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Biomedical subjects

B Portmann

Publications and source records attributed to B Portmann.

At least 199 records · Page 11Linked to original sources
PubMed1980

Extrahepatic biliary atresia: preoperative assessment and surgical results in 47 consecutive cases.

Of 47 consecutive infants with extrahepatic biliary atresia, effective bile drainage with the return of the serum bilirubin concentration to normal, was achieved in 17 (38%). Direct bile duct-to-bowl anastomosis, attempted in 15 infants, produced bile drainage in only those 4 (9%) in whom bile could be seen within the bile duct remnants at laparotomy. 13 (45%) of 29 infants subjected to portoenterostomy (direct liver-to-bowel anastomosis) had satisfactory prolonged bile drainage with normal serum bilirubin values. Although a correct preoperative diagnosis was made in each case, in 3 (6%) the 72-hour faecal rose bengal 131I excretion was greater than 10% of the injected dose, and in 5 (11%) the hepatic histology did not indicate bile duct obstruction, showing that both investigations are necessary for preoperative diagnosis. Preoperative clinical, laboratory, and hepatic histological features in the 16 jaundice-free survivors showed no significant difference when compared with the 31 infants in whom surgery was successful. Cholangitis occurred in only 7 (43%) of 16 infants with satisfactory bile drainage and was easily controlled with antibiotic treatment. No cutaneous enterostomies were performed. In most survivors liver function tests remain abnormal, but the patients are symptom-free. While it is too early to predict a long-term prognosis for these children, our eldest survivors are healthy and show normal development.

Bile Ducts↗
PubMed1980

Antibodies to a human liver membrane lipoprotein (LSP) in primary biliary cirrhosis.

Antibodies reacting with a human liver-specific membrane lipoprotein (LSP) have been detected using a sensitive and specific radioimmunoassay in 19 (51%) of 37 patients with primary biliary cirrhosis. The anti-LSP antibodies were found only in the later stages of the disease as judged by histological criteria, being present in 73% of those in stage IV, 44% of those in stage III, and none of those in stage I or II. Although there was no relationship between percentage binding and standard liver function tests, there was a close correlation between percentage binding of 125I-LSP by serum and the extent of piecemeal necrosis of periportal hepatocytes on liver biopsy. The timing of the anti-LSP response makes it very unlikely that it is involved in the pathogenesis of the early bile duct damage but the results of this and other studies suggest that antibodies to this hepatocyte membrane lipoprotein may be an important cause of periportal liver cell necrosis in both primary biliary cirrhosis and chronic active hepatitis and could be one of the factors determining progression to cirrhosis in both these conditions.

Adult↗
PubMed1980

A fatal case of Epstein-Barr virus infection with jaundice and renal failure.

A fatal case of Epstein-Barr virus infection in a 17-year-old male is described. The patient presented with an illness clinically typical of infectious mononucleosis but death followed development of renal failure, jaundice and pulmonary failure. There was no absolute lymphocytosis nor a significant number of atypical mononuclear cells in his peripheral blood. However, heterophile antibody and Epstein-Barr virus-specific IgM were present.

Acute Kidney Injury↗
PubMed1980

Observer variation in assessment of liver biopsies including analysis by kappa statistics.

In an observer variation study, six histopathologists independently coded 27 features and final diagnoses on the same 60 liver biopsies without knowledge of clinical data. Many features were shown by kappa statistics to have significantly high agreement, and differences were shown to be due in part to differences in definitions of particular features. Intraobserver variation was much lower than interobserver variation. Full agreement with respect to the final histopathologic diagnosis between the six observers occurred in only nine instances--all of these were cases of alcoholic liver disease. Some of the biopsies of acute hepatitis were confused with chronic active hepatitis, while others were mistaken for cholestasis, usually believed to have been related to drugs. Biopsies from patients with extrahepatic biliary obstruction were most commonly misdiagnosed as drug cholestasis. However, agreement was higher when the results were calculated in terms of observer pairs by comparing the final diagnosis of each observed with that obtained from each of the other five observers. The most striking finding here was the very high agreement rate (80%) between the two specialist pathologists compared with that obtained from the remaining fourteen pairs of observers who averaged an (43%) agreement rate.

Biopsy↗
PubMed1979

De novo acute infection and reactivation of hepatitis B virus in established cirrhosis.

Five patients with cirrhosis proved by biopsy had clinical, biochemical, and serological evidence of an acute hepatitis B infection. In two the illness was fulminant and led to death. Only one patient completely recovered. Serological markers for the hepatitis B virus were absent before the onset of the acute illness in four patients, which suggested that a de novo infection had been acquired as a result of recent transfusions of blood or blood products. The fifth patient, who had Goodpasture's syndrome, had antibody to the core of hepatitis B virus, indicating previous exposure to the virus; his acute hepatitis may have been related to immunosuppressive drug treatment, which may have reactivated a dormant virus infection. Thus an acute type B viral hepatitis due to either a de novo or a reactivated infection may be superimposed on cirrhosis.

Acute Disease↗
PubMed1979

Increased hepatic copper concentration in Indian childhood cirrhosis.

19 Indian children with liver disease were studied. 5 in whom a clinical and histological diagnosis of Indian Childhood Cirrhosis was made had massive orcein-staining deposits in liver cells. The hepatic copper content in these 5 cases was strikingly high (1389 microgram/g dry tissue, range 1045--2303) the normal range being 15--55 microgram/g. Of the other 14 cases, only 2 had hepatic copper levels above normal (170 and 262 microgram/g.) This high hepatic copper concentration may be caused by excessive copper ingestion or an abnormality of copper metabolism.

Coloring Agents↗
PubMed1979

Transmission of non-A non-B hepatitis to chimpanzees by factor-IX concentrates after fatal complications in patients with chronic liver disease.

6 cases of non-A non-B hepatitis which followed administration of four different batches of concentrates of coagulation factor IX from commercial and non-commercial sources are described. Of 17 patients who received the concentrate on account of chronic liver disease, 4 developed hepatitis, and in 3 of these the illness proved fatal. The incubation periods ranged from 42 to 103 days (mean 65 days). 3 chimpanzees were inoculated with concentrate from the same batch used on the above patients, a further commercial batch upon which no adverse reactions had been reported, and plasma from a known non-A non-B carrier. All developed hepatitis after 10 weeks' incubation. Liver biopsy when serum-aminotransferase was at its highest level showed features consistent with acute hepatitis. As in the patients, viral markers for hepatitis A and B, cytomegalovirus, and Epstein-Barr virus were unchanged.

Acute Disease↗
PubMed1979

Hepatitis from dantrolene sodium.

The clinical course and histological changes in the liver during a presumed adverse reaction to the drug dantrolene sodium are described in four patients. After a typical prodrome one developed a moderately severe hepatitis-like illness. Another also had a prodrome but never became jaundiced. In the other two, abnormal liver function tests were detected on routine screening. In each case liver biopsy showed changes typical of an acute hepatitis, but the severity was unrelated to the clinical presentation. In addition, there were also changes in the portal tracts resembling ascending cholangitis. In each case liver function tests returned to normal after withdrawing treatment with dantrolene.

Aged↗
PubMed1979

Hepatocellular carcinoma in primary biliary cirrhosis: report of four cases.

Of 98 patients dying with primary biliary cirrhosis only four developed hepatocellular carcinoma. It is suggested that the development of hepatocellular carcinoma is uncommon in this type of chronic liver disease because of its known female preponderance, and the fact that cirrhosis develops late in the course of the illness.

Carcinoma, Hepatocellular↗
PubMed1979

Lymphocyte cytotoxicity to autologous hepatocytes in HBsAg-negative chronic active hepatitis.

In a micrototoxicity assay, lymphocytes from ten out of sixteen patients with HBsAg-negative chronic active hepatitis have been shown to be cytotoxic to autologous hepatocytes isolated from percutaneous liver biopsies. This cytoxicity was demonstrable in all six untreated patients but in only four out of ten receiving immunosuppressive treatment, the presence of cytotoxicity showing a significant association with the activity of the disease assessed histologically. The addition of excess purified lipoprotein (LSP), derrived from the hepatocyte plasma membrane, blocked the reaction in all cytotoxic cases, indicating that LSP was the major target antigen. Enriched fractions of T cells were cytotoxic in only one case, whereas non-T cell fractions were cytotoxic in the other ten cases investigated in this way. For optimum T cell cytotoxicity, effector and target cells must share histocompatibility determinants and the results of this study using an autologous system show conclusively that the lymphocyte cytotoxicity found in HBsAg-negative chronic active hepatitis is mediated by a non-T cell population.

Adult↗
PubMed1979

Liver disease as presenting manifestation of Hodgkin's disease.

Six patients who were referred to the liver unit on account of jaundice are described. A different initial diagnosis has been made in each case, these being fulminant hepatic failure, severe hepatitis with renal failure, toxoplasma hepatitis, extrahepatic obstruction, sclerosing cholangitis, and liver abscess. After delays of four weeks to 12 months from the time of first symptoms all six patients were eventually found to have advanced Hodgkin's disease (stage 4). In four patients the diagnosis was made during life, but in two only at autopsy. In four lymphoma tissue was finally demonstrable in the liver, but in two liver biopsy showed only minor non-specific changes despite grossly abnormal liver function tests. Three of the six patients were treated with chemotherapy, and two of these recovered sufficiently to leave hospital. With the encouraging survival figures now being obtained in Hodgkin's disease, an awareness of the varied hepatic manifestations of the disease may allow treatment to be instituted at an earlier stage.

Adult↗
PubMed1978

Alpha-fetoprotein concentrations measured by radioimmunoassay in diagnosing and excluding hepatocellular carcinoma.

Serum alpha-fetoprotein (AFP) concentrations were estimated by sensitive radioimmunoassay in 30 patients with cirrhosis complicated by hepatocellular carcinoma and in 100 patients with cirrhosis in whom malignancy was excluded. Twenty-nine of the 30 patients with hepatocellular carcinoma had concentrations above 10 IU/ml (10.5 ng/ml) (median 3500 IU/ml (3675 ng/ml)), whereas only one of the 100 patients with cirrhosis and no tumour development had a raised concentration. Eleven out of 20 patients in whom hepatocellular carcinoma had developed in an apparently normal liver had raised AFP concentrations. In this group the differential diagnosis is usually secondary carcinoma, and three of 50 such patients had AFP concentrations above 10 IU/ml. Noting raised AFP concentrations is thus of considerable value both in detecting and in excluding hepatocellular carcinoma in cirrhosis, for in this case such concentrations gave only 1% false-positive and 3% false-negative results. They are less useful, however, in distinguishing between primary tumours arising in patients without cirrhosis and secondary hepatic deposits, giving 6% false-positive and 45% false-negative results.

Adult↗
PubMed1978

Renal failure in otherwise uncomplicated acute viral hepatitis.

Twelve patients with otherwise uncomplicated acute viral hepatitis (two were HBsAg-positive) developed renal failure. Apart from dehydration due to repeated vomiting in one patient, no factor responsible for precipitating renal failure could be identified. The clinical course was characterised by renal failure with plasma urea concentrations reaching maximum values of 26-69 mmol/l (175-416 mg/100 ml). Ten patients needed dialysis for up to two weeks. Seven patients recovered completely, while the other five died from sepsis. The types of renal failure were similar to those described in fulminant hepatic failure and cirrhosis--namely, functional renal failure in five patients and acute tubular necrosis in seven. Two of the patients with functional renal failure later developed tubular necrosis. The mechanism responsible for renal failure in acute viral hepatitis is uncertain, though endotoxaemia may contribute.

Acute Disease↗
PubMed1978

Orcein-positive liver deposits in Indian childhood cirrhosis.

A striking, previously unreported pattern of orcein-positive deposits attributed to excess copper-binding protein was found in liver-biopsy specimens from twelve cases of Indian childhood cirrhosis. A comparable picture was found only in biopsy specimens from patients with Wilson's disease. Deposits seen in about 20% of the two hundred and seventy-nine cases in various control groups, which were associated with prolonged cholestasis, were slight in comparison. This finding could have important therapeutic and pathogenic implications.

Child↗