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E Schrumpf

Publications and source records attributed to E Schrumpf.

At least 19 recordsLinked to original sources

[Hepatic vein thrombosis. Diagnostic and therapeutic difficulties].

Budd Chiari syndrome (liver vein thrombosis) may be a diagnostic and therapeutic problem. On the basis of four different cases we review the major diagnostic and therapeutic principles involved. Imaging techniques are necessary in order to establish the diagnosis. Ultrasound examination with Duplex doppler is usually sufficient, but MR angiography is also useful. Treatment options are thrombolysis, surgery or liver transplantation. What treatment is selected will depend on the clinical situation and the prognosis.

Adult

[Treatment of hepatitis C].

Hepatitis C virus (HCV) has been a major cause of post transfusion hepatitis, and is still an important cause of chronic liver disease throughout the world. How to treat patients with chronic HCV infection has been brought into focus in recent years, and a substantial amount of data has been obtained about the development of hepatitis C with and without treatment. This survey considers the diagnosis of hepatitis C, and present treatment modalities and their potential. The patients most likely to respond to treatment are described, and the authors finally discuss why treatment of hepatitis C still should take place in controlled studies.

Hepatitis C

[Liver transplantation in Norway. Results after 10 years and 114 transplantations].

A total of 114 liver transplantations were performed in 106 patients in Norway during 1984-1994. Survival after one year was 65% and after three years 57%. The most frequent causes of death were infections and rejections. The survival rate improved considerably during the period, and after 1990 the 1 year survival was 70%. Approximately 2/3 of the patients return to work or education. Very few patients die later than 12 months after the transplantation. The most frequent indications were primary biliary cirrhosis, metabolic liver disease, primary sclerosing cholangitis, autoimmune cirrhosis and fulminant liver failure. The number of liver transplantations (approximately 4 per million inhabitants) is lower in Norway than in the other Nordic countries. The number should be increased to 7-8 per million inhabitants.

Adolescent

[Is liver transplantation a therapeutic possibility of low priority in Norway?].

The number of orthotopic liver transplantations per year in Norway is smaller than in other countries of Northern Europe. Relatively fewer liver transplantations are performed both in the case of accepted indications such as primary biliary cirrhosis and acute liver failure, and for more arguable indications like post viral cirrhosis and alcoholic liver disease. The authors discuss priorities in health care in Norway. The efficacy of liver transplantation for selected groups of patients is compared with the efficacy of bone marrow transplantation for another selected group of patients. If indications and activity of bone marrow transplantation depict the threshold of prioritization, then more patients with chronic liver disease should be offered liver transplantation as therapy.

Europe

Features of autoimmune hepatitis in primary sclerosing cholangitis: an evaluation of 114 primary sclerosing cholangitis patients according to a scoring system for the diagnosis of autoimmune hepatitis.

Overlapping features between primary sclerosing cholangitis (PSC and autoimmune hepatitis (AIH) have previously been noted. To assess systematically similarities between these disorders, we have evaluated 114 PSC patients (36 women; 78 men), all confirmed by endoscopic retrograde cholangiography (ERC), according to a scoring system proposed by The International Autoimmune Hepatitis Group for the diagnosis of AIH. The scoring system attributes positive or negative scores to the parameters sex, ratio of elevation of serum levels of alkaline phosphatase (ALP) vs. aminotransferase, serum levels of immunoglobulins and autoantibodies, viral markers, history of drug and alcohol intake, genetic factors, liver histology, and response to therapy. Two of the PSC patients (2%) obtained scores above 15 before treatment, satisfying the diagnostic criterion of "definite" AIH. Thirty-eight patients (33%) scored between 10 and 15 points and could be classified as "probable" AIH. The serum level of immunoglobulin G (IgG) was elevated in 68 patients (61% of 111 cases tested), and positive titers of antinuclear antibodies (ANA) or smooth muscle antibodies (SMA) were detected in 24 patients (22% of 111 cases tested). Thirty-five of the PSC patients (33% of 105 evaluable biopsy specimens) obtained positive scores for histological features similar to those of AIH, but the total score for histology was in the negative range in 72 patients (69%) because of the presence of biliary changes. The frequent finding of high scores in PSC patients underlines the similarities PSC may have with AIH. A modification of the scoring system, in particular by increasing the negative score for histological biliary changes, would improve its potential to discriminate between AIH and PSC.

Adolescent

Involvement of guanylate cyclase and phosphodiesterases in the functional heterogeneity of human blood platelet subpopulations.

Cyclic nucleotides are involved in the regulation of platelet activation, shape change, and aggregation. In this study we have investigated the role of guanylate cyclase and phosphodiesterase in three functional heterogeneous human blood platelet subpopulations separated according to density. In low-density platelets aggregation was enhanced and inhibited less when cyclic GMP was increased by sodium nitroprusside, compared to high-density platelets. Low-density platelets possessed a lower basal level of cyclic GMP and exhibited a small increase in cyclic GMP after stimulation with sodium nitroprusside. Cyclic GMP-dependent phosphodiesterase activity was similar in high, low, and intermediate-density platelets. In contrast, the activity of the cyclic AMP-dependent phosphodiesterase was higher in low-density compared to high and intermediate-density platelets. These results suggest that regulation of cyclic GMP and cyclic AMP levels plays an important role in the functional heterogeneity of human blood platelets.

Analysis of Variance

Monstrous ascites in hereditary haemorrhagic telangiectasia.

BACKGROUND: Hepatic involvement in hereditary haemorrhagic telangiectasia (HHT) consisting of fibrosis, telangiectases, and cirrhosis, has been reported as a relatively frequent finding. CASE: A 50-year-old man with HHT presented with monstrous ascites. Liver biopsy demonstrated multiple dilated sinusoids but not cirrhosis. There were no findings indicative of portal hypertension or malignant disease. Portal pressure, recorded in hepatic vein wedge position, was normal. Arteriography showed numerous hypervascular lesions throughout the liver. The clinical course has been stable for more than 2 years. CONCLUSION: No other reason for the monstrous ascites could be found. We thus hypothesize that this case of monstrous ascites is due to hepatic involvement in HHT, presenting as numerous vascular lesions throughout the liver.

Ascites

Interferon-alpha 2b, with or without prior hepatic artery embolization: clinical response and survival in mid-gut carcinoid patients. The Norwegian carcinoid study.

BACKGROUND: Mid-gut carcinoid tumours often present with liver metastases, and survival has then been less than 2 years in earlier reports. We have evaluated the effects of interferon therapy on clinical response and survival, with or without hepatic artery embolization in these patients. METHODS: In a prospective study 30 female and 12 male patients, aged 23 to 75 years, with mid-gut carcinoid tumours and liver metastases underwent surgery with removal of as much as possible of their primary tumour. If technically feasible, embolization of hepatic arteries was performed in the absence of contraindications. Seventeen patients were embolized, and all patients received interferon-alpha 2b treatment for 1 year. Response factors were computer tomography (CT) measurement of the largest liver metastasis and the 24-h urinary excretion of 5-hydroxyindoleacetic acid (5-HIAA). After 12 months patients with objective response or stable disease either continued or withdrew from interferon therapy. Survival was estimated when all patients had been observed for at least 36 months. RESULTS: Nine patients reduced the dose, and five withdrew from interferon treatment owing to side-effects the 1st year. Three patients died. Fifteen patients (39%) showed objective response 12 months after inclusion. Cumulative 5-year survival estimated from inclusion was 37.5% in all 42 patients but 71.4% in those who continued interferon therapy. The difference in survival between the interferon-treated and those who withdrew from interferon therapy at 12 months was significant when embolization was corrected for in a Cox model (p < 0.0125). The seemingly increased survival in embolized versus non-embolized patients did not reach statistical significance (p = 0.07). CONCLUSION: Interferon induced an objective response in mid-gut carcinoid patients as judged by the 24-h urinary 5-HIAA excretion. Patients receiving continuous interferon therapy showed improved response and survival compared with patients who stopped the treatment. Regardless of medical therapy, more survivors and more responders, as evaluated from CT measurements, were found among the embolized patients than among the non-embolized. Embolization could, however, not be shown to have a significant effect on survival.

Carcinoid Tumor

Hepatic retention of copper and selenium in primary sclerosing cholangitis.

BACKGROUND: Previous studies have suggested abnormal copper metabolism in patients with primary sclerosing cholangitis (PSC). In the present work the trace element metabolism was studied in a group of 32 patients with PSC. METHODS: Hepatic copper and selenium concentrations were determined with a sensitive electrothermal atomic absorption technique. Serum concentrations of copper and zinc were determined by conventional atomic absorption. RESULTS: For the patient group serum copper values (20.3 +/- 4.5 mumol/l) were higher than those for the control group (14 +/- 3 mumol/l), and average hepatic copper concentrations were greater by a factor of four. Serum selenium values were slightly lower, although the average hepatic selenium was significantly higher than in the healthy control group. Previous studies have discussed possible toxic effects of hepatocellular copper accumulation, which may be accompanied by formation of activated oxygen species and depletion of glutathione. In the present study, however, it could not be demonstrated that the concentration of the lipoperoxidation product, malonic dialdehyde, was higher than normal in blood. Furthermore, blood concentrations of glutathione and glutathione peroxidase were not abnormal. CONCLUSION: Although a protective effect of the raised selenium concentrations in the liver might be discussed, it is apparent that the copper accumulation in the liver cells described here did not induce detectable changes in the indices studied.

Adolescent

[Treatment of paracetamol poisoning. An indication for liver transplantation?].

Development of metabolic acidosis (pH < 7.30) or the combination of encephalopathy grade III-IV, coagulopathy (PT > 100s) and oliguric renal failure are associated with a poor prognosis in paracetamol-induced fulminant liver failure. It is important to administer N-acetylcysteine as soon as possible after the overdose, but N-acetyl-cysteine also seems to improve survival when given 36-80h following ingestion. Liver transplantation has been performed in some patients with paracetamol-induced fulminant liver failure, but convincing evidence that transplantation improves survival in this group of patients is still lacking. We discuss the difficulties met in deciding if and when to perform liver transplantation. Renal failure may develop some days after paracetamol poisoning, even in the absence of severe liver damage, and haemofiltration and haemodialysis may be necessary.

Acetaminophen

Risk factors in primary sclerosing cholangitis.

The increasing use of liver transplantation and new treatment regimens requires an accurate estimate of the prognosis in primary sclerosing cholangitis. To clarify the natural history and prognosis of this disease, we studied the clinical features at the time of presentation and the outcome in 77 consecutive patients admitted to our hospital. The median age at diagnosis of primary sclerosing cholangitis was 32.5 years; 66% of the patients were male; 76 had concomitant inflammatory bowel disease and two had celiac disease. Thirty-four patients were classified as asymptomatic at diagnosis of primary sclerosing cholangitis. The mean follow-up time was 6.2 years; 25 patients have died or been transplanted. Cholangiocarcinoma has been diagnosed in 11 patients (14%). Female patients have a significantly poorer survival rate than male patients. The bilirubin level was found to be an independent risk factor for both mortality/transplantation, and for the occurrence of cholangiocarcinoma. Age at diagnosis of primary sclerosing cholangitis was an additional risk factor of death/transplantation. As bilirubin is an important prognostic factor for the development of both cholangiocarcinoma and death/transplantation, the construction of prognostic indices seems to be of limited value in the timing of transplantation of the individual patient.

Adolescent

Liver disease in anti-hepatitis C virus-positive Norwegian blood donors.

In a prospective study of 16,756 consecutive blood donors, we found 54 donors (0.3%) to be anti-hepatitis C virus (HCV)-positive by a first-generation enzyme-linked immunosorbent assay. After retesting, 18 donors were confirmed positive or indeterminate by a second-generation recombinant immunoblot assay. Sixteen of these donors were found positive by a second-generation enzyme-linked immunosorbent assay, and 15 of these were positive by HCV polymerase chain reaction with two primer sets. Nine donors (50%) had a history of drug abuse. In 15 donors found positive by a second-generation enzyme-linked immunoblot assay liver biopsy specimens were taken after at least 6 months' follow-up. In all except one hepatitis C RNA-negative donor, histologic abnormalities were observed, even when alanine aminotransferase (ALAT) levels were continuously normal or only moderately elevated. The abnormalities were less pronounced in these donors (n = 5) than in donors with ALAT levels increased more than twice the upper normal limit (p < 0.05). In conclusion, we found the proportion of previous drug abusers in anti-HCV-positive blood donors to be high. We confirm that the presence of anti-HCV (second generation) usually, and HCV-RNA always, seems to indicate ongoing infection--also when ALAT levels are normal. Our study further suggests that low-activity hepatitis, evaluated by ALAT levels, may indicate a milder disease.

Adult

Hepatobiliary disease in ulcerative colitis. An analysis of 18 patients with hepatobiliary lesions classified as small-duct primary sclerosing cholangitis.

BACKGROUND: The aim of the present study was to describe the characteristics of patients with ulcerative colitis (UC) and hepatobiliary disease that does not satisfy the diagnostic cholangiographic criteria of primary sclerosing cholangitis (PSC) and to compare this group with PSC patients. METHODS: Among 199 patients with UC admitted to our department during 1986-91, 64 patients had major hepatobiliary disease considered to be associated with the colitis. Biochemical tests, colonoscopy, endoscopic retrograde cholangiography (ERC), and liver biopsy were performed in these 64 patients and in 5 patients from our outpatient clinic. RESULTS: PSC was diagnosed in 51 patients (group I; 80%). The other 13 patients (20%) and the additional 5 patients (n = 18; group II) all had normal extrahepatic bile ducts. Five patients in group II also had normal intrahepatic ducts, whereas 13 patients had intrahepatic abnormalities. The male to female ratio in group II was 2.0:1. All of them had extensive colitis. The clinical symptoms and the biochemical and histologic findings were quite similar in groups I and II. CONCLUSIONS: The patients in group II of this study constitute a major group with hepatobiliary lesions associated with UC, amounting to one-fourth the number of PSC patients. They have several similarities with classical PSC of the large bile ducts, and we suggest that they be classified as having small-duct PSC.

Adult

Effects of omeprazole and eradication of Helicobacter pylori on gastric and duodenal mucosal enzyme activities and DNA in duodenal ulcer patients.

BACKGROUND: Duodenal and gastric content of mucosal enzymes in duodenal ulcer (DU) patients differs from that of controls. The purpose of this study has been to examine the effect of omeprazole and eradication of Helicobacter pylori on mucosal enzymes in DU patients. METHODS: The enzyme activities of seven gastric and duodenal mucosal marker enzymes from the brush border, lysosomes, and mitochondria have been studied. In study I the measurements were made in 29 patients with an active DU before and after 14 days of omeprazole treatment. In study II 22 duodenal ulcer patients were given bismuth subnitrate, oxytetracycline, and metronidazole (triple therapy) for 2 weeks to eradicate H. pylori. Biopsy specimens were taken from the duodenum and the stomach for enzyme measurements and histologic assessment. In study II additional specimens were obtained from the prepyloric region for urease tests and culture of H. pylori. RESULTS: The ulcer healing rates were more than 90% after both omeprazole and triple therapy. H. pylori was eradicated in 86% after triple therapy. The activities of the brush-border enzymes lactase, neutral-alpha-glucosidase, alkaline phosphatase, leucyl-beta-naphthylamidase, and gamma-glutamyltransferase (gamma-GT) increased significantly in the duodenal bulb and the descending duodenum during treatment with omeprazole. No changes in duodenal enzyme activity were detected after triple therapy, whereas a significant fall in gamma-GT and acid phosphatase activities was seen in the stomach. The mucosal DNA in the gastric antrum decreased both after treatment with omeprazole and after triple therapy. CONCLUSIONS: A similar decrease in mucosal DNA of the gastric antrum was demonstrated after both omeprazole and triple therapy with bismuth subnitrate, oxytetracycline, and metronidazole. Omeprazole also affects the content of duodenal mucosal enzymes, whereas triple therapy particularly affects the gastric mucosal enzyme activity.

Antacids

Etiology and pathogenesis in primary sclerosing cholangitis.

The etiology and pathogenesis of the inflammatory and fibrotic bile duct lesions characteristic of primary sclerosing cholangitis (PSC) is unknown, but several lines of evidence support the contention that genetic and immunologic factors are involved. There is an association with human leukocyte antigens (HLA) with an increased frequency of DR3, DR6, and DR2 positive haplotypes. DRB3*0101(DR52a) is the most strongly associated allele in some studies, but the HLA gene conferring the primary HLA associated susceptibility to PSC remains to be established. There is an aberrant expression of HLA class II antigens (DR and DP) on bile duct epithelial cells, with the potential to present antigens to the surrounding T-lymphocytes. A defective suppressor T-cell function has been suggested in some studies. The patients may have elevated levels of circulating immune complexes, immunoglobulins, and non-organ-specific autoantibodies. Antibodies to perinuclear antigens (pANCA) are present in about 80% of cases. Increased metabolism of complement C3, reduced clearance of immune complexes, and increased concentration of biliary immune complexes have been found. The strong association between PSC and ulcerative colitis (UC) has not been explained. The detection of circulating IgG antibodies against a specific epitope shared by epithelial cells in the bile ducts and colon in about two-thirds of PSC patients may be of importance. Portal bacteremia secondary to a diseased bowel may possibly contribute to development of liver disease in UC. Viral infections and toxic and ischemic factors have also been implicated in the pathogenesis of PSC. In conclusion, PSC seems to occur in genetically predisposed individuals, mediated by immunologic mechanisms. The primary event triggering the disease development is, however, unknown.

Antibody Formation

Duplex Doppler sonography and angiography in the evaluation for liver transplantation.

Duplex Doppler sonography (DDS) and angiography were performed in a prospective study comprising 22 adult patients with hepatic failure. The ability of the 2 methods to visualize the different splanchnic vessels was compared, as well as any abnormality in the liver parenchyma and vessels. The purpose of the study was to decide if DDS could replace angiography in the pretransplantation work-up in these patients. The portal vein was correctly identified as open in 20 patients using DDS and in 21 patients at angiography. Portal vein thrombosis in one patient was correctly diagnosed by both methods. The identification of other vascular structures in the upper part of the abdomen, e.g., liver arteries and splenic vein, was demonstrated in 14 and 16 patients respectively at DDS. Hepatoma in one patient was correctly identified by both methods, as well as the presence of ascites. Splenic artery aneurysms were found in 4 patients at angiography, and in none of these was DDS able to show this abnormality. We conclude that abdominal angiography still seems superior to DDS in the evaluation of patients with hepatic failure prior to orthotopic liver transplantation, especially to demonstrate possible splenic artery aneurysms and detailed mapping of the hepatic arteries.

Adult

[Hepatitis C].

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Hepatitis C