PubMed Health⌕ Search

Biomedical subjects

B Portmann

Publications and source records attributed to B Portmann.

At least 127 records · Page 7Linked to original sources
PubMed1990

Ground-glass hepatocytes with Lafora body like inclusions--histochemical, immunohistochemical and electronmicroscopic characterization.

We report two adult patients whose liver biopsy specimens revealed numerous ground-glass hepatocytes due to inclusions resembling Lafora bodies. The inclusions were large, intracytoplasmic, pale, eosinophilic and kidney-shaped and were periodic acid-Schiff positive and HBsAg negative. Immunoperoxidase studies showed that the inclusions were positive for cytokeratins and alpha 1-antitrypsin. In case 1, the inclusions were not membrane-bound and consisted of secondary lysosomes and degenerate organelles including rough and smooth endoplasmic reticulum. In case 2, electronmicroscopy showed the inclusions were not membrane-bound, but consisted of dense granules, fibrils and vacuoles, with appearances very similar to Lafora bodies. Neither patient had myoclonus or epilepsy. Electronmicroscopy is important in differentiating the type of Lafora body like inclusions found in liver biopsies.

Adult↗
PubMed1990

Acute liver failure induced by carbamazepine.

Two children developed acute liver failure while taking carbamazepine. Clinical and laboratory findings suggested an immunoallergic reaction, but only one child improved on steroids. Determination of liver function during the first few weeks of treatment and early detection of signs of idiosyncrasy may prevent this rare but severe complication.

Carbamazepine↗
PubMed1990

Hepatitis B virus (HBV) infection and liver disease after allogeneic bone marrow transplantation: a report of 30 cases.

Among 145 consecutive patients undergoing bone marrow transplantation (BMT) for leukemia or aplastic anemia. 30 (21%) were found positive for hepatitis B surface antigen (HBsAg) in serum either before or after BMT. Their serologic profile and clinical outcome are described. Nine out of 30 patients were HBsAg positive before BMT: four were chronic carriers and five were found HBsAg+ at transplant. Three of the former and one of the five latter patients remained persistently HBsAg+ after transplant with signs of liver disease; none developed liver failure, indicating that HBsAg positivity is not an absolute contra-indication to BMT. Among the remaining 21 patients. HBsAg was detected early (n = 12) or late (n = 9) after transplant. All 21 cleared the antigen during follow-up and liver disease was either mild and asymptomatic (nine cases) or clinically overt (12 cases), but none had life-threatening liver disease. Several HBV-infected patients were constantly seronegative for antibody to HBcAg even in the presence of active HBV replication. These results show that the serologic pattern of HBV markers in BMT patients is unpredictable. HBV infection was rarely associated with severe hepatitis and HBsAg carriage.

Adolescent↗
PubMed1989

Viruslike particles in liver in sporadic non-A, non-B fulminant hepatitis.

In a patient who followed the typical clinical course of fulminant hepatitis attributable to "sporadic" non-A,non-B (NANB) hepatitis and who finally received treatment by orthotopic liver grafting, three, apparently separate, virus-like agents (26, 45, and 80 nm) and cytoplasmic, reticular tubular structures (CTS) were identified in collapsed and regenerating areas of liver using electron microscopy. The 80-nm particles present within vacuoles, together with the finding of intranuclear rods in association with the smaller particles (26 nm), are similar to those found in the nuclei of cells infected with several different arboviruses. The third type of particle, existing as 45-nm spheres and rods, is similar in morphology only to some form of polyoma virus, which, hitherto, has not been reported as affecting the liver. Unlike typical polyoma virus, replication of the virus "cores" (25-26 nm) was extranuclear and appeared to be occurring in vacuoles. Although analysis for serological markers against a representative panel for arboviruses, flaviviruses, phleboviruses, arenavirus, and nairovirus was negative, an insect vector was implicated in the clinical history.

Adult↗
PubMed1989

Toga-like virus as a cause of fulminant hepatitis attributed to sporadic non-A, non-B.

Virus-like particles (60-70 nm) with spiked surfaces budding into cell vacuoles and rod-shaped inclusions were detected in nuclei of hepatocytes from a British patient transplanted for sporadic non-A, non-B fulminant hepatitis (NANB-FHF), probably contracted in Kenya. Identical particles were seen in two successive grafts (days 2 and 10) at regrafting for recurrent FHF. Ultrastructural features resembled those of the RNA-containing arbovirus, Rift Valley fever virus, but serological markers against a representative panel for arboviruses (Togaviruses) and transmission in mice proved negative. The particles shared features with the different arboviruses seen in the hepatectomy specimen of a second patient with NANB-FHF, and in both patients an insect vector was implicated in the clinical history. The particles were identical in size to those of a third patient with NANB-FHF, who had remained in the United Kingdom. These findings, together with the recent report of isolation of an RNA-containing virus resembling the Togaviridae, in parenteral NANB, suggest that several exotic virus-like agents resembling the arboviruses may be involved in the aetiology of NANB, including in the sporadic forms of FHF in the United Kingdom.

Adolescent↗
PubMed1989

Evidence for disease recurrence after liver transplantation for primary biliary cirrhosis. Clinical and histologic follow-up studies.

Twenty three patients with primary biliary cirrhosis surviving for greater than 1 yr after liver transplantation were studied. All reported marked symptomatic improvement, and had significant falls in serum bilirubin, alkaline phosphatase (p less than 0.0001), immunoglobulin M, and antimitochondrial antibody levels (p less than 0.005). Beyond 1 yr, liver biopsies showed features compatible with disease recurrence in 9 of 10 patients, and a further 4 patients developed pruritus or associated abnormalities. Immunoglobulin M levels were raised in 80%, with elevated antimitochondrial antibody titers in all those tested. Cyclosporine treatment in some patients initially given prednisone and azathioprine was followed by regression of histologic abnormalities. Of 102 patients with nonprimary biliary cirrhosis followed similarly, 50 underwent biopsy, and although 12 showed features of bile duct damage, all had additional histologic and clinical changes supporting an alternative diagnosis. These findings are consistent with previous reports that primary biliary cirrhosis can recur after transplantation, possibly modified by the use of cyclosporine.

Autoantibodies↗
PubMed1989

The application of quantitative cytochemistry to study the acinar distribution of enzymatic activities in human liver biopsy sections.

The zonal distribution of enzyme activities was measured by quantitative cytochemistry in cryosections of liver from three normal children and five infants with idiopathic hepatitis of infancy. Optimal conditions for cytochemical reactions were first validated in rat liver and subsequently used in human livers to quantify zonal activities of acid phosphatase (AP), succinate dehydrogenase (SDH), glutamate dehydrogenase (GDH), glucose-6-phosphatase (G6P) and NADPH-dehydrogenase (ND). In normal rat and human livers, activities were greater for SDH and G6P in periportal and for GDH and ND in perivenular hepatocytes, while AP was evenly distributed along the sinusoids. In five infants with idiopathic hepatitis of infancy (IHI), a similar trend of distribution was observed for the two mitochondrial (SDH and GDH) and the two microsomal (G6P and ND) enzymes, although the distribution gradient was less pronounced than, in normal livers. AP showed a mildly greater periportal than perivenular activity. This preliminary study shows that a similar metabolic zonation exists for these enzymes in human livers as is observed in rats.

Acid Phosphatase↗
PubMed1989

Developmental changes in the intra-acinar distribution of succinate dehydrogenase, glutamate dehydrogenase, glucose-6-phosphatase, and NADPH dehydrogenase in the rat liver.

Hepatocytes differ in their metabolism depending on their position in the liver acinus. To assess how such specialization changes during development, we used quantitative cytochemistry to measure succinate dehydrogenase (SDH), glutamate dehydrogenase (GDH), glucose-6-phosphatase (G6P), and NADPH dehydrogenase (ND) activities specifically in periportal and perivenular hepatocytes in developing rats, aged between 1-114 days. Important and distinct changes were observed in each zone for each enzyme during development. An intra-acinar gradient of distribution was present from day 1 for SDH and G6P and from day 5 for GDH and ND. It was being similar to the adult value for SDH but less pronounced for the remaining enzymes. The SDH and G6P activity was greater in periportal cells, and the GDH and ND activity was greater in perivenular cells. The more pronounced distribution with age was due, for G6P, to an initial specific periportal increase combined with a mild perivenular decrease and for GDH to a greater perivenular than periportal increase. The ND first increased simultaneously in both zones, but from day 20 the perivenular increase became prevalent. The SDH changes were parallel in both zones. All zonal enzyme activities changed distinctly after weaning. To what extent the changes in activities and metabolic zonation observed in our study reflect a response to specific metabolic demands of the liver or can be modified by environmental factors remains to be investigated.

Animals↗
PubMed1989

Predictability before transplant of hepatic complications following allogeneic bone marrow transplantation.

This study was undertaken to evaluate the occurrence of VOD and other liver diseases following BMT in a patient population with a high incidence of hepatitis before conditioning regimen. We prospectively reviewed 186 consecutive patients undergoing BMT from 1976 to 1986 to determine incidence and type of liver disease after BMT and predisposing factors. Two of 186 patients experienced VOD (1.07%). Acute and chronic liver GVHD were found in 25.8% and 36% of the patients, respectively. Acute hepatitis (AH) was diagnosed in 29.4% and chronic hepatitis (CH) in 42.6% of the patients. Statistical analysis showed no influence of pretransplant variables on the occurrence of acute GVHD and AH; there was a weak correlation (P = 0.01) between pre-BMT abnormal transaminases and occurrence of chronic GVHD. Contingency table and Cox analysis showed a greater risk of CH for patients with abnormal pretransplant SGPT levels (P = 0.0004 and P = 0.0022). No other variables could be associated with posttransplant CH. Actuarial survival was 71% versus 69% for patients with normal versus abnormal transaminases (P = 0.2). As VOD was a rare event, despite 53% of patients having abnormal transaminase values before transplant, we suggest that a lower and slower TBI is more important than pretransplant normal transaminases in preventing this complication. We conclude that evidence of compensated hepatitis is not a relative contraindication for BMT.

Actuarial Analysis↗
PubMed1989

Hepatic morphology and histochemistry of Wilson's disease presenting as fulminant hepatic failure: a study of 11 cases.

Eleven cases of Wilson's disease presenting as fulminant hepatic failure were analysed retrospectively to determine the specificity or otherwise of the histological findings. All cases were cirrhotic, eight with a micronodular pattern. There was marked parenchymal collapse with ductular proliferation and mild inflammation. Other features included cholestasis, hepatocyte necrosis, microvesicular fat and nuclear vacuolation. Orcein staining demonstrated copper-associated protein in the periphery of cirrhotic nodules in all cases and also variably within nodules in eight cases. Copper was demonstrable by the rhodanine method in similar locations but the staining reaction was qualitatively weaker in all cases. Characteristically, there was staining of both parenchymal and mononuclear phagocytic cells. This triad of cirrhosis, strong copper-associated protein deposition and copper positivity was not present in a control group of 20 cases of fulminant hepatic failure of other aetiology and with a similar clinical presentation. It is concluded that in the clinical context of fulminant hepatitis the presence of cirrhosis should raise the suspicion of Wilson's disease and that, with routinely processed and stained tissue, including autopsy tissue, the diagnosis can be made histologically.

Adolescent↗
PubMed1989

Malignant epithelioid haemangioendothelioma of the liver: a clinicopathological and histochemical study of 12 cases.

We describe the clinicopathological findings in 12 cases of hepatic epithelioid haemangioendothelioma in order to identify diagnostic and prognostic features of this unusual vascular neoplasm. Three main tumour patterns were observed histologically: (1) a peripheral pattern with neoplastic cells scattered between fairly normal liver cell plates; (2) a cellular pattern showing a confusing admixture of pleomorphic tumour cells and atrophied hepatocytes set in a small amount of fibrous stroma; and (3) a scarred pattern with sparse tumour cells in a dense fibrous matrix. There were two types of vascular invasion: tuft-like intravascular proliferations of epithelioid cells and fibro-thrombotic venous occlusions. Awareness of these different aspects is important, as they are variably sampled by biopsy needles. The clinical course in this series was less favourable than that previously reported. Eight patients have died, in six instances of liver failure within 4-41 months of diagnosis. Extensive involvement of both lobes of the liver heralds imminent hepatic failure. The slow growth of metastases may justify liver transplantation in order to prolong life.

Adolescent↗
PubMed1989

The fetal liver in PiZZ alpha-1-antitrypsin deficiency: a report of five cases.

The lack of information on the state of fetal liver in PiZZ alpha-1-antitrypsin (AAT) deficiency and a single case report claiming a hypoplasia of interlobular bile ducts in a 20-week PiZZ fetus, instigated this histologic study of the liver in five PiZZ fetuses, 17-20 weeks of gestation and five age-matched controls. We found no difference between the percentage of portal tracts with identifiable bile ducts in the PiZZ (median 22.2%, range 21%-23%) and in the control (median 21.4%, range 20%-24%) on hematoxylin- and eosin-stained sections. Immunostaining with AE1, a monoclonal antibody to cytokeratins restricted to normal bile ducts, doubled the number of recognizable ducts in both PiZZ and control livers. In four PiZZ livers, but in none of the controls, granular deposits of AAT could be detected by specific immunoperoxidase staining. We conclude that an apparent paucity of interlobular bile ducts is normal in the 20-week fetal liver, and our data may be taken as reference for future study dealing with similar material. Except for the cytoplasmic deposition of granules immunoreactive to AAT antiserum, there was no evidence of any developmental anomaly, in particular of the bile duct system in these five PiZZ fetal livers.

Antibodies, Monoclonal↗
PubMed1989

Acute liver decompensation on withdrawal of cytotoxic chemotherapy and immunosuppressive therapy in hepatitis B carriers.

Five chronic carriers of hepatitis B virus developed a fulminant hepatitis-like picture when immunosuppression or cytotoxic treatment, given for unrelated disorders, was withdrawn. Viral replication at the time of the final illness was confirmed in three of the five cases by measurement of serum HBV DNA or the presence of HBc antigen on liver biopsy. A cytoplasmic and nuclear pattern of HBc was seen in histological material during life, but at post-mortem was limited to a nuclear distribution, suggesting greater destruction of hepatocytes containing cytoplasmic HBc. In two of the cases, chronic liver disease was found at post-mortem, there being no previous clinical or laboratory abnormality, but it is unlikely that this was a factor in the development of the superimposed fulminating hepatitis-like illness. Immunosuppressive and cytotoxic agents must be used with extreme caution in any hepatitis B carrier, as withdrawal can precipitate acute decompensation regardless of whether or not there is underlying chronic liver disease.

Adult↗
PubMed1988

Cytomegalovirus infection and donor/recipient HLA antigens: interdependent co-factors in pathogenesis of vanishing bile-duct syndrome after liver transplantation.

The contribution of cytomegalovirus (CMV) infection and its interrelation with HLA antigens in the development of chronic rejection (vanishing bile-duct syndrome--VBDS) was investigated in 101 patients surviving for at least 3 months after liver transplantation. A 1-2 antigen match for HLA DR antigens (30.9% vs 4.5% for zero DR match; p less than 0.002), a zero match for HLA A/B antigens (27.5% vs 10.9% for 1 or more A/B match; p less than 0.05), and active CMV infection (26.3% vs 4.4% for no CMV infection; p less than 0.005) were independently associated with an increased risk of VBDS. The coexistence of a 1-2 HLA DR match and CMV infection carried the highest relative risk (10.1) of VBDS; these two variables were probably interdependent since either alone was associated with a low relative risk (0.45 and 0.5). The association between VBDS and active CMV infection was not a consequence of alterations in immunosuppressive therapy. The findings would be consistent with precipitation of chronic rejection by CMV-induced HLA antigen expression in patients rendered susceptible by the donor/recipient HLA antigen match.

Adolescent↗
PubMed1987

Evidence for an immune response to HLA class I antigens in the vanishing-bileduct syndrome after liver transplantation.

The relation between donor and recipient status for HLA class I and II antigens in 62 patients undergoing liver transplantation was examined with particular reference to a well-defined variant of chronic rejection, the vanishing-bileduct (VBD) syndrome. A complete mismatch for class I antigens was more common in those with the VBD syndrome than in those with normal graft function or chronic graft malfunction unrelated to the syndrome (p less than 0.025). In contrast, a complete mismatch for class II antigens was considerably less common in those with the VBD syndrome than in those without (p less than 0.02). The association of a complete mismatch for class I and a partial or complete match for class II antigens with the VBD syndrome was highly significant (p less than 0.0005). These findings support the hypothesis that in the VBD syndrome both class I antigen expression on bileduct epithelium and immunological interaction at the level of class II antigens are required for the rejection process to occur. In addition, high-titre donor-specific antibodies to class I antigen, which were present in 6 of 14 of those with the VBD syndrome but in none of those without (p less than 0.0005), may be involved in the pathogenesis of bileduct damage.

Adolescent↗
PubMed1987

Microbial structures in a patient with sporadic non-A, non-B fulminant hepatitis treated by liver transplantation.

Double-shelled virus-like particles (60 nm) and long cytoplasmic tubular structures were found in the cytoplasm of hepatocytes from areas of collapsed and regenerating areas of hepatectomised liver in a 13-year-old boy who received a liver graft for fulminant hepatitis attributed to sporadic non-A, non-B hepatitis. The patient died on the ninth postoperative day from acute graft failure. Although virus-like particles were not found, instead, gram-negative rods were identified in the necrotic graft and the most likely cause of death was a gram-negative septicaemia with a Shwartzman-like reaction localized to the liver.

Adolescent↗
PubMed1987

Normal venous circulation of the gastroesophageal junction. A route to understanding varices.

A study into the normal anatomy of the venous circulation of the gastroesophageal junction was undertaken using three complementary techniques (radiology, corrosion casting, and morphometry). Four distinct zones of venous drainage were defined as follows: (a) gastric zone, characterized by a longitudinal venous distribution; (b) palisade zone, composed of parallel vessels arranged in groups, lying mainly within the lamina propria; (c) perforating zone, characterized by "treble clef" shaped veins, which collect and channel blood into extrinsic veins; and (d) truncal zone, composed of four or five deep lying descending veins. This venous system appeared to be mainly distributed within the esophageal mucosal folds. The anatomic pattern suggests that venous flow is bidirectional at the palisade zone, which acts as a high-resistance watershed region between the portal and azygos systems. In patients with portal hypertension this normal vascular system has to accommodate greatly increased venous flow, and the anatomy as demonstrated here offers insight into variceal development.

Aged↗