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Biomedical subjects

A Stiehl

Publications and source records attributed to A Stiehl.

At least 55 records · Page 3Linked to original sources
PubMed1995

Biliary secretion of bile acids and lipids in primary sclerosing cholangitis. Influence of cholestasis and effect of ursodeoxycholic acid treatment.

In patients with primary sclerosing cholangitis cholestasis is a prominent feature of the disease. We studied the effect of cholestasis and of ursodeoxycholic acid treatment on the biliary secretion of bile acids and lipids in ten patients with primary sclerosing cholangitis. Ursodeoxycholic acid treatment for 3 months led to an increase in the biliary secretion rates of total bile acids from 0.91 mmol/h to 1.47 mmol/h, mainly due to an increase in urosodeoxycholic acid, which represented 31% of biliary bile acids. With increasing cholestasis, the biliary enrichment of the bile acid pool with urosodeoxycholic acid decreased. Biliary output of endogenous bile acids on average was unchanged, but in patients with cholestasis and diminished output before treatment, it increased after ursodeoxycholic acid. Phospholipid secretion increased from 0.26 mmol/h to 0.43 mmol/h without correlation to the degree of cholestasis. Biliary cholesterol secretion on average was unchanged after ursodeoxycholic acid (0.1 versus 0.09 mmol/h) but, in patients with cholestasis and diminished output before treatment, it increased after ursodeoxycholic acid. The decreasing enrichment of the bile acid pool with ursodeoxycholic acid with increasing cholestasis may be related to its slight effect in advanced disease. The increase in biliary phospholipid secretion may represent another mechanism of action of urosodeoxycholic acid responsible for its beneficial effect in cholestatic liver disease.

Adolescent↗
PubMed1994

Effect of ursodeoxycholic acid on liver and bile duct disease in primary sclerosing cholangitis. A 3-year pilot study with a placebo-controlled study period.

Primary sclerosing cholangitis is a cholestatic disease of the liver characterized by progressive fibrotic inflammation and obliteration of the extra- and/or intrahepatic bile ducts. There is no effective therapy. We, therefore, studied the safety and efficacy of ursodeoxycholic acid in patients with primary sclerosing cholangitis with or without additional ulcerative colitis. In a 1-year ursodeoxycholic acid treatment period, which preceded the controlled study period, ursodeoxycholic acid was well tolerated in 22 of 24 patients with ulcerative colitis and in all three patients without ulcerative colitis. In two patients with ulcerative colitis the dose of 750 mg ursodeoxycholic acid/day led to diarrhea, but following reduction of the dose to 500 and 250 mg/day ursodeoxycholic acid was well tolerated. After 1 year of ursodeoxycholic acid treatment, 20 patients were randomly assigned to receive either ursodeoxycholic acid 750 mg/day or placebo. All of them finished a double-blind, placebo-controlled study period. During ursodeoxycholic acid treatment, the liver enzymes improved markedly. The difference in alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase and gamma-glutamyltransferase between the placebo and ursodeoxycholic acid group was significant (p < 0.05). Following ursodeoxycholic acid treatment, pruritus and fatigue improved in half of the patients but the difference between the placebo and ursodeoxycholic acid group was not significant. According to the ethical guidelines, after 3 months of placebo treatment, the controlled study had to be discontinued because of a more than twofold increase of serum transaminases in 8/10 patients on placebo. After the end of the controlled study, all patients were continuously treated with ursodeoxycholic acid for up to 4 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1994

Biliary tract strictures after orthotopic liver transplantation: diagnosis and management.

Biliary tract complications in 105 patients who had undergone orthotopic liver transplantation were reviewed in order to determine their incidence and localization. In addition, the possible endoscopic and percutaneous management of such complications was evaluated. Signs of cholestasis appeared in 32 of 105 (30.5%) transplant recipients after a mean of 6.5 months (range 1-19 months), and visualization of the biliary system was performed in all. Twenty biliary tract complications were observed in these 32 patients (62.5%). There were multiple strictures, both intrahepatic and extrahepatic, in 11 grafts. Five of the nine extrahepatic strictures were not confined to the site of the bile duct anastomosis, and involved the whole common bile duct of the donor liver, while the remaining four were confined. The multiple intrahepatic and extrahepatic strictures were related either to occlusion of the hepatic artery or to the fact that the graft had been in a cold ischemic state for a prolonged time. In contrast, multiple strictures and necrosis of the whole extrahepatic bile duct were attributed to local ischemia due to the harvesting procedure. Stenoses strictly confined to the site of anastomosis were thought to be due to surgical technique. Ten extrahepatic bile duct stenoses with considerably impaired bile flow were corrected endoscopically (four), percutaneously (three) and by surgery (three). In four patients with complications in the whole intrahepatic and extrahepatic system, retransplantation was necessary. Biliary tract complications in our patients occurred in up to 19% after liver transplantation on long-term follow-up. Complications of only the extrahepatic system can be treated successfully, whereas complications involving multiple stenoses of the intrahepatic bile duct system frequently require retransplantation.

Adolescent↗
PubMed1994

Ursodeoxycholic acid therapy in treatment of primary sclerosing cholangitis.

The beneficial effects of ursodeoxycholic acid (UDCA) in patients with primary biliary cirrhosis led to therapeutic trials with this bile acid for the treatment of PSC. In two prospective placebo-controlled trials, UDCA led to a significant improvement of AP, GGT, ALT, AST, and, in one study, also of serum bilirubin. In both studies liver histology improved significantly, mainly due to a decrease of cellular infiltrates in portal triads. Pruritus and fatigue improved in approximately one-third of the patients, but, compared to placebo, this effect was not significant. In a follow-up study after on average 3.1 years of UDCA treatment, 7/43 of the patients with stages I-IV disease developed a stenosis of the common bile duct which was effectively treated by endoscopic dilatations. Of 57 patients with PSC included since 1987 in the study, 14 dropped out and of these in 10 information on the outcome is available. In patients treated by UDCA and, whenever necessary, by endoscopic dilatations, the frequency of transplantations was significantly reduced in comparison to patients who dropped out of the study. Bile duct carcinoma developed in 5% of our patients. The data indicate that treatment of patients with PSC with UDCA and by endoscopic dilatations of common duct stenoses is promising. In patients with endstage disease, the only effective therapy is liver transplantation. Therefore, the early diagnosis of the disease seems very important.

Cholangiography↗
PubMed1994

Ursodeoxycholic acid in the treatment of primary sclerosing cholangitis.

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease characterized by inflammation and obliterative fibrosis of bile ducts leading to their progressive destruction (1-4). As a consequence, cholestasis with elevated serum levels of alkaline phosphatase (AP) and gamme glutamyl transpeptidase (GGT) and bilirubin in more advanced disease is the most prominent feature of this disease. The diagnosis of PSC is primarily based on endoscopic retrograde cholangiography with demonstration of irregular strictures and dilatations. In liver biopsy, typical findings are portal and periodical inflammation and fibrosis. Since PSC is a focal disease, the characteristic histological findings may or may not be seen in a single liver biopsy. The cause of PSC is still unknown. The association with histocombatibility antigens indicates that immunological mechanisms may be involved but it is still unclear whether the disease is immunogenic. Alternatively, bacteria and bacterial toxins from the colon might play a role (3, 4). In 70% of cases PSC is associated with ulcerative colitis (5) and, therefore, in all patients with this intestinal disease who also have elevated levels of liver enzymes, a cholangiography should be performed. Recently, in up to 80% of patients with PSC anti-neutrophil-cytoplasmatic-antigens (ANCA) were found to be elevated (6) and, in future, this test may help to diagnose the disease more easily. Up to now, however, the disease is usually diagnosed at a relatively advanced stage when the patients have jaundice.

Cholangitis, Sclerosing↗
PubMed1994

Crohn's disease with Behçet's syndrome like appearance: a case report.

The differential diagnosis of Behçet's syndrome or Crohn's disease can be extremely difficult. In this case report we present a 32-year old female patient with Crohn's disease. The coexisting extraintestinal symptoms such as mucocutaneous apthous lesions of the mouth and vulva, the erythema nodosa of the lower extremities and recurrent arthralgias made the correct diagnosis even more difficult. The patient was treated with a combination of systemic corticosteroids and azulfidine enema, under which she recovered.

Adult↗
PubMed1994

Acetylsalicylic acid in the prevention of early stenosis and occlusion of transjugular intrahepatic portal-systemic stent shunts: a controlled study.

Stenosis or occlusion of the transjugular intrahepatic portal-systemic stent shunt may be initiated by aggregation and activation of thrombocytes on the surface of the metallic stent material. To find effective prevention of this event, we conducted a controlled trial administering acetylsalicylic acid for 3 mo. Forty-four patients (8 women and 36 men) with portal hypertension were included in this study. The patients were randomized into a group receiving 100 mg acetylsalicylic acid/day (n = 21) or into a control group (n = 23). Treatment was started immediately after transjugular intrahepatic portal-systemic stent shunt. Three months after transjugular intrahepatic portal-systemic stent shunt, 15 patients in the acetylsalicylic acid group and 19 patients in the control group underwent clinical reevaluation, gastroscopy and recatheterization with determination of the portal-systemic pressure gradient. No variceal bleeding occurred in any patients. In four patients in the acetylsalicylic acid group, erosive gastritis was observed in gastroscopy in contrast to only one patient in the control group. Complete patency of the stent was noted in 10 of 15 patients in the acetylsalicylic acid group and in 14 of 19 patients in the control group. Transjugular intrahepatic portal-systemic stent shunt restenosis associated with a significant increase of the portal-systemic gradient occurred in five patients in the acetylsalicylic acid group, which required redilation in all and additional stent placement for expansion of the stented tract in two patients. In the control group, redilation was necessary in five patients with additional stent extension in two patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1993

[Treatment of cholestatic liver diseases with phenobarbital and ursodeoxycholic acid].

Liver transplantation is the therapy of choice in the final stage of PBC, PSC and biliary atresia. UDCA leads to remission of the symptoms and the clinic in the early stages not only of these diseases but of cholestasis of pregnancy and of cholestasis in cystic fibrosis as well. In benign intermittent cholestasis only phenobarbital but not UDCA seems to be efficacious. The efficacy of UDCA in chronic active hepatitis and alcoholic hepatitis is not yet guaranteed.

Cholestasis, Intrahepatic↗
PubMed1993

Effect of ursodeoxycholic acid on the kinetics of cholic acid and chenodeoxycholic acid in patients with primary sclerosing cholangitis.

Treatment of patients with cholestatic liver diseases with ursodeoxycholic acid has been shown to have beneficial effects that may be related to a shift in the balance between hydrophilic and hydrophobic bile acids in favor of hydrophilic bile acids. During treatment of patients with primary sclerosing cholangitis with ursodeoxycholic acid, plasma concentrations of some endogenous bile acids decrease. To test whether the changes in plasma bile acids are due to decreases of their pool sizes or synthesis rates, we determined bile acid kinetics of cholic and chenodeoxycholic acid in six patients with primary sclerosing cholangitis, of whom four also had ulcerative colitis. All patients were studied before and 3 mo after the start of ursodeoxycholic acid treatment. Six healthy subjects served as controls. In patients with primary sclerosing cholangitis, pool sizes of cholic and chenodeoxycholic acid were considerably smaller than those in healthy controls; after ursodeoxycholic acid treatment they were unchanged. Fractional turnover and synthesis of cholic acid increased significantly after ursodeoxycholic acid administration. Fractional turnover of chenodeoxycholic acid also increased significantly, whereas synthesis of this bile acid was unchanged. Our data indicate that in patients with primary sclerosing cholangitis, pool sizes of bile acids are reduced. The decrease of levels of endogenous bile acids in plasma under ursodeoxycholic acid treatment despite unchanged bile acid pool sizes indicates redistribution of the bile acids into the enterohepatic circulation, probably because of improved hepatic clearance after ursodeoxycholic acid treatment.

Adult↗
PubMed1992

Assessment of hepatic phagocytic activity by in vivo microscopy after liver transplantation in the rat.

Phagocytic activity of sinusoidal lining cells was studied in 32 livers of male Lewis rats by in vivo fluorescence microscopy with epiillumination. Normal livers (group 1, n = 8) were compared with orthotopic syngeneic liver grafts 90 min after reperfusion after a period of cold storage in University of Wisconsin solution for 17 hr (group 2, n = 10) or 24 hr (group 3, n = 14). After bolus injection of fluorescence-labeled latex particles (3 x 10(8)/kg; diameter = 1.1 micron), zonal distribution and kinetics of adherence of latex beads were quantified by off-line video analysis. Hepatocellular function was estimated by the rate of bile production and biliary concentrations of bile acids. In normal livers 50%, 37% and 13% of injected latex beads adhered in zones 1, 2 and 3, respectively, whereas in transplanted livers a more homogeneous distribution was found (group 2: 37%, 41%, 22%; group 3: 37%, 39%, 24%; p less than 0.01 vs. controls by analysis of variance). Kinetic analysis of phagocytic activity showed no significant difference between group 1 (88% adherence of visible latex beads 3 min after injection) and group 2 (90% adherence). However, after long-term preservation in group 3, sinusoidal adherence was significantly faster (96.4% adherence; p less than 0.001). Bile secretion in group 2 was lower than in controls and severely depressed in group 3 (group 1: 1.1 +/- 0.07 microliter/min/gm liver [mean +/- S.E.M.]; group 2: 0.8 +/- 0.07; group 3: 0.1 +/- 0.04; p less than 0.001) without significant changes in bile acid concentrations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenosine↗
PubMed1992

Intestinal absorption of ursodeoxycholic acid in patients with extrahepatic biliary obstruction and bile drainage.

Ursodeoxycholic acid (UDCA) dissolves cholesterol gallstones and improves liver function test results in patients with cholestatic liver diseases. Its absorption was studied in patients who had complete extrahepatic biliary obstruction caused by pancreatic carcinoma but no intestinal or liver disease. Six patients received 500 mg chenodeoxycholic acid (CDCA) or 250-2000 mg UDCA in capsules in single oral doses in random order, with an interval of 2 days between the different treatment regimens. In the control period the patients excreted into bile 382.3 +/- 108.0 mumol CDCA (mean +/- SD) and 1866.7 +/- 172.6 mumol cholic acid per 24 hours. After administration of 1273.6 mumol (500 mg) CDCA, biliary excretion of this bile acid increased to 1370.9 +/- 185.7 mumol/24 h, indicating an intestinal absorption rate of 77.6% +/- 9.8%. After oral administration of 636.8 mumol (250 mg), 1273.6 mumol (500 mg), 2547.2 mumol (1000 mg), and 5094.4 mumol (2000 mg) of UDCA, the respective absorption rates were 60.3% +/- 7.4%, 47.7% +/- 9.0%, 30.7% +/- 7.5%, and 20.8% +/- 3.9%, and whereas in the control period no UDCA was detected in the bile, the UDCA percentages measured were 14.6% +/- 8.2%, 19.6% +/- 9.1%, 23.1% +/- 11.3%, and 27.4% +/- 12.1%. The coadministration of CDCA did not enhance the absorption of UDCA. The data indicate that absorption of orally administered CDCA is almost complete, whereas UDCA absorption is incomplete. With increasing doses UDCA absorption decreases. To achieve absorption of adequate amounts of UDCA, high and/or multiple doses are needed.

Administration, Oral↗
PubMed1992

[Treatment of cholestatic liver diseases; the role of ursodeoxycholic acid].

Ursodeoxycholic acid (UDCA) allows symptomatic treatment of cholestatic liver diseases such as primary biliary cirrhosis, primary sclerosing cholangitis, intrahepatic biliary atresia, and cholestasis of cystic fibrosis. Patients should be treated at an early stage of the disease in order to prevent progression to cirrhosis. Since UDCA has no toxic effects longterm treatment with this substance is possible without the risk of undesired side effects. In patients with primary biliary cirrhosis and rapid progression of the disease, UDCA may be combined with an immunosuppressive substance (i.e. cyclosporin). In primary sclerosing cholangitis, biliary atresia and cholestasis of cystic fibrosis, UDCA at present seems the only treatment of which a benefit for the patients can be expected. In endstage disease liver transplantation is indicated. The role of UDCA in chronic hepatitis and alcohol induced liver disease needs to be clarified in further studies. Whether the improvement of laboratory tests in such patients indicates amelioration of the course of disease, still is unclear.

Animals↗
PubMed1992

[Pathophysiology of cholestasis: correlation between bile acid metabolism and liver damage].

In patients with cholestasis the biliary excretion of bile acids is diminished. Hepatic sulfation, glucuronidation and hydroxylation of bile acids are increased and enhance their urinary excretion. These metabolic pathways however, are not sufficient to prevent hepatic damage by bile salts. Ursodeoxycholic acid, a very hydrophilic and therefore untoxic bile acid, in part prevents the hepatotoxicity of more hydrophobic bile acids.

Animals↗
PubMed1992

[Combined ursodeoxycholic acid plus colchicine--treatment of primary biliary cirrhosis: results of a placebo-controlled double-blind study].

In the present pilot study we investigated the effects of urso treatment alone in comparison to a combined treatment with urso plus colchicine in PBC. 22 patients with PBC in the histological stages 1-3 entered the study. All patients were pretreated with urso alone (10-12 mg/kg) for 12 months. Thereafter treatment was continued in a double-blind randomized fashion with urso plus placebo or urso plus colchicine (1 mg/day) for another 12 months. During the initial 12 months urso treatment liver function tests improved significantly in all patients, pruritus improved in 60% of patients. After randomization to the different treatment groups the biochemical parameters stabilized at the lower level and no significant differences could be found between urso plus placebo and urso plus colchicine treatment concerning aminotransferases, alkaline phosphatase, bilirubin, cholinesterase, albumin or cholesterol. The results of this pilot study suggest that the addition of colchicine to an initial urso treatment does not lead to further improvement of aminotransferases, alkaline phosphatase, bilirubin or clinical symptoms like pruritus.

Colchicine↗