Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The second child of healthy unrelated parents presented with chronic diarrhoea since the age of two months, initially associated with non-characteristic liver involvement. Recurrent infections, severe failure to thrive and various metabolic deficiencies complicated the further course, as well as profuse watery diarrhoea with elevated regulatory gut peptides, responding only to somatostatin analog treatment. At 22 months of age, intermittent cholestasis with permanently normal serum gamma-glutamyltransferase was evident. The child died of fulminant purulent meningitis at the age of three years six months. Liver histology showed intrahepatic cholestasis, bile duct paucity with focal proliferation as well as slight portal and intralobular fibrosis. The clinical, biochemical and histopathological findings were indicative of Byler's disease.
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PMID:Progressive idiopathic cholestasis presenting with profuse watery diarrhoea and recurrent infections (Byler's disease). 139 94

Thirty duodenal and three upper-jejunal endocrine tumors are reported. Clinical manifestations included: a) the Zollinger-Ellison syndrome (10 cases); b) peptic ulcer disease in which hypergastrinemia was not documented (3 cases); c) cholestasis or cholelithiasis (4 cases); d) abdominal pain (4 cases); e) gastro-intestinal bleeding (1 case); f) celiac sprue (1 case). Ten further tumors were discovered incidentally, at autopsy or in pathological specimens after gastrectomy or duodenopan-createctomy. Histological pattern was trabecular in 19 cases, insular in 2 and mixed in ten cases. Two cases were typical ganglioneuromatous paragangliomas. All tumors were examined immunohistochemically. Twelve tumors contained gastrin, four somatostatin, six both of these peptides, one serotonin, two both gastrin and serotonin, and two tumors contained gastrin, serotonin and somatostatin. Ganglioneuromatous paragangliomas combined somatostatin and/or pancreatic polypeptide containing endocrine cells with protein-S100-positive Schwann cells. In four tumors no peptide or amine was demonstrated. Gastrin cell tumors (63.6% of our cases), both functionally active (gastrinomas) and clinically silent, predominated in the proximal duodenum, while somatostatin cell tumors (15.1%) and paragangliomas were mostly found in the periampullary region. Two tumors were classified as malignant on the basis of lymph node metastases, and both were jejunal gastrinomas associated with Zollinger-Ellison syndrome. Two somatostatin cell tumors had manifestations of von Recklinghausen's disease.
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PMID:Endocrine tumors of the duodenum and upper jejunum. A study of 33 cases with clinico-pathological characteristics and hormone content. 216 Apr 22

Twenty patients undergoing sphincteroplasty for cholelithiasis were randomly divided into two groups of 10. The former (T) were treated with a 4-h somatostatin intravenous drip (250 micrograms/h), started at the beginning of operation, while the latter (C) made up the control group. Serum and urine amylase, amylase creatinine clearance ratio, and liver function tests were assessed for 2 days before surgery, after the operation and for a period of 5 postoperative days. Homogeneity between the two series was verified in experimental conditions. Statistical differences occurred postoperatively in amylase creatinine clearance ratio, which proved higher in C group, and gamma-GT, which was higher in T group. Short-term somatostatin administration proved effective in reducing the postoperative amylase creatinine clearance ratio, although more evident results are reported after long-term administration. Cholestasis or any serious impairment in liver function did not occur, suggesting the suitability of somatostatin use even in patients with jaundice. Since a relationship between postoperative amylase levels and risk of pancreatitis has not yet been proved, the value of somatostatin in the prevention of postoperative pancreatitis after sphincteroplasty needs to be further verified.
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PMID:Effect of perioperative somatostatin administration of sphincteroplasty-induced increase of amylase. 242 27

Somatostatin (SST) has been shown to induce cholestasis in the dog and in the rat. In man, it is still unknown whether SST modifies bile formation. The present study was undertaken to examine the influence of SST on bile secretion in man. Two volunteers who had a total external biliary fistula received 1-hour SST infusions (3.5 micrograms/kg/h). Bile flow, bile acid, phospholipid and cholesterol biliary outputs were measured before, during and 1 h after the infusion. The SST infusion was associated with a pronounced decrease in bile flow and in bile acid secretion and with an increase in bile cholesterol saturation. These findings suggest that SST has cholestatic properties in man as in other species. This may provide a rational explanation for the formation of gallstones and for the steatorrhea observed in patients with somatostatinomas or during therapeutic SST administration.
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PMID:Pharmacological effect of somatostatin on bile secretion in man. 256

A case of a 36-year-old man with pancreatic somatostatinoma is reported. Such cases are very rare. The following signs were found; quick ESR, increased alpha 2- and beta-globulins, shortened Weltmann's coagulation band, increased serum bilirubin level, especially of the conjugated fraction, enzyme constellation of cholestasis and moderate liver cytolysis. Ultrasound examination of the abdomen, retrograde cholangiopancreatography and percutaneous liver cholangiography were performed in order to prove the diagnosis. They demonstrated an obstruction in Vater's papilla region and a bulky process in the head of the pancreas. The patient was operated. The microscopic, electron microscopic and immunohistochemical examinations proved a somatostatinoma of the pancreas. The positive immunohistochemical tests for somatostatin and carcinoembryonal antigen in the tumor cels are especially indicative.
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PMID:[A case of somatostatinoma of the pancreas]. 284 67

Previous experiments have demonstrated the cholestatic effects of somatostatin administration in several animal species. These effects were confirmed in the rat. Nine pairs of intact awake rats received intravenous sodium taurocholate (23 mg hr-1) to stabilize bile flow, and half were later given somatostatin at doses of 185 micrograms hr-1. After 1 hr of somatostatin the experimental group showed a significant decrease in bile flow compared to the control group. Cholestasis reversed when somatostatin infusion was stopped. An in situ isolated perfused rat liver technique was used to assess the direct effects of somatostatin on biliary flow. In 10 pairs of rat livers, after achieving stable bile flow, half of those perfused (the "experimentals") received continuous (370 micrograms hr-1) somatostatin infusion, while the controls received saline. The percentage change in bile flow from baseline in the somatostatin group was not significantly different from that in controls for any test period. Bile analysis revealed no significant differences between groups for cholesterol, phospholipid, or bile salt concentrations or outputs. These data suggest that somatostatin inhibits bile secretion by some mechanism other than direct inhibition of bile secretory mechanisms.
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PMID:Is somatostatin a directly acting cholestatic hormone? 286 22

Somatostatin was administered intravenously to male Wistar rats, recovered for 3 h from an anesthesia during which the common bile duct and jugular vein were cannulated. Different bile acid-secretory rates were obtained by infusion of saline, or of Na+-taurocholate (150 nmol/min/100 g body wt), or by 8-h bile depletion. At the dose of 2 micrograms/h/100 g body wt, somatostatin causes a prompt decrease of bile flow (about 30%) and of bile acid secretion (32%-47%). The bile acid-independent fraction of canalicular bile is more decreased than the one associated with bile acid secretion. The changes are dose dependent and show a saturation pattern, with half-maximal saturation already at 2.2 ng/min/100 g body wt. Despite this cholestasis, endogenous bilirubin secretion remained unchanged, pointing to different secretory mechanisms for bilirubin and bile acids. In the isolated and perfused liver, somatostatin displays an anticholeretic effect, proportional to the amount of Na+-taurocholate present in the system. Hepatic blood flow and O2 consumption remained constant during perfusion, and were not affected by somatostatin. The hepatic transport of bile acid, and the water and electrolyte secretion are directly affected by somatostatin, and the experimentally-induced cholestasis seems a new and suitable model for studying mechanisms of bile secretion.
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PMID:Cholestatic action of somatostatin in the rat: effect on the different fractions of bile secretion. 611 14

Short-term experiments were performed on adult mongrel dogs (15 to 25 kg) anesthetized with sodium pentobarbital. The operative procedure included cholecystectomy, side-to-side mesocaval shunt with ligation of the portal vein, and cannulation of the common bile duct. Intravenous sodium taurocholate (500 mg/hr) was administered to prevent depletion of bile salts. Somatostatin (125 micrograms over 30 minutes) was given to six dogs after 2 hours of bile salt infusion, while six additional dogs received saline to serve as control. Bile flow decreased significantly during administration of somatostatin (206 +/- 28 to 150 +/- 21 microliters kg-1 15 min-1, P less than 0.001) and was unchanged during administration of saline (216 +/- 45 to 216 +/- 46 microliters kg-1 15 min-1). This decrease persisted for 1/2 hour after cessation of the somatostatin infusion. Bile salt outputs were similar for both groups throughout the experiment. The data demonstrate that somatostatin-induced cholestasis can be independent of portal blood flow.
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PMID:Somatostatin-induced cholestasis can be independent of portal blood flow. 613 63

The liver is a parenchymal organ that has a substantial capacity to regenerate after damage. Obstructive jaundice is a common surgical disease and potentially risky. A successful outcome of operations depends upon the hepatic regeneration reserve. Insulin is one of factors responsible for hepatotrophic regeneration and somatostatin has a reversal suppressive action. Experimental obstructive jaundice was introduced and relieved. In addition, serum insulin and somatostatin concentrations were measured. We used immuno-histochemical study of pancreatic tissue by immunogold to express the tissue relative insulin and somatostatin concentrations. Nucleolar organizer regions (NORs) were used to predict the nucleolar activity of liver cells. In our studies, we observed the serum concentrations of insulin and somatostatin were similar to the relative tissue concentration in pancreatic tissues. The relative tissue gold-particle score of insulin in group A (rats with common bile duct tied), was CONT: T4: T7: T14 = 100%: 90.5%: 68.3%: 46.2%; of somatostatin was 100%: 120%: 118.2%: 115.5% respectively. In group B (common bile duct tied for 4 days then relieved), the gold-particle score of insulin was T4: T4R4: T4R7: T4R14 = 90.5%: 62.8%: 72.2%: 95.4%; of somatostatin was 120.2%: 114.3%: 108.1%: 106.2% respectively. In group C (common bile duct tied for 7 days then relieved), the gold-particle score of insulin was T7: T7R4: T7R7: T7R14 = 68.3%: 53.3%: 73.5%; of somatostain was 118.2%: 109.4%: 104.6%: 102.1% respectively. The mean numbers of AgNORs in group A revealed CONT: T4: T7: T14 = 2.24 +/- 0.24: 3.02 +/- 0.96: 3.26 +/- 1.02:3.08 +/- 0.84, group B was T4: T4R4: T4R7: T4R14 = 3.02 +/- 0.96: 3.03 +/- 0.73: 3.36 +/- 1.12: 3.72 +/- 1.46, and group C showed T7: T7R4:T7R7: T7R14 = 3.26 +/- 1.02: 3.26 +/- 0.84: 3.31 +/- 1.24: 3.54 +/- 1.24. In conclusion, our studies suggested: (1) liver regeneration appeared promptly after obstructive jaundice developed, but prolonged cholestasis inhibited this process. (2) Insulin levels gradually fell during the process of obstructive jaundice. Those levels elevated when cholestasis was improved. Nevertheless, both insulin and hepatic regeneration power could not reflect the initial improvement of cholestasis simultaneously. It took a longer time for the improvement of cholestasis and the recovery of the liver function. (3) Patho-physiologically, somatostatin had a weak influence on hepatic regeneration during obstructive jaundice. (4) Our studies provided clues that early biliary drainage might improve hepatic regeneration capacity. Supplement of insulin during the obstructive jaundice might be helpful for the improvement of hepatic regeneration power.
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PMID:Changes of insulin and somatostatin and their relationship to liver regeneration in experimental obstructive jaundice. 942 63

A 74-year-old male suffering from Recklinghausen's fibromatosis (NvR) is reported. He presented with weight loss, cholestasis, endocrine and exocrine pancreatic insufficiency. These symptoms were caused by a neuroendocrine tumor of the ampulla of Vater containing somatostatin. The tumor induced an obstruction of both the common bile and the pancreatic duct. In addition to this uncommon tumor, a silent pheochromocytoma was found. The patient was treated by endoscopic papillotomy, substitution of pancreatic enzymes and additional enteral nutrition. After recovery no progression of the disease was observed over one year. A review of the literature shows that patients with neurofibromatosis are at high risk for periampullar tumors. In particular, somatostatin-rich carcinoids were previously documented. Pheochromocytomas are also quite prevalent in NvR. However the combination of NvR, pheochromocytoma and somatostatin-rich neuroendocrine tumors of the duodenum has only been reported a few times. An explanation for the high prevalence of neuroendocrine tumors in NvR might be the loss of neurofibromin, a tumor suppressor protein, which is the main product of the neurofibromatosis-l-gene.
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PMID:[A rare combination of pheochromocytoma ans somatostatin-rich neuroendocrine tumor of Vater's papilla (carcinoid) in a patient with von Recklinghausen neurofibromatosis]. 957 7


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