Accuracy of computed tomography in diagnosis of fatty liver.
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Adult↗
Biomedical subjects
S Sherlock
Publications and source records attributed to S Sherlock.
Primary or secondary sicca complex? Investigation in primary biliary cirrhosis by histocompatibility testing.
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Acidosis, Renal Tubular↗
Primary biliary cirrhosis is a dry gland syndrome with features of chronic graft-versus-host disease.
Primary biliary cirrhosis (PBC) is part of a disease complex characterised by dry eyes, dry mouth, and both biliary and pancreatic hyposecretion: it is a "dry gland" syndrome resulting from damage to ductular epithelium. Additional extraglandular features include scleroderma-like skin lesions, pigmentation, Raynaud phenomenon and severe abnormalities of both humoral and cellular immunity. Identical ductular and extraductal features, including abnormalities of the immune system, are seen in chronic graft-versus-host disease after bone-marrow transplantation. It is suggested that in PBC the ductular lesions and severe disturbance of the immune system including macroglobulinaemia and immune complex formation occur as a result of an immune response to the histocompatibility (HC) complex antigens which are present in high density on ductular epithelial cells of the biliary tree. This response could be caused by altered antigenicity of epithelial cell HC antigens or by failure of the HLA-dependent T cell self-recognition system.
Antigen-Antibody Complex↗
Computed tomography for determining liver iron content in primary haemochromatosis.
Dual-energy computed tomography (CT) was used to estimate hepatic iron concentration in eight patients with primary haemochromatosis with varying degrees of iron overload. The values corresponded closely with these derived from chemical analysis of liver tissue obtained by biopsy (correlation coefficient 0.993). Dual-energy CT therefore seems to provide an accurate and non-invasive alternative to liver biopsy as a means of measuring liver iron concentration in patients with primary haemochromatosis and possibly other iron overload states.
Adult↗
Focal nodular hyperplasia of the liver.
Focal nodular hyperplasia of the liver (FNH) in a fit young Iranian girl of 19 months presented with asymptomatic hepatomegaly. Liver scan and ultrasound examinations demonstrated a space-occupying lesion involving both hepatic lobes. Arteriography revealed a vascular pattern suggestive of liver cell carcinoma with dilated arterial branches and tumour blush on the capillary phase. At laparotomy the large mass involved both right and left hepatic lobes and was not resected. Tissue diagnosis was established by surgical wedge biopsy. FNH should be considered in the differential diagnosis of an hepatic mass in a clinically well child, particularly female, in which the liver biopsy suggests cirrhosis but arteriography favours liver cell carcinoma. If FNH is diagnosed and the lesion is not resected it is recommended that patients be advised against sex hormone therapy as there may be an increased risk of rupture with intraperitoneal haemorrhage.
Biopsy↗
Hyperglucagonaemia in cirrhosis. Relationship to hepatocellular damage.
Plasma glucagon and growth hormone concentrations were measured fasting and after oral glucose in 19 patients with portal vein block with extensive portal-systemic shunting but minimal liver cell damage, 11 cirrhotic patients and 12 matched control subjects. Portal vein block patients and controls had similar fasting glucose and glucagon levels (glucose 3.8 +/- 0.1 mmol/l VS control 3.4 +/- 0.1 mmol/l (mean +/- SEM); glucagon 57.5 +/- 9.1 pg/ml VS control 51.3 +/- 7.8 pg/ml). Cirrhotic patients were hyperglycaemic (cirrhosis 4.3 +/- 0.2 mmol/l VS control 3.4 +/- 0.1 mmol/l, p < 0.01) with significantly elevated glucagon levels (167.3 +/- 61.1 pg/ml VS control 51.3 +/- 7.8 pg/ml, p < 0.05), which suppressed towards control values after oral glucose. There was no correlation between fasting plasma glucagon levels and the degree of portal-systemic shunting in cirrhotic patients. There was a strong correlation between fasting plasma glucagon concentrations and aspartate transaminase levels (r = 0.68; p < 0.01) in cirrhotic and portal vein block patients. Significant elevations of growth hormone were seen only in cirrhotic patients. It is concluded that hyperglucagonaemia is a feature of hepatocellular damage rather than portal-systemic shunting but the relationship between elevated glucagon and growth hormone concentrations and carbohydrate intolerance in cirrhosis remains unclear.
Adolescent↗
A non-transferrin-bound serum iron in idiopathic hemochromatosis.
The nature of iron in the serum of patients with idiopathic hemochromatosis has been studied utilizing an isotope labeling method and results have been compared with those from normal individuals and patients with other forms of liver disease. Between 2 and 4% of a tracer dose of 59Fe added to normal serum was retained by DEAE Sephadex and has been designated non-transferrin-bound. Alcoholic liver disease, chronic active hepatitis, and iron deficiency have no effect on this fraction. In idiopathic hemochromatosis 34.6 +/- 3.9% of the added iron was not bound to transferrin at diagnosis, representing approximately 700 microgram Fe/liter serum. Treatment lowers this fraction before serum iron concentration falls to normal. The majority of the non-transferrin-bound iron is of low molecular weight and is not bound to albumin. The presence of this fraction may contribute significantly to the development of tissue siderosis.
Binding Sites↗
Hair copper in primary biliary cirrhosis.
Rats fed on a copper-enriched diet develop increased liver copper concentrations, which correlate well with hair copper content. It has been suggested that in man, hair copper analysis may be a useful noninvasive method for predicting liver copper concentrations. We have tested this hypothesis in 11 patients with primary biliary cirrhosis, a disease complicated by the accumulation of copper in the liver. Ten patients had increased liver copper concentrations, but only one had increased hair copper. In primary biliary cirrhosis, hair copper does not reflect liver copper content and is of no value as a biopsy material for copper analysis.
Ceruloplasmin↗
The epidemiology of testicular cancer in young adults.
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Adolescent↗
Late results of the Royal Free Hospital prospective controlled trial of prednisolone therapy in hepatitis B surface antigen negative chronic active hepatitis.
A long-term follow-up of at least 10 years or until death of 44 patients taking part in a controlled prospective trial of prednisolone therapy in hepatitis B antigen negative chronic active hepatitis (lupoid hepatitis) has been performed at the Royal Free Hospital, London. Patients presenting between 1963 and 1967 were randomly allocated into control and treatment groups. Ten year life table survival curves showed a significantly improved survival in the treatment group where 63% of patients were alive at 10 years compared with only 27% in the control group (log rank test, P = 0.03). The median survival in the treatment group was 12.2 years compared with 3.3 years in the control group. The mean duration of treatment was 4.5 years. Age, presence of antinuclear factor, cirrhosis, or level of serum transaminases at presentation did not appear to affect survival. Male patients if untreated had a poorer prognosis than females (P = 0.02). The natural history of chronic active hepatitis appeared from clinical, biochemical, and histological findings to be from an active hepatitis or cirrhosis to inactive macronodular cirrhosis. Prednisolone therapy significantly improved survival by reducing mortality in the early active phase of the disease.
Adolescent↗
Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology.
Twenty-nine patients with primary sclerosing cholangitis were reviewed. Males predominated (2:1). Seventy-six per cent presented with cholestasis and cholangitis, 17% with cirrhosis and portal hypertension, and 7% were asymptomatic, presenting with a raised serum alkaline phosphatase. The serum immunoglobulin IgM concentration was raised in 45% of the patients, but no patient had serum mitochondrial antibody present. Seventy-two per cent had ulcerative proctocolitis. There was no relationship between either duration or severity of ulcerative proctocolitis and the development of primary sclerosing cholangitis. Four patients were not benefited by colectomy. None of the patients ahd Crohn's disease. The prognosis was variable. Corticosteriods and azathioprine were ineffective. Eleven patients (38%) had died with a mean survival time of seven years from diagnosis. Three patients with ulcerative proctocolitis developed bile duct carcinoma. The cholangiograms and liver biopsies were reported without reference to clinical information together with 41 patients with other biliary diseases. Cholangiography was diagnostic in 18/22 (82%). Hepatic histology was diagnostic in 8/22 (36%). Ten showed features of large bile duct disease and three were misdiagnosed as primary biliary cirrhosis. Reduced numbers of bile ducts, ductular proliferation, portal inflammation, and substantial copper deposition, in combination with piecemeal necrosis, are commonly seen in primary sclerosing cholangitis and indicate the need for cholangiography.
Adolescent↗
Treatment of alcohol-related liver disease with (+)-cyanidanol-3: a randomised double-blind trial.
A randomised double-blind trial of (+)-cyanidanol-3(Catechin), 2 g/day versus placebo, was carried out in 40 patients with pre-cirrhotic alcohol-related liver disease over a three month period. Twenty received the active drug and 20 placebo; one non-compliant patient in the treatment group was withdrawn. Forty-one per cent (16/39) abstained from alcohol and showed significant improvements (P < 0.005) in mean values for serum aspartate transaminase, serum gamma glutamyl transpeptidase, and mean corpuscular volume. Ten of the 16 showed overall histological improvement on liver biopsy. Fifty-nine pr cent (23/39) continued to drink, though significantly reducing their mean daily alcohol intake (P < 0.001). No significant changes occurred in this group in mean serum enzyme values, though the mean value for mean corpuscular volume improved significantly (P < 0.01) and 16 of the 23 showed overall histological improvement. Changes occurred irrespective of treatment with Catechin which suggests that, over a three month period, this drug did not influence the course of alcohol-related liver disease.
Adult↗
Observations on copper associated protein in childhood liver disease.
Hepatic copper concentrations were compared with staining grades of copper associated protein (CAP) and histochemical copper in liver sections from 44 patients (one fetus, one pre-term infant, four term infants, eight normal children, 16 children with various liver diseases, and 14 patients with intrahepatic cholestasis of childhood (IHCC)). A similar comparative study of hepatic copper concentration with CAP and histochemical copper was performed in 21 patients with Wilson's disease. CAP occurred in the fetus, pre-term infant, and term infants without liver disease. This suggests that CAP is a normal constituent of the hepatocyte and is not a consequence of liver disease or biliary obstruction. CAP was not seen when hepatic copper concentration was normal; it was absent in eight children with no evidence of liver disease, eight children with non-cirrhotic liver disease, and seven of eight children with cirrhosis. When hepatic copper concentration exceeded 4.0 mumol/g dry liver weight grade 2 or grade 3 staining for CAP and histochemical copper was found in the fetus, pre-term infant, infants, and IHCC. CAP was found in IHCC only in the presence of raised hepatic copper levels, supporting evidence of a relationship between copper and CAP. In 17 of 21 patients with Wilson's disease hepatic copper concentrations exceeded 4 mumol/g. Positive staining for CAP was seen in seven of these patients being usually grade 1. CAP is a normal associated protein, present when hepatic copper concentrations are increased in normal liver cells. It is usually absent in hepatocytes from Wilson's disease despite similar hepatic copper levels. CAP may represent material which protects the hepatocyte from the toxic effects of copper.
Adolescent↗
Benign recurrent intrahepatic cholestasis: studies of bilirubin kinetics, bile acids, and cholangiography.
Three patients with benign recurrent intrahepatic cholestasis are described. They had had between five and 16 attacks of cholestasis. Between attacks the liver function tests, including serum bile acids, were normal. Serial serum bilirubin and bile acid estimations during the cholestasis in one patient revealed a consistent discrepancy between the serum bilirubin and bile acid concentrations during three consecutive attacks. In the other two patients the serum concentrations of bile acids and bilirubin varied in parallel. Analysis of the individual serum bile acids did not reveal high concentrations of any 'toxic' bile acid. In one patient, plasma bromsulphthalein (BSP) curves were obtained during both remission and cholestatic periods. The 45 minute retention was slightly increased (10.8%) during remission. During the cholestasis, the 45 minute retention (25%) and the fractional extraction coefficient (Ke=0.069 min-1) were markedly abnormal. The hepatic clearance of unconjugated radiobilirubin was normal at all times in this patient, although during cholestasis, conjugated bilirubin reflexed from the liver to the plasma and was then cleared slowly with a half life of approximately 12 hours. Treatment with corticosteroids, cholestyramine, and phenobarbitone was unsatisfactory.
Adult↗
Pancreatic hypofunction in extrahepatic portal venous obstruction.
Pancreatic function was studied in 29 patients with extrahepatic portal venous obstruction, and 30 age-matched controls. The aetiology of the portal venous obstruction was known in 20 out of 29 patients. No patient had a history of pancreatitis. Serum trypsin, both fasting and for 120 minutes after a Lundh meal, was significantly lower in the patients than in controls (P less than 0.005). 5/28 (18%) patients had reduced fasting serum trypsin levels and in 5/14 trypsin was abnormal after a Lundh meal. Mean fasting serum glucose (3.8 +/- ).49 mmol/l was significantly higher than in control subjects (mean 3.4 +/- 0.42 mmol/l) (P less than 0.05). After 100 g glucose orally, mean serum glucose at 180 minutes (5.26 +/- 1.58 mmol/l) was higher than the control value (3.96 +/- 1.45 mmol/l) (P less than 0.05), and 30 and 60 minutes serum C-peptide values were significantly lower (P less than 0.005). Pancreatic hypofunction found in these patients probably results from an abnormal portal circulation in association with mild pancreatic damage secondary to chronic venous congestion.
Adolescent↗
HLA-B8, immunoglobulins, and antibody responses in alcohol-related liver disease.
Ninety-two British, caucasian, alcoholic patients with liver disese were grouped on the basis of hepatic histology into fatty change, hepatitis with or without cirrhosis, and cirrhosis alone. Men with alcoholic hepatitis with or without cirrhosis showed an increased incidence of the histocompatibility antigen HLA-B8 (P less than 0.02). Increased measles antibody titres were found in patients without cirrhosis with or without hepatitis and were associated with the B8 phenotype in both sexes. Rubella antibody titres and percentage DNA-binding were raised in patients with cirrhosis and showed no association with the B8 phenotype. Concentrations of IgM and IgA were were raised in patients with stetosis and with hepatitis, while in patients with cirrhosis IgG concentrations were also increased. Low titres of autoantibodies were found in all histological groups. It is possible that the development of hepatitis in response to alcohol abuse may be influenced, at least in men, by a gene linked to the B locus. Otherwise, immune processes associated with alcohol-related liver disease are probably secondary phenomena.
Antibodies, Viral↗
Viral antibody titres in HBs antigen-positive and -negative chronic active hepatitis.
A comparative study of antibody titres to nine common viruses has been carried out in hepatitis B surface (HBs) antigen-positive and -negative chronic active liver disease. The results show that increased titres of antibody to morbilli virus by complement fixation and to rubella by haemagglutination inhibition are found in HBs antigen-negative but not in HBs antigen-positive chronic active liver disease. There was a significant association of increased morbilli but not of rubella virus antibody titres with the presence of high-titre nuclear antibodies (ANA) but no association with smooth-muscle antibody or the presence of HLA-B8.
Adolescent↗
Levamisole therapy for HBsAg-positive chronic liver disease.
Eight subjects with HBsAg-positive chronic liver disease received levamisole therapy continuously for 6 to 8 weeks. Depressed T-lymphocyte concentrations, found in all patients before therapy, transiently returned towards normal. All of the three patients who had normal delayed hypersensitivity responses before treatment showed an acute hepatitic reaction manifested by elevation of serum aspartate transaminase (AsT) and HBsAg levels, with transient cell-mediated immunity to HBsAg. This was interpreted as indicating increased lysis of infected hepatocytes. All of the five anergic patients showed no change in AsT or HBsAg levels. The clinical features and hepatic histology were unchanged. In patients with the capacity to mount normal delayed hypersensitivity responses, levamisole enhanced the cell-mediated response to HBsAg, and there was increased lysis of infected cells. Nevertheless, this treatment failed to eradicate the virus, as all patients remained HBsAg-positive.
Adult↗