Primary polydipsia and autoimmune chronic active hepatitis.
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Biomedical subjects
Publications and source records attributed to R N Macsween.
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Serum liver function tests were estimated in 57 patients admitted to an Intensive Therapy Unit (ITU) with a diagnosis of septic shock. Following an initial biochemical disturbance, persisting hyperbilirubinaemia was associated with a poor prognosis. Post-mortem liver histology in 22 patients showed varying degrees of non-specific reactive change, venous congestion, ischaemic necrosis, fatty change and intrahepatic cholestasis in 16 cases. In the remaining six cases there was moderately severe cholestasis with inspissated bile in the cholangioles. The possible aetiology of the observed cholestasis is discussed.
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Antisera were raised in rabbits against rat peritoneal and alveolar macrophages and against rat thymocytes. In vitro investigations of these antisera using dye-exclusion tests and cytotoxicity and immunofluorescence methods indicated that the immunizing cell types exhibited shared and specific antigens. Extensive absorption studies confirmed these findings, and showed some specificity of the anti-peritoneal serum for peritoneal macrophages and of the anti-alveolar serum for alveolar cells.
Monitoring the incidence of angiosarcoma of the liver (ASL) between 1974 and 1977 has led to the confirmation by a panel of pathologists of 7 new cases of ASL in patients who had received intra-arterial Thorotrast for radiological investigations. A cluster of cases has appeared in and around Edinburgh where the use of Thorotrast was pioneered in Britain in 1933-48, and a mortality study of 113 Edinburgg patients has confirmed a significant excess of liver-tumour deaths in recent years. Deaths from cancers of the lung and breast, and from hepatic cirrhosis, were also in excess, but the limitations of the death-certificate data are described in relation to the clinical and pathological findings. Thorotrast was also used in other centres, and an increased incidence in Britain of liver tumours attributable to this agent is indicated.
The annual occurrence of angiosarcoma of the liver (ASL) in Britain from 1963 to 1977 was studied, including clinical and occupational details for those cases agreed as ASL by a panel of histopathologists. Thirty-five cases (28 men, six women, and one infant girl) were agreed as ASL. The increase in the incidence of ASL observed in recent years was attributable to Thorotrast (thorium dioxide) usage (eight cases) and exposure to vinyl chloride (two cases) in the past. In its clinical presentation and prognosis ASL resembled primary liver carcinoma, except that extrahepatic metastases were found in only eight (23%) cases, and haemoperitoneum ws more common in those cases due to Thorotrast. The results suggested a possible increased risk of ASL in the electrical and plastics fabrication industries, but information on exposure was inadequate to implicate specific chemicals. The clinical features of one case were indicative of arsenical intoxication, but medications in the other patients did not appear to be of aetiological importance.
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.
A two-fold increase in the incidence of hepatocellular carcinoma in the west of Scotland is reported on the basis of a 25-year retrospective necropsy review (313 cases). This increase is not accompanied by a corresponding increase in the incidence of hepatic cirrhosis. The relationship between hepatocellular carcinoma and hepatic cirrhosis is discussed in the light of these findings.
Lymphocyte subpopulations were measured in the peripheral blood of 13 patients with primary biliary cirrhosis (PBC), 13 with chronic active hepatitis (CAH), and 16 age- and sex-matched normal subjects. A significant, relative and absolute, reduction in activated T cells and K cells, compared with that in normal subjects, was found in PBC but not in CAH. The reduction in K cells observed in PBC was found to be accompanied by a parallel decrease in lymphocyte-mediated antibody-dependent cytotoxicity.
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A simple immunofluorescence test for antibody to a mitochondrial antigen present in many tissues is a reliable method of distinguishing most cases of primary biliary cirrhosis from jaundice due to extrahepatic biliary tract obstruction. Of 30 cases diagnosed as primary biliary cirrhosis, 26 had antimitochondrial antibody whereas none of 77 cases with jaundice due to extrahepatic bile duct obstruction showed this serological abnormality. The antibody was also found in the serum of three of 42 patients who had other forms of cirrhosis and in two of 266 patients with no evidence of liver disease.Clinical, biochemical, and serological findings favour the view that primary biliary cirrhosis is a real entity which, in our present state of knowledge, cannot be defined clearly by any single method of investigation. In particular, the liver may show a variety of histological appearances which, interpreted without regard to the other features of the case, may lead to errors in diagnosis.