.w.) and routes of administration (i.p. and p.o.) of both chelators. Several parameters of organotoxicity (thymus weight and cellularity, bile duct diameter, histological lesions of pancreas and liver, activities of amylase, lipase and alkaline phosphatase, bilirubin and hyaluronic acid in serum) were measured from 6 h to 8 weeks. 2. DMPS and DMSA diminished the DBTC induced bile duct, pancreas and liver lesions stronger than the thymus atrophy. Moreover, the development of a fibrosis of the pancreas and a cirrhosis of liver several weeks after single administration of DBTC to rats was inhibited by DMPS and DMSA. The antidotal effects on serum parameter were observed after both administration routes of the chelators. DMPS was more effective than DMSA in most measured parameters. The decrease in the biliary excretion of organotin by DMPS and DMSA seems to be the reason for the pronounced protective effects of DMPS and DMSA on bile duct, pancreas and liver. 3. For the treatment of poisonings with dibutyltin compounds, the administration of DMPS or DMSA can be recommended.
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PMID:Antidotal effects of 2,3-dimercaptopropane-1-sulfonic acid (DMPS) and meso-2,3-dimercaptosuccinic acid (DMSA) on the organotoxicity of dibutyltin dichloride (DBTC) in rats. 1077 44

Di-n-butyltin dichloride (DBTC) induced thymus atrophy, bile duct lesions, pancreatitis, and liver lesions in rats. Depending on dose [6 and 8 mg/kg intravenous (i.v.) DBTC] and time (1-24 weeks), the lesions in pancreas developed to a pancreatic fibrosis and the lesions in liver to liver cirrhosis. A single i.v. administration of 4 mg/kg DBTC induces a mild interstitial pancreatitis after 2-4 days followed by a restitutio ad integrum after 21-28 days. In the present study, the lesions of biliopancreatic duct, pancreas, and liver of rats after repeated administration of 4 mg/kg DBTC i.v. at intervals of 3 weeks have been investigated. The histopathological changes of pancreas and liver were examined by light microscopy 1,4, and 7 days and 2,3,4,6,9, and 12 weeks after administration of DBTC. Furthermore, pathobiochemical parameters of pancreatitis (amylase and lipase activity in serum), liver lesions (alkaline phosphatase activity and bilirubin in serum), and of fibrosis (hyaluronic acid in serum) were studied. Repeated administration of rats with DBTC (4 mg/kg i.v.) at intervals of 3 weeks induced an acute interstitial pancreatitis and after 9-12 weeks, a pancreatic fibrosis and liver lesions (intrahepatic bile duct hyperplasia, inflammation in periportal tract, and necrosis). In serum, elevated levels of alkaline phosphatase, bilirubin, and hyaluronic acid were found. This study demonstrates that the organotin compound induces toxic effects on pancreas and liver of rats by repeated administration of lower doses at long intervals. The risk of exposure to organotin at long intervals should be considered.
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PMID:Repeated administration of a mild acute toxic dose of di-n-butyltin dichloride at intervals of 3 weeks induces severe lesions in pancreas and liver of rats. 1172 88

Hemobilia is an rare cause of acute pancreatitis. The most frequent causes are iatrogenic trauma (percutaneous liver biopsy) and hepatic artery aneurysm. To our knowledge, this is the second published case of acute pancreatitis related to hemobilia secondary to hepatocarcinoma complicated cirrhosis in a patient treated with anticoagulants for a mechanical valvular aortic prosthesis. The clinical picture included acute epigastric pain, fever and jaundice. Increased amylase and lipase serum activities, and abdominal CT data confirmed the diagnosis of acute pancreatitis. Gallstone induced acute pancreatitis was suspected and thus, a cholecystectomy was performed. No bile duct stones were found but a clot was extracted from the extrahepatic bile duct during surgery. Arterial embolization was then performed and repeated 1 and 3 months later for recurrence. The patient was asymptomatic eight months later. Hepatic arterial embolization is an effective haemostatic treatment for hemobilia, even though, in this case treatment had to be repeated because of an anticoagulant therapy.
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PMID:[Acute pancreatitis related to hemobilia complicating hepatocarcinoma]. 1248 43

Malignancy, surgical trauma, cirrhosis and tuberculosis account for more than 95% of causes for chylous ascites. We report a case of persistent chylous ascites following acute pancreatitis that responded to parenteral nutrition and octreotide. A 50 year-old male was diagnosed with acute alcoholic pancreatitis after presenting with typical abdominal pain, and elevated amylase and lipase. The acute symptoms resolved within one week. Four weeks later he started developing increased abdominal girth. Examination revealed the presence of shifting dullness and paracentesis confirmed diagnosis of chylous ascites. Investigations for the common causes of chylous ascites were negative. Laparoscopy confirmed the presence of fat necrosis within mesenteric lymph nodes linking the chylous ascites to the episode of pancreatitis. The Chylous ascites was resistant to the usual medical therapy, but responded only to the combination of octreotide and total parenteral nutrition with complete resolution of ascites in 8 weeks. This case of chylous ascites secondary to pancreatitis represents an uncommon presentation with effective management resulting in a dramatic response.
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PMID:Chylous ascites secondary to pancreatitis: management of an uncommon entity using parenteral nutrition and octreotide. 1832 Mar 9

The material comprises 34 patients with anicteric biliary diseases, 20 with obstructive jaundice, 8 with hepatic cirrhosis, and 3 with haemochromatosis. The intestinal contents were aspirated in four subsequent periods of 20 minutes each after ingestion of a standard meal. The volume, pH, and the concentration of alpha-amylase, trypsin, chymotrypsin and lipase were determined in the collections. The concentration of lipase was more markedly reduced than concentrations of amylase and of trypsin in patients with anicteric biliary diseases and in patients with hepatic cirrhosis. Concentrations of enzymes below the lowest normal value throughout the period of digestion represented an uncommon finding. The exocrine pancreatic function is rarely found to be reduced in patients with biliary or with hepatic disorders.
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PMID:pH and concentration of pancreatic enzymes in aspirates from the human duodenum during digestion of a standard meal in patients with biliary or hepatic disorders. 2018 82

The gastric mucosal lesions represent a frequent cause of hemorrhage in the portal hypertension (PHG) and in the hepatic cirrhosis. This study was undertaken to assess the structural and ultra structural modifications in the intimal lamina of the stomach in this pathology. The cells of mucosa show graded alterative transformations. In the gastric mucosa, some of the chief (enzymatic) cells present a quasi-normal histological organization; others increased alterations such as irregular and heterochromatic nuclei, fewer cytoplasm organelles, numerous clear vesicles and heterogeneous lysosomes. The parietal (oxyntic) cells show in their apical cytoplasm wide dilatations of the intracellular canalicles, vesicles, a reduced number of organelles and irregular nuclei. The enteroendocrine cells (APUD) present an increased number of granules and organelles in the cytoplasm. The connective inter-glandular tissue contains active fibroblasts, micro inflammatory zones, blood vessels with hypertrophied endothelium, irregular and dilated lumen. Subsequent to these alterations in the structures of the gastric mucosa in portal hypertension, the function of synthesis/excretion of the pepsinogen, lipase, hydrochloric acid, intrinsic factor, serotonin, etc. are affected.
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PMID:The gastric mucosa in portal hypertension: structural and ultrastructural observations. 2049 42


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