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Query: UMLS:C0004153 (atherosclerosis)
 77,401 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although the usual diet may contain 150-250 mg of plant sterols, chiefly beta-sitosterol, only trace amounts of these sterols have heretofore been found in human or animal blood and tissues. We now report elevated plant sterol levels in the blood and tissues of two sisters with extensive tendon xanthomas but normal plasma cholesterol levels. Besides beta-sitosterolemia and xanthomatosis, no other physical, mental, or biochemical abnormalities were detected.Repeatedly, the plasmas of the two sisters have contained 27.1 and 17.7 mg/100 ml of beta-sitosterol, 9.7 and 8.2 mg/100 ml of campesterol, and 0.5 and 0.5 mg/100 ml of stigmasterol, respectively. These plant sterols constituted 15.6 and 11.3% of the total plasma sterols. Some 60% of the plasma beta-sitosterol and campesterol was esterified; the measurable stigmasterol was entirely unesterified. The transport of the plasma beta-sitosterol and campesterol was largely in low density lipoproteins (76 and 83%, respectively). High density lipoproteins carried the remainder. Plant sterols were barely detectable in the very low density lipoprotein fraction. Only trace amounts of stigmasterol could be detected in the low density and high density lipoprotein fractions. The plant sterol content of the red blood cells averaged 12-13 mg/100 ml packed cells or about 13% of the total sterols. Two tendon xanthoma biopsies with the usual high concentration of cholesterol had 36.7 and 4.0 mg of plant sterols/g dry wt, of which 25.7 and 2.9 mg were beta-sitosterol, entirely in the free form. Plant sterols were also found in adipose tissue (0.2 mg/g wet wt) and in skin surface lipids (3.2 mg/g of lipid). The intestinal absorption of beta-sitosterol in both the patients, measured by two techniques, indicated greatly increased absorption of this sterol (about 24 and 28% in the patients L. H. and R. H., respectively, normal absorption being <5%). We suggest that increased absorption of beta-sitosterol must be considered as one cause of this disease. The reason for the extensive xanthomatosis in these two patients remains unknown. Perhaps in some way plant sterols initiated the development of xanthomas with otherwise normal plasma cholesterol levels. Clinical atherosclerosis has not yet occurred. The occurrence of beta-sitosterolemia in these two sisters with un-affected parents suggests an inherited recessive trait.
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PMID:Beta-sitosterolemia and xanthomatosis. A newly described lipid storage disease in two sisters. 436 Aug 55

The water-insoluble cholesterol-cholestanol-water adduct C-C-2W, chemical and physical cause of atherosclerosis and gallstones, has now been found in tendinous xanthoma as well; C-C-2W, and not cholestanol, is the initial compound deposited in hereditary CTX (cerebrotendinous xanthomatosis). From these and other findings it is theorized that what have been termed cholesterol or cholestanol lipidoses should instead be characterized as C-C-2W lipidoses. More than 1 mg of cholestanol . H2O present in the body causes crystallizaion of C-C-2W . This happens when the steroid meets cholesterol . H2O in sufficient concentration to reach a solubility product of 10(-7) mg/ml. In this light the literature can be interpreted to indicate that C-C-2W exerts negative effects on liver, intima tissue, eyes, lungs, and other parts of the body. Those effects include inflammation, cell necrosis, destruction of cell membranes, abnormal growth and, perhaps, neoplastic activity. Up to 200 g of C-C-2W in the body may be tolerated if evenly spread but not if localized in one or two areas only; e.g., the brain or the cardiovascular system. It is estimated that about 1000 g of C-C-2W, even if spread, are beyond the limit of human tolerance.
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PMID:A new unified theory: C-C-2W is the chemical cause of all so-called cholesterol and cholestanol lipidoses. 677 47

Type III hyperlipoproteinemia is characterized by increased plasma levels of triglycerides and cholesterol, palmar-tuberoeruptive xanthoma, and premature cardiovascular disease. Three major classes of molecular defects will predispose patients to develop type III hyperlipoproteinemia: a deficiency in apolipoprotein E, a structural defect in the E apolipoprotein, and a functional defect in the liver receptor system. Most patients with type III hyperlipoproteinemia have a structural defect in apolipoprotein E associated with increased synthesis and decreased catabolism of apolipoprotein E, delayed catabolism of chylomicron remnants, and development of plasma lipoprotein abnormalities characteristic of type III hyperlipoproteinemia. Analysis of cardiovascular disease in patients with type III hyperlipoproteinemia showed extensive coronary and peripheral vascular atherosclerosis indistinguishable from the atherosclerosis of non-hyperlipidemic and other dyslipoproteinemic patients. The xanthoma and elevated plasma cholesterol and triglyceride levels in patients with type III hyperlipoproteinemia respond to dietary and drug therapy.
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PMID:NIH conference. Type III hyperlipoproteinemia: diagnosis, molecular defects, pathology, and treatment. 684 77

Colestipol is a safe, effective, cholesterol-lowering, bile-acid sequestrant that lowers low-density-lipoprotein (LDL) and total plasma cholesterol levels without consistently affecting high-density-lipoprotein (HDL) cholesterol levels. Long-term colestipol therapy in conjunction with diet may reduce xanthoma size, arrest progression of coronary artery atherosclerosis, and may reduce mortality from coronary heart disease. Probucol, a bisphenol cholesterol-lowering drug, is an effective cholesterol-lowering agent that reduces levels of HDL cholesterol, HDL cholesterol, and apoprotein A-1, the major apolipoprotein of HDL. Because HDL cholesterol is independently and inversely associated with development of coronary heart disease, the ramifications of simultaneous lowering of LDL and HDL cholesterol levels by probucol treatment need further study. Long-term, placebo-controlled studies of repetitive coronary arteriography, coronary heart disease morbidity and mortality, or both are needed to ascertain the efficacy of long-term probucol use in relation to development of atherosclerosis.
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PMID:Colestipol and probucol: treatment of primary and familial hypercholesterolemia and amelioration of atherosclerosis. 703 45

Apolipoprotein E isomorphs in very low density lipoproteins and apolipoprotein B of low density lipoproteins were measured in the plasma of normolipidemic subjects with xanthelasmas of the eyelids and in appropriate control groups. All patients tested in the experimental group had an apolipoprotein EII to apolipoprotein EIII ratio typical of the heterozygous state for familial dysbetalipoproteinemia, a hyperapobetalipoproteinemia, or both. Some patients had concomitant atherosclerosis. This is the first report of an increased frequency of the apolipoprotein E-ND phenotype in normolipidemic xanthelasma. This condition should not be dismissed as benign; tissue lipid deposition in the absence of hyperlipidemia might be related to the presence of lipoproteins of abnormal composition with an enhanced atherogenic potential.
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PMID:Increased Frequency of Apo E-ND phenotype and hyperapobeta-lipoproteinemia in normolipidemic subjects with xanthelasmas of the eyelids. 705 63

500 mg of probucol were given twice a day for 6 months to 20 type II hyperlipoproteinemic patients, 14 men and 6 women, including 10 type IIb and 10 type IIa cases. Tendon xanthomas were present in 11 and xanthelasma in 4. Their mean blood cholesterol level was 435 +/- 100 mg/100 ml and triglycerides 210 +/- 138 mg/100 ml. A normal diet was maintained during the treatment period. Skin biopsies were made on the forearm, before and after the 6 month treatment. After lyophilization of the skin fragments, the free and esterified cholesterol contents were measured by gas chromatography after preparative thin layer chromatography on silica gel. The free and esterified cholesterol contents of the skin both appear to be significantly increased when the values found before treatment in these patients are compared with those of skin analysis in 10 normal controls: 2.25 to 1.58 micrograms/mg freeze-dried skin, p less than 0.001 for the esterified fraction. After 6 month probucol treatment the free cholesterol does not change significantly (2.25 to 2.16) and the esterified cholesterol increases (0.44 to 0.66, p less than 0.01). This effect is suggestive of an interaction of probucol with the synthesis and transport of cholesterol at the tissue level. It may be significant for the understanding of the effects of this drug and others on xanthelasma, tendon xanthomas, and atherosclerosis. In one of the cases studied here, the xanthelasma was greatly reduced during the treatment.
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PMID:Effects of probucol on the cholesterol content of skin in type II hyperlipoproteinemias. 709 77

Serum lipids and lipoprotein lipids were studied in 53 patients (21 males and 32 females) with xanthelasma palpebrarum and 40 age-matched normal controls (20 males and 20 females). Patients were subdivided into patients with normolipidemia, hyperlipidemia or familial hypercholesterolemia (FH). In both male and female patients with hyperlipidemia or FH, the serum cholesterol (Chol) levels were significantly higher than in normal controls. In both male and female patients with normolipidemia or hyperlipidemia, the VLDL-Chol levels were significantly higher than in normal controls. Male patients with FH showed significantly higher levels of VLDL-Chol than normal controls. Both male and female patients with normolipidemia, hyperlipidemia or FH showed significantly higher levels of LDL-Chol, lower HDL-Chol levels and lower HDL-Chol/LDL-Chol ratios than normal controls. In both male and female patients with hyperlipidemia and in male patients with FH, the serum triglyceride (TG) levels were significantly higher than in normal controls. Both male and female hyperlipidemic patients showed significantly higher levels of VLDL-TG than normal controls. In male patients with FH, the VLDL-TG levels were significantly above the control levels. In male patients with normolipidermia, the LDL-TG levels were significantly higher than in normal controls. In both male and female patients with hyperlipidemia or FH, the LDL-TG levels were significantly higher than in normal controls. The HDL-TG levels in patients with normolipidemia (males) or FH (females) were significantly lower than in normal controls. The prevalence of coronary heart disease in patients with normolipidemia, hyperlipidemia or FH was 29.4%, 24.0% and 45.4%, respectively.
Atherosclerosis
PMID:Serum lipids, lipoprotein lipids and coronary heart disease in patients with xanthelasma palpebrarum. 722 67

In an attempt to correlate xanthomas with atherosclerosis, the characteristics of serum lipid and lipoprotein profiles are explored in xanthoma patients. Xanthomas are classified into 5 subtypes: xanthelasma, planar xanthoma, papulo-eruptive xanthoma, tuberous xanthoma and tendon xanthoma. The clinical characteristics of xanthoma patients are summarized in the following. 1) Xanthelasma in 2 different types: one normolipemic and the other hyperlipidemic; of 30 xanthelasma patients, 5 were normolipemic, one of them had low HDL-cholesterol. 2) Tuberous and tendon xanthomas were all hypercholesterolemic, with serum cholesterol above 300 mg/dl and LDL-cholesterol above 255 mg/dl, while HDL-cholesterol was within normal range. 3) The xanthoma patients were generally not obese. 4) Their laboratory findings often showed such abnormalities as elevated levels in serum fibrinogen, LDH, CPK and uric acid. The resemblance of the clinical characteristics between xanthomas and atherosclerotic vascular disease, e.g., myocardial infarction, was striking. If the causation of their common tissue alterations by lipid accumulation is pathologically and biochemically defined, the correlation between those 2 kinds of disease can be established.
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PMID:Serum lipid and lipoprotein profiles in patients with xanthomas: a correlative study on xanthoma and atherosclerosis (I). 730 4

The WHHL-rabbit (Watanabe-heritable hyperlipidemic rabbit) is a strain of rabbit with a consistently inherited hyperlipidemic trait produced by inbreeding from a mutant discovered in 1973. (1) The alleles for this trait have been inherited unfailingly to yield a total of 154 WHHL-rabbits to date. (2) These rabbits showed abnormally increased serum levels of cholesterol (S-Ch) and triglyceride (S-TG) at various ages, increased approximately 8- to 14-fold in comparison with control levels in normal Japanese white rabbits. The serum lipoproteins displayed an electrophoretic pattern characterized by a broad beta-lipoprotein band a markedly diminished alpha-lipoprotein band. (3) Necropsy examination performed on 75 WHHL-rabbits showed spontaneous development of aortic atherosclerosis in all cases over 5 months of age (63/63) and xanthoma of digital joints in 60% of cases from the ages of about 16 months.
Atherosclerosis 1980 Jun
PMID:Serial inbreeding of rabbits with hereditary hyperlipidemia (WHHL-rabbit). 740 53

Xanthomas ordinarily occur in the skin and tendons of patients with severe hyperlipidemia. These xanthomas include xanthelasma, tuberous xanthoma, tendon xanthoma and eruptive xanthoma. Two patients with hypercholesterolemia are now described with xanthomas found in other locations, one deep in the mediastinum and one in the muscles of the buttock. These masses simulated the occurrence of neoplastic tumors but on pathological and chemical examination proved to be typical xanthomata. Ectopic xanthomas should be considered in the differential diagnosis of masses in patients with familial hypercholesterolemia.
Atherosclerosis 1980 Oct
PMID:Ectopic xanthomas in familial (type II) hypercholesterolemia. 742 5


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