[Hepatic vein thrombosis. Diagnostic and therapeutic difficulties].

Budd Chiari syndrome (liver vein thrombosis) may be a diagnostic and therapeutic problem. On the basis of four different cases we review the major diagnostic and therapeutic principles involved. Imaging techniques are necessary in order to establish the diagnosis.

[Allogeneic bone marrow transplantation in adult patients with serious hematological diseases 1985-1992].

We present updated results from allogeneic bone marrow transplantation in adult patients who received transplants between 1985 and 1992. Of 47 patients, 36 where disease-free survivors 8-93, mean 32 months after transplantation. Of these, seven had received marrow from unrelated donors.

The genetic defect of the original Norwegian lecithin:cholesterol acyltransferase deficiency families.

Three of the original Norwegian lecithin:cholesterol acyltransferase (LCAT) deficiency families have been investigated for mutations in the gene for lecithin:cholesterol acyltransferase by DNA sequencing of the exons amplified by the polymerase chain reaction. A single T----A transversion in codon 252 in exon 6 converting Met(ATG) to Lys(AAG) was observed in all homozygotes. In spite of the identical mutation, the disease phenotypes differed in severity.

Lipoproteins, lipases, and the metabolic cardiovascular syndrome.

High levels of plasma triglycerides and very-low-density lipoproteins and low levels of high-density lipoproteins are consistently found in the metabolic cardiovascular syndrome. These changes are exaggerated postprandially. In the liver, synthesis and secretion of triglyceride-rich particles are increased.

Liver steatosis in hypobetalipoproteinemia. A case report.

A case of hypobetalipoproteinemia is described; a 16-year-old girl had been suffering for nearly 2 years from diffuse abdominal pain. The only clinical features were liver steatosis, slightly increased amino transferases and an incipient polyneuropathy. No sign of malabsorption or gastrointestinal disease was found.

Intracellular regulation of lipoprotein lipase in human monocyte-derived macrophages.

The intracellular pathway of lipoprotein lipase (LPL) has been examined in human monocyte-derived macrophages in culture. These cells were previously shown to synthesize and constitutively secrete LPL. The secretion is dependent on new enzyme synthesis.

[Drug therapy of hypercholesterolemia. Treatment of hypercholesterolemia in adults--a Norwegian therapeutic program 1988].

There are indications that treatment of hypercholesterolemia by means of drugs reduce risk of atherosclerosis in patients with increased concentrations of atherogenic lipoproteins. Such therapy should be initiated only after satisfactory exclusion of secondary causes of hyperlipoproteinemia, and should be regarded as an adjunct to appropriate dietary therapy. Drug therapy should be strongly considered in patients with total cholesterol above 8-9 mmol/l on diet therapy only.

Identification of homozygosity for a human apolipoprotein A-I variant.

An apolipoprotein (apo) A-I variant, previously described in two Norwegian families (Schamaun et al. 1983. Hum.

Release of hepatic lipase and very low density lipoprotein by cultured rat hepatocytes.

Primary cultures of rat hepatocytes were used to study the release of hepatic lipase and very low density lipoprotein (VLDL). The presence of hepatic lipase activity was proved by salt-resistance, affinity chromatography and inactivation by a hepatic lipase antibody. Cellular rate of hepatic lipase release increased by prolonged time in culture, whereas VLDL secretion decreased.

Synthesis and secretion of lipoprotein lipase by human monocyte-derived macrophages.

Human monocytes isolated from either defibrinated blood or buffy coat were shown to produce and secrete lipoprotein lipase during culture. The secretion occurred constitutively. Low levels of enzyme activity in the medium from freshly isolated cells increased with time of incubation, and maximal activity was attained after 9 days.

Effect of high-density lipoproteins on cholesterol efflux and esterification in lipid-enriched human skin fibroblasts.

The ability of high-density lipoprotein (HDL) to reduce the cholesterol content was studied in cultured fibroblasts enriched with cholesterol esters. Incubation of cholesterol-enriched cells with HDL in a final concentration of 1 g protein/l for 24 h reduced the total and esterified cholesterol content by 23% as compared with control fibroblasts incubated with albumin. Similar cholesterol efflux was obtained with HDL isolated from lecithin:cholesterol acyltransferase (LCAT)-deficient plasma.

Follow-up and prospective studies of the classification of liver disease.

Two hundred patients with different liver diseases were observed during a period of 6-8 years. The diagnosis at the first hospitalization was based on morphological criteria (and, in some cases, additional clinical information). In 162 of the cases an initial 'specific' diagnosis could be made.

The effect of systemic heparinization on plasma lipoproteins and toxicity in patients on hemodialysis and continuous ambulatory peritoneal dialysis.

The lipid patterns of plasma from 6 patients on hemodialysis (HD) and 6 patients on continuous ambulatory peritoneal dialysis (CAPD) were compared and correlated to plasma toxicity as measured by the survival of human macrophages cultured in vitro. The median values for plasma triglycerides (TG), cholesterol, low density lipoprotein (LDL) cholesterol, apolipoprotein B and lipolytic activities (lipoprotein lipase and hepatic lipase) were insignificantly higher in CAPD plasma than in HD plasma. The median high density lipoprotein (HDL) cholesterol/LDL cholesterol ratio was significantly higher in HD plasma than in CAPD plasma.

Hemodialysis and cell toxicity in vitro related to plasma triglycerides, post-heparin lipolytic activity and free fatty acids.

Plasma triglyceride (TG) concentrations, post-heparin lipolytic activities and the free fatty acid (FFA) pattern of uremic and normal plasma were compared and correlated to plasma toxicity as measured by the effect on human mononuclear phagocytes cultured in vitro. Plasma TG concentration and FFA: albumin molar ratios were significantly higher in uremic plasma, and a correlation was found between TG concentrations prior to heparinization and post-heparin FFA concentrations. Uremic plasma toxicity was significantly correlated to increased post-heparin FFA: albumin molar ratio.

Pulmonary hydrogen and methane and plasma ammonia after the administration of lactulose or sorbitol.

The gut microfloral metabolism of sorbitol and lactulose was investigated in 10 healthy control subjects and 10 patients with cirrhosis of the liver, by measuring the pulmonary excretion of the fermentational gases hydrogen and methane during 3 h. A possible acute effect on venous plasma ammonia after a single dose of sorbitol or lactulose was also investigated. No significant difference in fermentation gas excretion was found after ingestion of sorbitol compared with lactulose in healthy controls or cirrhotic patients (p greater than 0.

Serum selenium levels in liver diseases.

A possible pathogenetic role of selenium deficiency in alcoholic cirrhosis of the liver has previously been discussed. In the present study serum selenium was analyzed in 5 groups of liver diseases. The method used for selenium determination was electrothermal atomic absorption, after thermal stabilization of selenium compounds by addition of nickel nitrate.

Immunohistochemical characterization of hepatic tissue lymphocyte subpopulations in liver disease.

Liver biopsies from 27 patients were studied by the indirect immunofluorescence and immunoperoxidase technique for relative distribution of B cells and T-cell subpopulations. T-cell subsets were defined for OKT4(+) (helper/inducer) and OKT8(+) (suppressor/cytotoxic) cells by using mouse hybridoma monoclonal antibodies. Patients with chronic active hepatitis and those with primary biliary cirrhosis showed marked relative increments in suppressor/cytotoxic OKT8(+) lymphocytes within hepatic lymphocytic infiltrates.

Diagnostic and prognostic value of laboratory tests assessed in a follow-up study of 200 patients with liver disease.

Two hundred patients with liver disease were re-studied after six to eight years. The initial routine diagnosis (mainly based on morphological criteria) was confirmed in almost 80% of cases of chronic liver disease. In chronic active hepatitis, agreement was found in 86%.