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At least 19 recordsLinked to original sources
PubMed1988

Further resolution and comparison of the heterogeneity of plasma low-density lipoproteins in human hyperlipoproteinemias: type III hyperlipoproteinemia, hypertriglyceridemia and familial hypercholesterolemia.

The heterogeneity of the plasma low-density lipoproteins (LDL) in subjects with type III hyperlipoproteinemia (3 cases), with hypertriglyceridemia (4 cases) and with the heterozygous form of familial hypercholesterolemia (FH, 4 cases) has been evaluated using a new, high resolution equilibrium density gradient ultracentrifugation procedure. The mass distribution profile, physicochemical properties, particle heterogeneity and apoprotein B content of a series of 13 LDL subfractions was examined in the 3 hyperlipidemic groups and the data were compared with those reported earlier in normolipidemic subjects. In FH, LDL mass was distributed as a narrow peak of d approximately 1.031-1.034 g/ml, whereas the distribution in hypertriglyceridemia was markedly asymmetric with a single peak of elevated density (d approximately 1.037-1.043 g/ml); the distribution in type III subjects was distinguished by its bi- or trimodal nature and broad profile. The chemical composition of LDL gradient subfractions in FH and in hypertriglyceridemia markedly resembled that of the respective parent LDL of d = 1.019-1.063 g/ml, displaying elevated proportions of cholesteryl ester in FH and of protein in hypertriglyceridemia. LDL subfractions in type III disease were enriched in free cholesterol. The Stokes diameters of LDL particles in corresponding subfractions from the 3 hyperlipidemic states were similar; however, whereas a single particle species was characteristic of each LDL subfraction in both FH and in our normolipidemic group, 2 species were frequently present in each subfraction in both type III and type IV diseases; in addition, subfractions from type III subjects occasionally exhibited 3 size species. Apolipoprotein B-100 was the predominant protein component in LDL subfractions from all 3 hyperlipidemic groups. Plasma LDL consist then of multiple particle species which constitute a particularly complex spectrum in type III hyperlipoproteinemia and in hypertriglyceridemia. The origin(s) of such particle subspecies is indeterminate at present; moreover, they may differ in their intravascular metabolism, in their degradation in tissues and in their relative atherogenicities.

Adolescent↗
PubMedDate not supplied

[Anti-kappa elastin antibody titer in the sera of patients with hyperlipoproteinemia type II, III and IV].

Titre of antibodies against elastin degradation product (kappa-elastin) was measured in patients with atherogenic types of hyperlipoproteinemia. The hemagglutination technique was used. A significant decrease in titres of the tested antibodies was found. It was the most prominent in IIa, III and IIb types of hyperlipoproteinemia and rather mild in IV type. The authors attempted to explain causes of antibodies titres decrease in tested patients and relate their results with those of other authors.

Autoantibodies↗
PubMed1993

[Apolipoprotein E phenotyping--useful in the study of hyperlipoproteinemia type III].

Apolipoprotein E (apo E) is a protein constituent of several plasma lipoproteins. Its major physiological role is to mediate the interaction of these lipoproteins and receptors. Three common apo E alleles and six phenotypes are known. The phenotype E2/E2 is associated with type III hyperlipoproteinemia. As a consequence of the increasing clinical interest in apo E phenotypes, especially in relation to the diagnosis of type III hyperlipoproteinemia, we have established a method for apo E phenotyping. Allele frequencies from a population of 211 healthy individuals are presented. The results show that, like as in Finland and Sweden the e4 allele is more frequent than in non-Nordic countries.

Adult↗
PubMed1992

[The relationships between the lipoprotein profile and rheological-coagulation parameters in patients with hyperlipoproteinemia type II].

The aim of the study was to evaluate the direct influence of lipid parameters (total and HDL-cholesterol, triglycerides and total lipids) on the rheologic-coagulative pattern. We studied blood rheological properties--blood (BV), plasmatic (PV), and seric (SV) viscosity, whole blood (WBF) and red cell (RCF) filterability--and some coagulative factors--fibrinogen (Fib), levels of clotting factor VII (fVIIc) and VIII (fVIIIc) activity--in 156 men aged 40-54 years; 87 patients had type II hyperlipoproteinemia (46 type IIa and 41 type IIb) and 69 were normolipemic controls. Smokers, patients with arterial hypertension, diabetes mellitus or cardiovascular clinical manifestations were excluded. Type IIb hyperlipoproteinemic patients had increased blood viscosity (shear rate 225 sec-1, p. less than 0.01), which was positively correlated with triglycerides and fibrinogen concentration. Levels of fibrinogen, fVIIc and fVIIIc activity did not differ significantly in hyperlipemic patients and controls, although fVIIc activity and fibrinogen were both positively related with lipid parameters. These data suggest that, in absence of other major risk factors, the alterations of the rheologic-coagulative pattern are mainly dependent on the severity of the lipid disorder.

Adult↗
PubMed1979

[Rheumatism caused by lipid thesaurismosis. Articular involvement in hyperlipoproteinemia type II : presence of microcrystals in the mitochondria of synoviocytes].

For 13 years polyarthritis with specific synovial involvement was observed in a case of type II hyperlipoproteinemia. Microcrystals similar to those described in Gaucher's and Fabry's disease were seen in synovial cytoplasm and mitochondria. These data suggest a microcrystalline pathogenesis for type II hyperlipoproteinemia arthritis as in gout and chondrocalcinosis.

Adult↗
PubMed1999

Acquired lipoprotein lipase deficiency associated with chronic urticaria. A new etiology for type I hyperlipoproteinemia.

Type I hyperlipoproteinemia (type I HLP) is a rare disorder of lipid metabolism characterized by fasting chylomicronemia and reduced postheparin plasma lipoprotein lipase (LPL) activity. Most cases of type I HLP are due to genetic defects in the LPL gene or in its activator, the apolipoprotein CII gene. Several cases of acquired type I HLP have also been described in the course of autoimmune diseases due to the presence of circulating inhibitors of LPL. Here we report a case of type I HLP due to a transient defect of LPL activity during puberty associated with chronic idiopathic urticaria (CIU). The absence of any circulating LPL inhibitor in plasma during the disease was demonstrated. The LPL genotype showed that the patient was heterozygous for the D9N variant. This mutation, previously described, can explain only minor defects in the LPL activity. The presence of HLP just after the onset of CIU, and the elevation of the LPL activity with remission of the HLP when the patient recovered from CIU, indicate that type I HLP was caused by CIU. In summary, we report a new etiology for type I HLP - a transient decrease in LPL activity associated with CIU and with absence of circulating inhibitors. This is the first description of this association, which suggests a new mechanism for type I HLP.

Adolescent↗
PubMed1981

The effect of weight reduction on biliary lipid metabolism in patients with hyperlipoproteinemia type IV.

Nine slightly obese patients with hyperlipoproteinemia type IV were studied, before and after a mean weight reduction of about 15 kg, with respect to bile acid kinetics, cholesterol balance and biliary lipid composition. The bile acid pool size was not consistently changed. The synthesis of cholic acid and chenodeoxycholic acid was decreased by about 65% and 50%, respectively. The net steroid balance, calculated as bile acid synthesis plus fecal excretion of neutral steroids minus dietary cholesterol intake, was reduced by about 50%. In all but one of the patients bile was supersaturated with cholesterol but weight reduction was not associated with any change in cholesterol saturation. The results indicate that hyperlipoproteinemia type IV may be associated with some metabolic defects which are not corrected for by weight reduction.

Adult↗
PubMed1979

[The range and lipid-lowering effect of etofibrate as a retard-preparation in patients with hyperlipoproteinemia type IIa (author's transl)].

Patients with hyperlipoproteinemia, type IIa, were treated with an adequate diet; additionally they were given 500 mg etofibrate daily. Within 14 days the total cholesterol-level in serum, LDL and LDL-cholesterol were reduced significantly, whereas the level of HDL was not affected. The potency of etofibrate is equal to approximately 50 to 80% of the lipid-lowering effect of clofibrate in an optimal dosis of 2 g/day.

Adult↗
PubMed1976

[Treatment of primary hyperlipoproteinemias type IV with different biguanides (author's transl)].

In 12 patients with primary type IV hyperlipoproteinemia the lipid lowering effect of constant doses of the biguanides metformin, phenformin and buformin was compared. After a 2 months dietary period bodyweight and initial triglyceride and cholesterol plasma concentrations were stabilized. The three biguanides were given for two months each, and this 6 months drug period was followed by another dietary period. While, 0.15 g phenformin and 2.55 g metformin reduced triglycerides by about 50% the reduction by 0.30 g buformin was only half. This was due to 4 nonresponders with initial triglycerides of more than 700 mg/100 ml. In contrast the triglyceride lowering effect of the other two biguanides was positively correlated to the pretreatment triglycerides. The cholesterol lowering effect of biguanide treatment was far less pronounced. There were no significant alterations of body weight and no influence on basal insulin and glucose concentrations. In conclusion, all three biguanides tested in a dosis of 3 tablets each are effective in lowering triglycerides although differing in efficiency and depending from the degree of typ IV hyperlipoproteinemia.

Biguanides↗
PubMed1979

[Study of lipid metabolic coefficient K2 in patients with hyperlipoproteinemia type IV before and after reduction of triglyceride level by adapted diet therapy].

An intravenous fat tolerance test (IV FTT) was performed in a group of patients with type IV hyperlipoproteinemia before and after reduction of triglyceride level, the latter is obtained through a diet adapted to the nutritional sensitivity, body weight remaining constant. Before the diet the increase in cholesterol (CT) and triglycerides (TG) was related in both obese and non-obese patients to an increase in very-low-density lipoproteins (VLDL), while other lipoprotein fractions were not affected. In these patients, K2 is significantly lower than in controls whatever the nutritional sensitivity. However, K2 is lower in obese patients than in patients with normal weight, VLDL-CT and VLDL-TG are tremendously reduced by the relevant diet and K2 is constantly and significantly increased, although it does not reach a normal value in obese subjects. There is a highly significant correlation between VLDL-TG levels and K2 in obese and non-obese subjects. Several physiopathological explanations are discussed to account for the variation of K2 according to TG levels. The results obtained support the view that the K2 defect is secondary to the hyperlipoproteinemia.

Adult↗
PubMed1979

[Comparative analysis of incidence of main risk factors, behavior characteristics and work activity in ischemic heart disease with normolipemia and hyperlipoproteinemia type II and IV].

Examination was conducted of 180 healthy males 35 to 44 years of age, working as leading engineers, and of 174 patients with ischemic heart disease selected according to sex and occupation. A comparative analysis was made of the frequency of the main risk factors and some peculiarities of the labour activity and behaviour of patients with types II and IV hyperlipoproteinemia and those with normal lipid level. It is shown that a combination of known risk factors, behaviour peculiarities and conditions of labour activity is typical of patients with type IV hyperlipoproteinemia, which may promote the development of ischemic heart disease. Patients with normal blood lipid livel merit special attention. Additional factors contributing to the development of ischemic heart disease in these cases should be searched for.

Activities of Daily Living↗
PubMed1979

Polymorphism of apolipoprotein E. II. Genetics of hyperlipoproteinemia type III.

Apolipoprotein E from human serum shows a genetic polymorphism determined by two autosomal codominant alleles, Apo En and Apo Ed. Homozygosity for the gene Apo Ed (phenotype Apo E-D) results in primary dysbetalipoproteinemia, but only some individuals with this phenotype develop gross hyperlipidemia (hyperlipoproteinemia type III). Vertical transmission of dysbetalipoproteinemia represents pseudodominance due to the high frequency of the gene Apo Ed. Dysbetalipoproteinemia is already expressed in childhood. To assess the influence of other genes on the expression of hyperlipidemia in phenotype Apo E-D, comparative studies were carried out in kindreds of hypercholesterolemic (group A) and normo- or hypocholesterolemic probands with dysbetalipoproteinemia (group B). This demonstrated the occurrence of familial (non-type III) forms of hyperlipidemia in group A but not in group B kindreds. Distribution of lipoprotein phenotypes in five of the group A kindreds was consistent with the occurrence of familial combined hyperlipidemia. Apo E phenotypes and hyperlipidemia segregated independently. It is concluded that primary dysbetalipoproteinemia is a frequent monogenic variant of lipoprotein metabolism, but not a disease. Coincidence in one individual of genes for this specific dyslipoproteinemia with any of the genes for monogenic or polygenic forms of familial hyperlipidemia results in hyperlipoproteinemia type III. Hence hyperlipoproteinemia type III is caused by at least two non-allelic genes and is a polygenic disorder.

Adolescent↗
PubMed1977

[Therapy of hyperlipoproteinemia type II with Xantinol-nicotinate (author's transl)].

In 61 out-patients with hyperlipoproteinemia type II the action of 50 mg Xantinol-nicotinate per kg body weight on lipids of plasma and lipoproteins was investigated in a three week trial. The results of six patients had been discharged: two patients failed to attend the follow-up examinations, in four patients a lipid lowering effect could not be demonstrated. 40 Type IIa patients and 15 Type IIb patients were examined. In this two groups cholesterol, phospholipids and triglycerides of plasma were significantly lowered. In Type IIa hyperlipoproteinemia mainly the LDL lipids and in Type IIb additionally the VLDL lipids decreased. In Type IIb patients the decrease of lipids were more rapid than in Type IIa patients. This effect is interpreted by the short half life of VLDL. Withdrawel of the drug resulted in an increase of the lipids to the mean values of the untreated patients within ten days.

Adolescent↗
PubMed1979

Gel isoelectric focusing method for specific diagnosis of familial hyperlipoproteinemia type 3.

We describe a gel isoelectric focusing procedure for resolving into at least five bands the arginine-rich protein of very-low-density lipoproteins, and use the method in diagnosis of hyperlipoproteinemia type 3. We find that deficiency of one band, designated E3, relative to E2, expressed as an E3/E2 ratio less than or equal to 0.3, is specifically associated with hyperlipoproteinemia type 3 as diagnosed by traditional criteria in a large family study. In addition, we used the procedure to test 47 referral samples with demonstrable beta-migrating very-low-density liproprotein grouped by very-low-density lipoprotein cholesterol/total triglyceride ratios of greater than or equal to 0.3, from 0.25 to 0.3, and less than 0.25. All 24 of the first group, three of six in the second, and only one of 17 in the third had E3/E2 ratios of less than or equal to 0.3. Also, two normolipidemic children, without detectable lipoprotein abnormalities but related to subjects with hyperlipoproteinemia type 3, had E3/E2 ratios of less than or equal to 0.3. Use of our procedure improves the specificity of diagnosis and allows sensitive detection of asymptomatic subjects who may be at risk of developing the disorder.

Apolipoproteins↗