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Biomedical subjects

S Sherlock

Publications and source records attributed to S Sherlock.

At least 253 records · Page 14Linked to original sources
PubMed1978

Hepatic iron clearance from serum in treated hemochromatosis.

Hepatic iron uptake from transferrin has been studied in 7 patients with treated hemochromatosis. When compared with a control group with similar serum iron and transferrin saturation the hemochromatotics had a significantly higher 4-hr iron uptake value. Uptake in the precirrhotic and cirrhotic hemochromatotics was not significantly different, suggesting the existence of a cellular abnormality of hepatic iron metabolism in this disease. The results provide an explanation for the development of siderosis in some patients with idiopathic hemochromatosis and a normal serum iron concentration.

Adult↗
PubMed1978

Liver copper levels in intrahepatic cholestasis of childhood.

Liver copper concentration was studied in four groups of age-matched patients and in 9 patients with Wilson's disease of comparable age. Neutron activation analysis of hepatic copper concentration revealed markedly elevated hepatic copper levels, comparable with those in Wilson's disease, in 13 of 15 patients (P less than 0.001) with intrahepatic cholestasis of childhood. In 1 patient, autopsy confirmed very high liver copper values, whereas other tissues, including brain and kidney, gave normal results. Total plasma copper values were elevated in 11 of 13 patients with intrahepatic cholestasis of childhood (P less than 0.001); 24-hr urinary copper excretion was normal in 7 of 12 patients and slightly elevated in 5 (P less than 0.001). Kayser-Fleischer rings were sought in 10 patients and were absent. Hepatic copper concentration was not accurately predicted by plasma copper or 24-hr urinary copper. However, increased urinary copper excretion was associated with hepatic copper concentration greater than 250 microgram per g. It is concluded that copper metabolism is abnormal in intrahepatic cholestastis of childhood and can be distinguished from Wilson's disease. If hepatic copper concentration is greater than 250 microgram per g, copper chelation therapy should be considered.

Activation Analysis↗
PubMed1977

Is primary biliary cirrhosis an immune complex disease?

Large immune complexes are present in the circulation of patients with primary biliary cirrhosis and result in the activation of complement by the classical pathway. Such large complexes are capable of producing tissue damage. The granulomatous lesions surrounding the small bile-ducts within the liver of patients with primary biliary cirrhosis and the vasculitis, rheumatoid arthritis, and associated lesions are all compatible with immune complex injury. It is postulated that such large complexes could be formed in the vicinity of the bile-ducts by an antigen absorbed from the bile or biliary epithelium. Complexes reaching the systemic circulation might be responsible for the associated extra-hepatic diseases.

Antibody Formation↗
PubMed1977

Grey-scale ultrasonography of portal vein.

The state of the extrahepatic portal venous system was correctly assessed by grey-scale ultrasonography in twenty-one patients with extrahepatic portal-vein obstruction and the results agreed with those obtained by portal venography. In twenty-two age-matched controls a patent portal vein was displayed. The diameter of the portal vein on the ultrasound scan was significantly less in the twenty-two controls than in eighteen patients with chronic liver disease. Grey-scale ultrasonography is a reliable, inexpensive, and non-invasive method for diagnosing extrahepatic portal-vein obstruction.

Adolescent↗
PubMed1977

Fulminant Wilson's disease with haemolysis and renal failure: copper studies and assessment of dialysis regimens.

Two girls, aged 12 and 17 years, presented with hepatocellular dysfunction and severe haemolysis due to Wilson's disease (hepatolenticular degeneration). This was accompanied by acute renal failure. In the absence of renal function sufficient for the urinary excretion of penicillamine, studies were performed to assess the potential of peritoneal dialysis, ascites removal by ultrafiltration-reinfusion, and haemodialysis as alternative excretory pathways for copper. The greatest amount of copper, as judged by rising bath concentrations, seemed to be eliminated with haemodialysis. But this was accompanied by a progressive increase in serum copper concentrations with rapid clinical and biochemical deterioration leading to death within 48 hours. A small amount of copper was lost with ascites removal. Significant amounts of copper were removed during peritoneal dialysis (36 mumol/day (2287 microgram/day)), although a clinical response was not evident before haemodialysis was introduced. The administration of penicillamine orally, intravenously, or intraperitoneally produced no measurable increase in copper excretion into the peritoneal dialysate. Hence peritoneal dialysis alone appears to offer the greatest potential benefit with regard to both eliminating copper and altering the course of this fulminant form of Wilson's disease.

Acute Kidney Injury↗
PubMed1977

Sex-related differences among 100 patients with alcoholic liver disease.

During 1975 we studied 100 patients--77 men and 23 women--who had a history of alcohol abuse and disturbed liver function test results. On presentation the women were less likely to be suspected of alcohol abuse (9; 38%) than the men (59; 77%). Although the quantity of alcohol consumed and length of history of alcohol abuse were similar for men and women, the incidence of chronic advanced liver disease was higher among women (86%) than among men (65%). Women, however, were less likely to have developed primary liver cell cancer. Overall the women had a higher incidence of other alcohol-related disorders and were less likely to stop abusing alcohol (2; 9%) than were their male counterparts (22; 29%). Women seem to be more susceptible to alcohol-related disease.

Adult↗
PubMed1977

25-Hydroxylation of vitamin D in primary biliary cirrhosis.

The ability to 25-hydroxylate vitamin D was investigated in thirty-nine patients with symptomatic primary biliary cirrhosis (P.B.C.). In seven previously untreated patients serum-25-hydroxy-vitamin-D (25-OHD) concentration increased after regular monthly injections of vitamin D. After a single injection of vitamin D in eight P.B.C. patients serum-25-OHD did not change significantly over 12 days; in contrast there were significant increases in eight normal subjects and in seven patients with nutritional osteomalacia. Twenty-three of twenty-five P.B.C. patients on regular vitamin-D therapy had normal serum-25-OHD values. These results indicate that serum-25-OHD concentrations become normal in P.B.C. if adequate amounts of vitamin D are presented to the liver as substrate.

Adult↗
PubMed1977

Adult Niemann-Pick disease: its relationship to the syndrome of the sea-blue histiocyte.

Three unrelated female patients with adult Niemann-Pick disease are described. All the patients had reduced coagulation factors and involvement of the marrow, liver, spleen and lungs. Two patients were shown to have abnormal platelet function; two patients also had pingueculas and a late onset of a menarche. Foam cells and sea-blue histiocytes were seen in the marrow and livers in all three patients, in the spleen in two patients in the lymph nodes in one patient. The clinical presentation, the histologic appearance, the histochemical staining reactions, the lipid analysis and the ultrastructure were all consistent with a diagnosis of adult Niemann-Pick disease. On the basis of these observations, it is clear that adult Niemann-Pick disease is a cause of the syndrome of the sea-blue histiocyte. The existence of the syndrome of the sea-blue histiocyte as a separate entity is also questioned.

Adolescent↗
PubMed1977

Detection, by three techniques, of hepatitis B surface antigen (HBsAg) and determination of HBsAg and anti-HBs titres in patients with chronic liver disease.

The sensitivities of three technqiues used to detect serum hepatitis B surface antigen (HBsAg) were compared in 411 patients with various types of chronic liver disease. Counterimmunoelectrophoresis proved an unreliable test. Two haemagglutination technqiues were slightly less sensitive than radioimmunoassay but were more rapidly performed. Less sensitive techniques were particularly unreliable in active liver disease where HBsAg titres were low. HBsAg was detected in patients with chronic persistent hepatitis, alcoholic liver disease, chronic active liver disease with or without cirrhosis, and primary liver cell carcinoma. Forty-six of the 68 (68%) HBsAg positive subjects were males coming from outside the United Kingdom. The HBsAg titres in 13 subjects with chronic persistent hepatitis were significantly higher (P less than 0-001) than those in 43 subjects with chronic active liver disease. Corticosteroid therapy did not alter the HBsAg titre significantly. None of the 28 HBsAg positive subjects studied serially for up to two years cleared HBsAg from the serum. Anti-HBs was examined by passive haemagglutination and found in 35 subjects, 26 of whom had no evidence of liver disease, 80% came from abroad. Anti-HBs was believed to be of epidemiological rather than of pathological importance.

Antibodies↗
PubMed1977

Chronic pancreatitis: a cause of cholestasis.

The bile ducts were visualised using endoscopic retrograde cholangiopancreatography (ERCP), percutaneous or intravenous cholangiography in 38 patients with non-gallstone chronic pancreatitis. Stenosis of the intrapancreatic portion of the distal common bile duct was demonstrated in 11 patients. Ten of the 11 developed transient cholestasis during exacerbations of their chronic pancreatitis. In six cholestasis eventually persisted requiring surgical relief. Secondary biliary cirrhosis was present in one patient. No evidence of pancreatic carcinoma was found in the patients explored surgically. Ten of the patients are alive more than one year after diagnosis. Chronic pancreatitis was of alcoholic aetiology in 10 of the patients with biliary stenosis. Cholestasis and biliary stricture are common but poorly recognised complications of non-gallstone chronic pancreatitis, especially when pancreatitis is severe and due to alcohol.

Adult↗
PubMed1977

Lymphocyte populations in liver biopsy specimens from patients with chronic liver disease.

The characterisation of lymphocytes from liver biopsies indicates that 'activated' T lymphocytes are present in the liver in alcohol induced hepatitis, chronic active hepatitis (HBS+ve and -ve), and in primary biliary cirrhosis but not in inactive cirrhosis, chronic persistent hepatitis, extrahepatic and drug induced cholestasis. A greater percentage of lymphocytes bear Fc-receptors in chronic active hepatitis than in alcohol induced hepatitis or cholestatic liver disease. The concentration of 'activated' T cells in the peripheral blood in all groups studied was within the normal range, suggesting that the 'activated' T cells found in the liver were reacting to either native or foreign antigens within the liver. The data on Fc-receptor bearing cells are consistent with the involvement of antibody assisted K cell mediated cytotoxicity in chronic active hepatitis.

Binding Sites, Antibody↗
PubMed1977

Assessment of the plasma disappearance of cholyl'l14C-glycine as a test of hepatocellular disease.

The plasma disappearance of a tracer dose of cholyl-l14C-glycine has been examined in 12 control subjects and in 32 patients with hepatocellular dysfunction. Simple analysis of the data did not detect hepatic dysfunction except in severe hepatocellular disease. The greatest degree of discrimination between normal subjects and patients with mild liver disease was obtained by taking the ratio of the plasma retention at 60 minutes to that at 10 minutes; it was similar to that obtained with serum gamma-glutamyl transferase. The two hour post-prandial plasma "total" bile acid concentration gave complete separation between the control subjects and patients with liver disease.

Adult↗
PubMed1977

Reduced oxidase activity in the caeruloplasmin of two families with Wilson's disease.

Caeruloplasmin has been determined immunologically and by coper oxidase activity in 25 normal subjects, 20 patients with Wilson's disease, and 80 patients with chronic liver disease. Repeated estimation in four patients with Wilson's disease and two heterozygous mothers from two families revealed a consistent reduction in the copper oxidase activity of caeruloplasmin (12-32 units copper oxidase activity/mg caeruloplasmin) relative to the values obtained in normal subjects, patients with chronic liver disease, and other Wilson's disease patients (mean 65-79 units/mg). It is suggested that the functional abnormality in caeruloplasmin observed in these two families is an inherited variant, which does not appear to be due to the presence of a serum inhibitor of copper oxidase.

Adolescent↗