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Results for “Hyperlipoproteinemia Type II”

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At least 19 recordsLinked to original sources
PubMed1976

Probucol: a new cholesterol-lowering drug effective in patients with type II hyperlipoproteinemia.

Type II hyperlipoproteinemia or hyperbetalipoproteinemia (B-HLP), a condition with considerable atherogenic potential, is one of the most difficult lipid disorders requiring treatment. Since this abnormality responds minimally to dietary therapy alone, supplemental drug therapy is usually essential. Although the available bile-sequestering resins are effective in B-HLP, these substances are unpalatable and constipating. Since lifelong drug therapy is necessary as an adjunct to diet in the treatment of B-HLP, the ideal drug should be both effective and well tolerated. Probucol, a new cholesterol-lowering drug in tablet form without serious adverse effects, was evaluated in a 12-wk double-blind crossover trial in 11 patients with B-HLP whose serum cholesterol levels were in excess of 275 mg/dl. Probucol, in a dosage of 500 mg twice daily, produced a 10% or greater reduction in serum cholesterol levels in all 11 patients. Serum cholesterol was lowered (p less than 0.01) from 353 to 291 mg/dl in the entire group receiving probucol. There was no significant change (p greater than 0.1) in serum cholesterol (352 mg/dl) during placebo administration. These were no untoward drug effects during the study, and all patients maintained excellent complicance to the schedule of medication. These results indicate that probucol possesses considerable cholesterol-lowering activity and may be a promising new nontoxic therapeutic agent in type II hyperlipoproteinemia.

Cholesterol↗
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↗
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

Treatment of hyperlipoproteinemia type II with etofibrate.

Seven patients with hyperlipoproteinemia (HLP) type II (four patients with type II A and three patients with type II B), who were experienced to be resistant to hypolipidemic drugs, were treated for 6 months with etofibrate, a double-ester of nicotinic acid and clofibrinic acid, at a dose of 0.3 g t.i.d. Mean serum cholesterol level decreased by up to 18% from a pre-treatment value of 7.7 +/- 1.4 mmol/l. The reduction of serum cholesterol was due both to a decrease in very low density (VLDL) and low density (LDL) lipoprotein cholesteral by 61 and 25%, respectively (after 6 months). Furthermore alpha-LP (HDL) cholesterol increased by 8%, (after 6 months). All seven patients had previously received clofibrate and had obtained a mean decrease in plasma cholesterol by 6%. There was a slight transient increase in S-ASAT and S-ALAT simultaneous with in increase in serum urate. However, these values returned after 3 months to pre-treatment level. No influence on glucose tolerance was recorded. There were no bothersome side effects except a transient discomfort in the form of flushing or acid indigestion which occurred after 1--2 months of treatment with etofibrate.

Body Weight↗
PubMed1976

Therapeutic failure in familial type II hyperlipoproteinemia.

The extended use of diet and cholestyramine therapy in familial type II hyperlipoproteinemia was examined in patients who previously participated in a short-term, double-blind trial. A striking secondary failure in therapeutic response during 4 yr of use of this therapy was noted with plasma cholesterol rising an average of 15%. A 3 mo, out-patient, follow-up study designed to reinforce patient motivation and dietary and drug adherence resulted in a prompt but partial reversal of this therapeutic deterioration in 16 patients. Additional inpatient studies confirmed that patient noncompliance with the dietary regimen was the major factor responsible for the secondary failure. Cholestyramine together with a low cholesterol diet can be an effective agent in familial type II hyperlipoproteinemia, given a comprehensive program of out-patient follow-up with continued emphasis on dietary principles and drug adherence.

Adult↗
PubMedDate not supplied

Results of colestipol therapy in Type II hyperlipoproteinemia.

Twenty-five patinets with well defined Type ii hyperlipoproteinemia were treated with a divided 15 g daily dose of colestipol, a bile acid sequestrant, for periods of up to 20 months. The patients were divided into 3 groups: Those with no obvious sequelae, those with arcus corneae, xanthomas, and/or xanthelasmas only, and those with atherosclerotic complications. Colestipol lowered plasma cholesterol in all 3 groups, but reduced it to normal or near-normal levels in only 9 of the 25 patients (36%). The response of plasma triglycerides was highly varible; the mean for each group was elevated by the drug. Colestipol was well-tolerated and its effect did not diminish with time. It is a useful drug in the treatment of hypercholesterolemia.

Adolescent↗
PubMed1976

Pseudohomozygous and pseudoheterozygous type II hyperlipoproteinemia.

A 4-year-old boy had typical features of homozygous type II hyperlipoproteinemia with planar xanthomas, a plasma cholesterol level greater than 600 mg/dl, and an estimated beta-lipoprotein cholesterol concentration greater than 500 mg/dl. Both he and his sister, who had the biochemical features of the heterozygous state, responded unusually well to a diet low in saturated fat and cholesterol content. These features, combined with the finding of normocholesterolemic parents, are highly suggestive of a newly described syndrome. pseudohomozygous type II hyperlipoproteinemia.

Adult↗
PubMed1978

Evaluation of an automated immunoassay procedure for apo-LDL for phenotyping type II hyperlipoproteinemia.

Apo-low density lipoproteins were determined by an automated immunoassay procedure on serum samples from 88 normolipidemic individuals and 84 hyperlipoproteinemic subjects, to establish whether this method was useful in the routine detection of type II hyperlipoproteinemia. The results obtained were compared with the cholesterol levels of the same specimens. In subjects with type II hyperlipoproteinemia, the apo low density lipoprotein levels, as well as the ratio of low density lipoprotein cholesterol/apo-low density lipoprotein were higher, as expected, than in normals or in subjects with other types of hyperlipoproteinemia. However, there was considerable overlap in individual values of both these parameters, between patients with type II hyperlipoproteinemia and normals or subjects with other types of hyperlipoproteinemia, suggesting that apo low density lipoprotein levels alone were not sufficiently discriminatory for the laboratory determination of type II hyperlipoproteinemia.

Adult↗
PubMed1975

Tuberous xanthoma in homozygous type II hyperlipoproteinemia. A histologic, histochemical, and electron microscopical study.

Histologic, histochemical, and ultrastructural studies of a tuberous xanthoma from a patient with homozygous type II hyperlipoproteinemia showed that all of the lipid was within histiocytic foam cells; no lipid was identified in interstitial regions or in blood vessels. Primitive mesenchymal cells, elongated perivascular and fibroblast-like cells, and lysosome-filled macrophages also were present within the xanthoma, indicating possible stages in the evolution of dermal mesenchymal cells into mature, cholesterol-rich foam cells. Morphologically, the lipid was in four different forms: large droplets, which were the dominant form, and membrane-bound crystals, concentric lamellar bodies, and ceroid. The paucity of membrane-bound lipid forms, relative to the abundant free lipid droplets, indicated that lysosomal digestion was a minor metabolic pathway for the intracellular metabolism of lipid in the xanthoma. Thus, nonlysosomal lipid storage in foam cells is a characteristic tissue response to the underlying metabolic defect in type II hyperlipoproteinemia.

Adolescent↗
PubMed1975

Control of sterol synthesis and of hydroxymethylglutaryl CoA reductase in skin fibroblasts grown from patients with homozygous type II hyperlipoproteinemia.

In skin fibroblasts grown from four children with a homozygous form of type II hyperlipoproteinemia, the feedback control of sterol synthesis and the inhibitory effect on hydroxymethylglutaryl (HMG) CoA reductase activity by serum or low density lipoprotein were present, though diminished compared with the effects in normal fibroblasts. Stimulation of HMG CoA reductase by insulin and inhibition of acetyl CoA carboxylase by serum lipids were not impaired in these type II cells, indicating a degree of specificity in the abnormal response of the reductase. A rapid and convenient method for isolation of mevalonolactone in the course of the assay of HMG CoA reductase is described.

Acetyl-CoA Carboxylase↗
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↗
PubMed1976

Colestipol in familial type II hyperlipoproteinemia: a three-year trial.

Colestipol, a new bile acid sequestrant polymer, has been shown to lower the serum cholesterol level more than 30% in 13 patients with familial type II hyperlipoproteinemia. Placebo for 6 wk was followed by colestipol for periods up to 36 mo. A slight but not significant increase of serum triglyceride concentrations was observed during the first 18 no, but they returned to values under the baseline level thereafter. No signs of impaired intestinal fat resorption were noted. Side effects were primarily gastrointestinal (mild and transient constipation). Colestipol seems to be an effective and safe drug in the treatment of the famiial type II hyperlipoproteinemia, without escape phenonmenon.

Adolescent↗
PubMed1975

Lipids and lipoproteins of serum and organ tissues in a case of familial hyperlipoproteinemia type II.

Lipids and lipoproteins of serum and various organ tissues of a patient with familial hyperlipoproteinemia homozygous Type II were studied. It was found that low density lipoprotein between densities of 1.006 and 1.063 was a major component of serum of the patient. The isolated low density lipoprotein appeared to be rather similar to that of normal human serum from the viewpoints of chemical composition and physico-chemical properties. Antibody against the low density lipoprotein of the patient reacted not only with the same lipoprotein, but also with normal human low density lipoproteins. Immunoelectrophoresis of the antibody gave a single precipitin line against only the low density lipoprotein. Immunofluorescent techniques using the fluorescein labeled antibody showed localization of low density lipoprotein in cytoplasm of hepatocytes of both the patient and other human. No fluorescences were found in other organ cells except in their capillary tubes and renal glomerulus in the hyperlipoproteinemia Type II. It was noted that tuberous xanthomata contained fairly large amounts of cholesterol and cholesterol ester. Fatty acid composition of neutral lipids and phospholipids from the serum low density lipoprotein, xanthomata, liver and other organ tissues were compared with each other. Chomesterol ester of the xanthomata contained much smaller amounts of polyunsaturated fatty acids as compared with those of the low density lipoprotein and other tissue. Also, composition of free fatty acids in liver differed from those of other tissues.

Amino Acids↗
PubMed1976

Neonatal diagnosis of familial type II hyperlipoproteinemia.

Cord serum prebeta-beta-lipoprotein concentration was measured in a radial immunodiffusion assay in 303 randomly selected, full-term infants whose parental phenotypes were unknown. Six infants had elevated concentrations, that is above a cut-off limit of 197 mg/100 ml (97.5th percentile). Three of these infants suffered from classic type II hyperlipoproteinemia, and this diagnosis was confirmed by family studies at follow-up. The three other infants and their parents were shown to be normolipemic at follow-up. The elevated cord serum prebeta-beta-lipoprotein in these three infants could be explained by highly elevated cord serum triglyceride and/or cholesterol. The 297 infants with normal cord serum prebeta-beta-lipoprotein and their parents were all shown to be normalipemic at follow-up. It is concluded that the measurement of prebeta-beta-lipoportein in cord serum allows the identification of children with familial type II hyperlipoproteinemia, if infants with transient neonatal hypertriglyceridemia and hypercholesterolemia are excluded.

Adult↗
PubMed1979

Use of combined diet and colestipol in long-term (7--7 1/2 years) treatment of patients with type II hyperlipoproteinemia.

Long-term effects of diet and colestipol (a bile acid sequestrant) were studied in 25 patients with familial type II hyperlipoproteinemia. Serum lipids and body weights of an initial group of 30 patients were stabilized by low cholesterol-saturated fat-refined carbohydrate diet and the patients were then randomized into placebo and drug-treatment groups. After explaining that the drug is nontoxic and effective in lowering serum lipids, total cholesterol (C) and low-density lipoprotein cholesterol (LDL-C), colestipol (30 g/day) and diet were given to the 25 patients who remained in the long-term follow-up program. The treatment resulted in highly significant lowering of serum lipids (mg/dl, mean +/- SEM): C and LDL-C from 412.7 +/- 24.4 and 331.1 +/- 22.8 to 270 +/- 11.0 and 188.1 +/- 13.8, respectively (p less than 0.001 in each instance) over 7--7 1/2 years. Although we observed no absolute increase in high density lipoprotein (HDL), the HDL/LDL ratio was elevated. Long-term colestipol and diet treatment reduced the xanthoma size and stabilized serially angiographically visualized atherosclerotic lesions in 21 of the 25 patients who showed a satisfactory hypolipemic response. It did not cause nutritional or metabolic disturbances.

Adult↗
PubMed1976

Clofibrate in type II hyperlipoproteinemia.

As a part of a double-blind randomized study, the safety and the lipid- and uric acid-lowering effect of clofibrate have been evaluated in 28 patients with type II hyperlipoproteinemia (HLP). A highly significant reduction of serum cholesterol occurred in type IIa and of serum triglyceride and choelsterol in type IIb HLP throughout the 60-week observation period (p less than 0.01). Of the patients with types IIa and IIb HLP, 65% had at least a 25% reduction of serum cholesterol. Uric acid was significantly reduced only during the first period of treatment (p less than 0.05). In the laboratory measurements concerning safety, a persistent, slight reduction was observed in Hb, hematocrit and alkaline phosphatase. No significant clinical side-effects were noted. Clofibrate is considered effective as a lipid-lowering agent in many cases of type II HLP.

Adult↗
PubMed1975

[Lipid constellation in low density lipoproteins of primary type II hyperlipoproteinemia (author's transl)].

The lipid composition of purified low density lipoproteins (LDL) was investigated in normal subjects and in type II a and b hyperlipoproteinemia. A significant increase of ester cholesterol in LDL of type II a patients and a decrease of phospholipids in LDL of type II b patients as compared with normal controls, were encountered. The results are compared with other reports from the literature and discussed with regard to the disturbance of LDL metabolism in type II hyperlipoproteinemia.

Adolescent↗