No abstract available.
Humans ; Hyperlipoproteinemia Type III ; Male

Although elevated serum low-density lipoprotein-cholesterol (LDL-C) is without any doubts accepted as an important risk factor for cardiovascular disease (CVD), the role of elevated triglycerides (TGs)-rich lipoproteins as an independent risk factor has until recently been quite controversial. Recent data strongly suggest that elevated TG-rich lipoproteins are an independent risk factor for CVD and that therapeutic targeting of them could possibly provide further benefit in reducing CVD morbidity, events and mortality, apart from LDL-C lowering. Today elevated TGs are treated with lifestyle interventions, and with fibrates which could be combined with omega-3 fatty acids. There are also some new drugs. Volanesorsen, is an antisense oligonucleotid that inhibits the production of the Apo C-III which is crucial in regulating TGs metabolism because it inhibits lipoprotein lipase (LPL) and hepatic lipase activity but also hepatic uptake of TGs-rich particles. Evinacumab is a monoclonal antibody against angiopoietin-like protein 3 (ANGPTL3) and it seems that it can substantially lower elevated TGs levels because ANGPTL3 also regulates TGs metabolism. Pemafibrate is a selective peroxisome proliferator-activated receptor alpha modulator which also decreases TGs, and improves other lipid parameters. It seems that it also has some other possible antiatherogenic effects. Alipogene tiparvovec is a nonreplicating adeno-associated viral vector that delivers copies of the LPL gene to muscle tissue which accelerates the clearance of TG-rich lipoproteins thus decreasing extremely high TGs levels. Pradigastat is a novel diacylglycerol acyltransferase 1 inhibitor which substantially reduces extremely high TGs levels and appears to be promising in treatment of the rare familial chylomicronemia syndrome.
Apolipoprotein C-III ; Cardiovascular Diseases ; Diacylglycerol O-Acyltransferase ; Fatty Acids, Omega-3 ; Fibric Acids ; Hyperlipoproteinemia Type I ; Life Style ; Lipase ; Lipoprotein Lipase ; Lipoproteins ; Metabolism ; Mortality ; PPAR alpha ; Risk Factors ; Triglycerides

Xanthomas are localized lipid deposits within organs that may manifest as papules, plaques, or nodules in skin. They are commonly associated with all types of hyperlipidemia. Xanthoma striatum palmare characterized by xanthomas of the palmar creases is a rare but important diagnostic physical sign of dysbetalipoproteinemia, also known as type III hyperlipoproteinemia. Type III hyperlipoproteinemia is characterized by the early onset of cardiovascular disease and peripheral vascular disease. We describe herein the case of a 51-year-old female patient affected by xanthoma striatum palmare associated with elevated plasma levels of triglycerides and cholesterol and a lipoprotein electrophoresis pattern consistent with type III hyperlipoproteinemia.
Cardiovascular Diseases ; Cholesterol ; Electrophoresis ; Female ; Humans ; Hyperlipidemias ; Hyperlipoproteinemia Type III* ; Lipoproteins ; Middle Aged ; Peripheral Vascular Diseases ; Plasma ; Skin ; Triglycerides ; Xanthomatosis*

A 55-year-old female presented with extensive yellowish eruptive plaques over both elbows and the buttocks that she had first noticed 2 years earlier. Yellowish orange discoloration of her palmar creases was noted. Her serum cholesterol and triglyceride were markedly elevated. Lipoprotein electrophoresis showed a broad beta band. On apolipoprotein E genotyping, the arginine at position 158 had been replaced by cysteine in both alleles (E2/E2). Under a diagnosis of type III hyperlipoproteinemia, combined atorvastatin and fenofibrate therapy for 2 months normalized the serum cholesterol and triglyceride levels.
Alleles ; Apolipoproteins ; Arginine ; Buttocks ; Cholesterol ; Citrus sinensis ; Cysteine ; Elbow ; Electrophoresis ; Female ; Fenofibrate ; Heptanoic Acids ; Humans ; Hyperlipoproteinemia Type III ; Hyperlipoproteinemias ; Lipoproteins ; Middle Aged ; Pyrroles ; Xanthomatosis ; Atorvastatin Calcium

Animals ; Apolipoproteins E ; deficiency ; Female ; Hyperlipoproteinemia Type III ; blood ; metabolism ; Lipid Metabolism, Inborn Errors ; blood ; metabolism ; Male ; Mice ; Mice, Inbred C57BL ; Receptors, Calcitriol ; metabolism ; Vitamin D ; blood

Adult ; Female ; Humans ; Hyperlipoproteinemia Type III ; pathology ; Male ; Middle Aged ; Nephrosis, Lipoid ; pathology

OBJECTIVEThe aim of the study was to investigate apolipoprotein(apo) E polymorphism and its relationship with serum lipids and apolipoprotein, serum high density lipoprotein(HDL) subclasses in patients with type IV hyperlipidemia.

METHODSapoE genotype was assayed by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). The subclasses of serum HDL in 103 patients with type IV hyperlipidemia and 146 normolipidemic subjects were determined by two-dimensional gel electrophoresis in conjunction with immunodetection method.

RESULTSThe apoE3/3 genotype frequency and allele epsilon 3 frequency were both the highest in the frequency distribution profiles of the type IV hyperlipidemia group and the control group. In type IV hyperlipidemia group, the genotype of apoE2 had higher serum HDL-C,apoE, HDL(2a) apoE/apoCIII ratio but lower TG/HDL-C,apoCIII, HDL(3c) levels when compared with the genotype of apoE(3) (P<0.05). In control group, the genotype of apoE(2) had higher serum TG, apoE levels and apoE/aopCIII ratio but lower HDL (3a) level when compared with the genotype of apoE(3) (P<0.05).

CONCLUSIONAn association of allele epsilon 2 of apoE gene with the maturation of HDL in type IV hyperlipidemia was noted in the study.

Adult ; Aged ; Apolipoprotein C-III ; blood ; Apolipoprotein E2 ; blood ; genetics ; Apolipoprotein E3 ; blood ; genetics ; Apolipoproteins E ; blood ; genetics ; Cholesterol, HDL ; blood ; Female ; Humans ; Hyperlipoproteinemia Type IV ; blood ; genetics ; Lipoproteins, HDL ; blood ; Male ; Middle Aged ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Polymorphism, Restriction Fragment Length ; Triglycerides ; blood

Generalized plane xanthoma is a group of plane xanthomas that appear as yellow to yellow-brown flat patches or slightly elevated plaques with a wide-spread distribution. The disease is frequently associated with multiple myeloma or other reticulo-endothelial malignancies and monoclonal gammopathy with unknown significance, and when accompanied by these hematologic malignancies, normolipoproteinemia is invariably associated. We present a case of generalized plane xanthoma associated with multiple myeloma and type III hyperlipoproteinemia in a 48 year-old woman, and this is the first report in Korean literature.
Female ; Hematologic Neoplasms ; Humans ; Hyperlipoproteinemia Type III ; Hyperlipoproteinemias* ; Middle Aged ; Multiple Myeloma* ; Paraproteinemias ; Xanthomatosis*

A 61-year-old female patient presented with the type III hyperlipoproteinemia (HLP) in association with generalized eruptive and tuberous xanthomas. She had hypercholesterolemia and hypertriglyceridemia, and extensive coronary atherosclerosis. Further studies revealed a positive standing plasma test, abnormal beta-very low density lipoprotein (VLDL) on lipoprotein electrophoresis, markedly elevated very low density lipoprotein-cholesterol (VLDL-C) to plasma triglycerides (TG) ratio (0.86) and homozygosity for apolipoprotein E2. After about one year of therapy with lipid-lowering agents and diet restriction, a significant reduction of serum cholesterol and TG was observed and the yellowish orange discolorations of palmar creases disappeared from her palms.
Apolipoprotein E2 ; Cholesterol ; Citrus sinensis ; Coronary Artery Disease ; Diet ; Electrophoresis ; Female ; Humans ; Hypercholesterolemia ; Hyperlipoproteinemia Type III* ; Hypertriglyceridemia ; Lipoproteins ; Middle Aged ; Plasma ; Triglycerides ; Xanthomatosis*

We report a case of type III hyperlipoproteinemia associated with xanthoma eruptivum. Xanthoma tuberosum, and xanthoma striatum palmare whose clinical symptoms have been improved by the treatment of gemfibrozil. A 31-year-old male patient visited our clinic for evaluation of multiple yellowish nodules on the elbows, multiple yellowish plaques on the buttocks and linear yellowish plaques along with the palmar creases on both palms. The blood chemistry showed an elevation of cholesterol and triglyceride. Lipoprotein electrophoresis showed broad betazone and plasma standing test showed turbid plasma. Lipoprotein ultracentrifugation showed that the ratio of VLDL cholesterol and plasma triglyceride was 0.38. According to the above findings, we diagnosed this patient as type III hyperlipoproteinemia. After 4 months of treatment with diet and gemfibrozil, the serum level of cholesterol and triglyceride were reduced to normal range. The skin lesions of both palms showed marked improvement but the skin lesions on elbows and buttocks were not much improved.
Adult ; Buttocks ; Chemistry ; Cholesterol ; Cholesterol, VLDL ; Diet ; Elbow ; Electrophoresis ; Gemfibrozil ; Humans ; Hyperlipoproteinemia Type III* ; Lipoproteins ; Male ; Plasma ; Reference Values ; Skin ; Triglycerides ; Ultracentrifugation ; Xanthomatosis*