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Biomedical subjects

B Portmann

Publications and source records attributed to B Portmann.

At least 181 records · Page 10Linked to original sources
PubMed1982

High serum vitamin B12 binding capacity as a marker of the fibrolamellar variant of hepatocellular carcinoma.

Ten (9.3%) of 107 patients with hepatocellular carcinoma had considerably increased serum unsaturated vitamin B12 binding capacity. All 10 were young (mean 12 years), had no serum alpha-fetoprotein, and no underlying cirrhosis; all had a longer survival compared with patients without increased serum unsaturated vitamin B12 binding capacity in the study. Seven of the 10 patients had fibrolamellar hepatocellular carcinoma, a recently recognised histological variant, which was found in only one young patient without increased serum unsaturated vitamin B12 binding capacity and no alpha-fetoprotein among the remaining 97. This high degree of correlation between increased serum unsaturated vitamin B12 binding capacity and fibrolamellar hepatocellular carcinoma has not been reported before. Increased serum unsaturated vitamin B12 binding capacity may be of considerable help in diagnosis, prognosis, and monitoring treatment of this well-defined group of patients with hepatocellular carcinoma but no alpha-fetoprotein.

Adolescent↗
PubMed1982

Accelerated development of alcoholic cirrhosis in patients with HLA-B8.

To test whether the increased prevalence of HLA-B8 reported in patients with alcoholic cirrhosis is due to the antigen being a genetic marker of susceptibility to liver damage from alcohol, patients who had cirrhosis of comparable clinical and histological severity were investigated for HLA-B8 status and cumulative alcohol intake. Both male and female cirrhotics with HLA-B8 had been drinking greater than 40 g alcohol/day for a shorter period of time (16.6 +/- 1.4 men, and 9.4 +/- 2.0 years, women) than their counterparts without this antigen (23.7 +/- 1.7, p less than 0.005, and 15.8 +/- 2.0 years, p less than 0.05, respectively), but the mean daily alcohol intake was similar whether patients had HLA-B8 or not. These results suggest that genetic determinants linked to HLA-B8 enhance the rate of development of liver damage in those who drink potentially hepatotoxic amounts of alcohol.

Adult↗
PubMed1982

Histological evidence of hepatitis-B-virus infection with negative serology in children with acute leukaemia who develop chronic liver disease.

A high percentage of patients with acute leukaemia in established remission develop chronic liver disease: how important hepatitis-B-virus (HBV) infection is as an aetiological factor is not clear. The presence of HBV markers in liver and serum of 23 leukaemic children with liver disease was investigated at the time of a diagnostic biopsy just before treatment withdrawal. Although, at this time, none had HBV antigens or antibodies in the serum by radioimmunoassay, HBsAg was detected by direct immunofluorescence in the cytoplasm of hepatocytes in 13 children, 7 of whom also had hepatitis-B core or e antigens in hepatocyte nuclei. This pattern of both cytoplasmic and nuclear fluorescence was present in 4 of 6 patients with the histological features of chronic active hepatitis on liver biopsy. The failure of release of viral antigens into serum and the absence of an adequate immune response were probably due to the intense chemotherapy used to induce and maintain remission, since HBV markers appeared in the serum within 15 months of stopping treatment in 8 of the children in whom viral antigens had been detected in liver tissue but in none of those whose biopsy specimens were negative by immunofluorescence. These results suggest that HBV infection may be an important cause of chronic liver disease in children with leukaemia and show that during treatment serological tests may fail to detect the presence of the virus.

Adolescent↗
PubMed1982

Recurrence of primary biliary cirrhosis after liver transplantation.

Three patients who had undergone orthrotopic liver transplantation for primary biliary cirrhosis and were being maintained on immunosuppressive therapy were investigated 31/2 to 41/2 years later because of the redevelopment of pruritus and mild jaundice. In one patient pigmentation was again evident, and all three had a rise in the titer of serum mitochondrial antibody after an initial fall. Liver histology showed features of primary biliary cirrhosis with non-suppurative destructive cholangitis, lymphoid aggregates, and increased deposition of copper-binding protein in the absence of cholestasis. None of these features was found in patients who had received grafts for other conditions and had lived for comparable periods, nor were they found in patients who had had rejection with bile-duct abnormalities. The overall findings indicate a recurrence of primary biliary cirrhosis in the donor organ.

Adult↗
PubMed1982

Lymphocyte cytotoxicity to autologous hepatocytes in HBsAg positive chronic liver disease.

Lymphocytes from 39 patients with HBsAg positive chronic liver disease were incubated with their own hepatocytes to investigate mechanisms of lymphocyte-mediated liver damage. Cytotoxicity was significantly increased in 46% overall, and in 73% of those with chronic active hepatitis. Unlike HBsAg negative chronic active hepatitis where only non-T cells were cytotoxic, HBsAg positive patients had both cytotoxic T and non-T cells. A purified liver membrane complex (LSP) and aggregated IgG both blocked non-T cytotoxicity without affecting T cell cytotoxicity; this suggests that the former is probably an antibody-dependent cell-mediated reaction against normal membrane components. This was confirmed in preliminary studies which demonstrated that preincubation of hepatocytes with the F(ab)2' fragment of an anti-human IgG reduced non-T lymphocyte cytotoxicity. T-cell cytotoxicity was restricted to HBeAg-positive patients, suggesting a relationship between T-cell cytotoxicity and viral replication. Purified HBsAg, however, blocked cytotoxicity in only three of 11 cases. Non-T lymphocytes reacting with normal membrane components may contribute to liver damage in both 'autoimmune' and virus-associated chronic liver disease, whereas cytotoxic T-cells, probably reacting with viral determinants, are exclusive to those with viral replication.

Adolescent↗
PubMed1982

Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease.

Serum glycocholic acid (SGC) was measured by radioimmunoassay in 277 samples from 122 children with hepatobiliary disorders and from 23 healthy age-matched controls. In patients with hepatobiliary disease the SGC was more frequently abnormal (83%) than values for serum albumin (7%), prothrombin time (17%), bilirubin (22%), alkaline phosphatase (45%), aspartate transaminase (57%) and gammaglutamyl transpeptidase (63%). The cumulative frequency of abnormality of these six tests was equal to that of SGC alone. Serum glycocholic acid concentrations were raised in 13 patients in whom all other tests of liver function were normal. Two of these had clinical and histological evidence of liver disease, while four had biopsy-proven hepatic fibrosis or cirrhosis, and two of three with chronic active hepatitis in remission subsequently relapsed. Four patients have as yet, no other clinical or biochemical evidence of continuing liver disease. Serum glycocholic acid was normal in seven children with abnormal aspartate transaminase or gammaglutamyl transpeptidase in whom there is strong suspicion of significant hepatic disease. A wide range of values of SGC was found with marked overlap between the values found in the different disease entities studied. The SGC value was related to the serum concentration of aspartate transaminase and gammaglutamyl transpeptidase but not to other tests of liver function. Serum glycocholic acid concentration was considered in relation to the severity of histological abnormality in 25 percutaneous liver biopsies. The extent of the rise in SGC was related to the presence or degree of histological severity of oedema in the portal tracts, disruption of the limiting plate, parenchymal fibrosis and hepatocellular necrosis but not to other histological features. The very high incidence of abnormal SGC values found in this study does suggest that in an ordinary inpatient and outpatient service SGC determination is a practical and sensitive indicator of the presence of significant liver disease but for its comprehensive identification aspartate transaminase and gammaglutamyl transpeptidase must also be determined.

Adolescent↗
PubMed1982

Specificity of T lymphocyte cytotoxicity to autologous hepatocytes in chronic hepatitis B virus infection: evidence that T cells are directed against HBV core antigen expressed on hepatocytes.

Peripheral blood T lymphocytes from 21 patients with chronic HBV infection were incubated with autologous hepatocytes in a microcytotoxicity assay. Cytotoxicity was significantly increased in 13 cases, and in 12 of these the cytotoxic effect of the T lymphocytes was inhibited by preincubating the liver cells with IgG containing antibodies to the hepatitis B core antigen (HBcAg). Normal human IgG and IgG containing antibodies to the hepatitis B surface antigen (HBsAG) were without effect. Control experiments using autologous fibroblasts as target cells showed low levels of T cell cytotoxicity and no blocking effect of anti-core antibody. All patients in whom it was possible to demonstrate HBcAg in liver tissue had significantly increased T cell cytotoxicity to autologous hepatocytes. These studies suggest that T cell cytotoxicity in patients with chronic HBV infection is directed against determinants resembling the hepatitis B core antigen on the plasma membrane of hepatocytes.

Adolescent↗
PubMed1982

Budd-Chiari Syndrome: clinical patterns and therapy.

Retrospective analysis of 36 patients (25 women, 11 men) with the Budd-Chiari syndrome diagnosed between 1971 and 1980 showed a wide range in aged at presentation (12 to 68 years) with the peak incidence in the third decade for women and in the fourth for men. The 11 patients below the age of 30 were women and six of these had been taking oral contraceptive preparations. There was also a wide range in duration of illness before establishment of diagnosis ranging from eight weeks or less in 20 patients, to up to six years in the other 16. Those in the former group had a high incidence of the 'classical' clinical features (tender hepatomegaly with ascites) and most severe abnormalities in liver function tests. Liver scintiscanning showed the characteristic pattern of maximum colloid uptake in the caudate lobe in only 46 per cent of patients. Mistaken diagnosis in eight cases led to early exploratory laparotomy with serious deterioration in four. One year survival of 58.3 per cent was unrelated to age, sex, or aetiology and little benefit was seen in the few cases treated early with fibrinolytic agents. Of the surgical measures employed only hepatic transplantation has proven worthwhile with three out of four cases alive at 14, 16 and 52 months respectively from the time of operation.

Adolescent↗
PubMed1981

Hepatic complications of cystic fibrosis.

74 patients with cystic fibrosis aged 1-19 years were assessed prospectively for 1-7 years for evidence of liver involvement. 20 of these patients were referred primarily because of hepatic problems. 3 of 4 with neonatal hepatitis recovered. Chronic active hepatitis developed in a further child but resolved spontaneously. 6 patients had abnormal liver-function tests without clinical evidence of liver disease. In 18 cirrhosis was detected at age 4-13 years. Liver disease was stable in these except terminally in 3 with cor pulmonale. The principal hepatic problem was variceal bleeding, which occurred in 6 patients. 50% of bleeds followed aspirin ingestion. This drug therefore should be avoided in such patients. 13 had hypersplenism. 2 had severe splenic pain necessitating splenectomy with lienorenal shunt, which was performed also in 2 patients who had bled. 3 remain well up to 5 years later. In 3 patients seen in the past 3 years injection sclerotherapy has controlled bleeding. This technique was well tolerated without the pain associated with, or intensive physiotherapy necessary after, shunt surgery; and this may be the method of choice for controlling variceal bleeding in cystic fibrosis.

Adolescent↗
PubMed1981

Relationship between serum alpha-foetoprotein, cirrhosis and survival in hepatocellular carcinoma.

An analysis of survival time of 57 West European patients with hepatocellular carcinoma was carried out to define which of several possible factors (age, sex, cirrhosis and raised serum alpha-foetoprotein (AFP)) influenced survival. Although survival was significantly longer in younger patients (P less than 0.02) and in patients with normal serum AFP (P less than 0.01), multivariate analysis showed that significant variation in survival time is better explained by the single factor, the presence of cirrhosis, than by AFP level. This does not seem to apply for patients with this tumour in Africa and the Far East, and there may be a fundamental difference in the natural history of the tumour between high- and low-incidence areas.

Adolescent↗
PubMed1981

Autoimmunity to a liver membrane lipoprotein and liver damage in alcoholic liver disease.

Antibodies reacting with a liver membrane lipoprotein (LSP) have been detected by radioimmunoassay in the sera of 15 (27%) of 55 patients with alcohol-related liver lesions. There was a close association between the presence of the anti-LSP antibody and the findings on liver biopsy of a lymphocytic infiltrate in the portal tracts together with piecemeal necrosis of periportal hepatocytes. These histological features are characteristically found in the autoimmune disorder of chronic active hepatitis, in which anti-LSP antibodies are almost invariably present. It is suggested that in these cases of alcoholic liver disease there is loss of tolerance, and continued production of anti-LSP could promote periportal inflammation and accelerate the progression to cirrhosis. In the cases of acute alcoholic hepatitis without periportal inflammation studied, anti-LSP was not detected demonstrating that production of this autoantibody is not simply secondary to liver damage.

Autoantibodies↗
PubMed1981

Hepatocellular carcinoma in HBsAg-negative chronic active hepatitis.

The occurrence of hepatocellular carcinoma in HBsAg-negative chronic active hepatitis is described in two patients. signs of the tumour appeared 18 years and nine years after the first clinical signs of chronic active hepatitis, although in one patients analysis of stored sera showed the serum alpha-fetoprotein levels had been rising over the previous 33 months. Female preponderance in HBsAg-negative chronic active hepatitis may partly account for the rarity of this complication, and the occurrence of hepatocellular carcinoma may also be a reflection of the better survival now being obtained in HBsAg-negative chronic hepatitis.

Adolescent↗
PubMed1981

Reactivation of hepatitis B virus infection in two patients: immunofluorescence studies of liver tissue.

Two patients, 1 with chronic active hepatitis, the other with Goodpasture's syndrome, became HBsAg-positive after the start of immunosuppressive drug therapy. At the same time of presentation, sera contained anti-HBc antibodies of IgM and IgG class in the absence of HBsAg or anti-HBs and immunofluorescent studies of liver biopsy specimens showed core antigen in the nuclei of liver cells. It is suggested that these findings reflected an unusual pattern of hepatitis B virus infection with a very low level of viral replication and that the appearance of HBsAg in both serum and liver cells after immunosuppressive drug therapy was due to reactivation of this infection, possibly as a result of changes in levels of antibodies which suppress viral replication. In 1 patients, the change in viral replication was associated with continuing liver cell necrosis and progressive deterioration in liver function.

Anti-Glomerular Basement Membrane Disease↗
PubMed1980

Oral-contraceptive-associated liver tumours: occurrence of malignancy and difficulties in diagnosis.

Seven of ten women with oral-contraceptive-associated liver tumours were found to have hepatocellular carcinoma. The diagnosis was often delayed, although hepatomegaly was always present on examination, and liver-function tests and erythrocyte sedimentation-rates were abnormal in most cases. Other investigations, including routine technetium liver scans and biopsy, were sometimes misleading. There were important differences in alpha-fetoprotein concentration, vascularity on angiography, and survival between liver tumours in pill users and non-users.

Adenoma↗
PubMed1980

Evidence for hepatitis B and other virus infection in HBsAg-negative chronic active hepatitis.

Antibody to the hepatitis B core antigen (anti-HBc) was detected in sera from 6 (19%) of 32 patients with HBsAg-negative chronic active hepatitis. In three cases with the highest anti-HBc titers, core antigen was detected in liver cell nuclei and in one case HBsAg was also present in hepatocytes, suggesting continuing hepatitis B virus infection. During follow-up, anti-HBc titers fell slowly in those with no viral antigens in liver tissue, and in two cases with virus in the liver at presentation, viral antigens were no longer demonstrable four and eight years later. In one case, clearance of virus was preceded by the appearance of HBsAg in liver and serum, suggesting reactivation of viral replication. In three of the anti-HBc-negative cases a nuclear antigen unrelated to the hepatitis B virus was detected by immunofluorescence, and it is possible that liver disease in these patients may be related to persistent non-A, non-B virus infection.

Adult↗
PubMed1980

Acute liver disease with encephalopathy and renal failure in late pregnancy and the early puerperium -- a study of fourteen patients.

Fourteen patients, aged 18 to 38 years, presented in the last trimester of pregnancy, or immediately post partum, with severe acute hepatic dysfunction. Liver biopsy confirmed the presence of acute fatty liver in six patients; the cause of hepatic dysfunction was presumed viral hepatitis in five patients, pre-eclampsia in two, and gram-negative septicaemia in one. There was one set of twins and the perinatal mortality was 33 per cent; there were three maternal deaths (21 per cent). Two survivors with acute fatty liver subsequently had successful pregnancies without evidence of hepatic dysfunction.

Acute Disease↗