Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0242339 (dyslipidemia)
 13,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To investigate early detection of young families with inherited dyslipidemia, we assessed changes in circulating apolipoprotein (apo) B and A-I levels and the apo B/A-I ratio during the 1st year of life and their relations to parental values. After measuring initial dried blood spot capillary blood levels in 919 babies when aged 4.25 +/- 0.98 d (mean +/- SD), we recalled at a mean age of 8.5 +/- 2.3 mo those with levels in one or more of the following categories: the top 5% of apo B values (group 1), the top 5% of apo B/A-I ratios (group 2), the bottom 5% of apo A-I values (group 3), and a group of 18 infants (group 4) who were not in any of the above three categories. We thus obtained serum lipid measurements in 51 infants and their parents. Two of the 13 group 1 infants had persistent hyperapo B at 8.5 mo; there was an inverse relationship between apo B and apo A-I levels in this group (p less than 0.001). Two of 11 babies in group 2 had lipid profiles consistent with familial hypercholesterolemia. However, none of the infants in group 3 had evidence at 8.5 mo of abnormal apo A-I or HDL levels, nor did their parents. For groups 1, 2, and 3, there was at 8.5 mo a regression of apo B and A-I toward mean levels for the "normal" 18 babies of group 4. In contrast, the high apo B/A-I ratios in group 2 remained high (p less than 0.01) and showed evidence of tracking.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Apolipoproteins A-I and B and the B/A-I ratio in the first year of life. 180 51

The frequency of familial dyslipidemia syndromes was determined from blood tests in 33 objectively ascertained families with early coronary heart disease (CHD) (two or more siblings with CHD by the age of 55 years). Three fourths of persons with early CHD in these families had 90th percentile lipid abnormalities (cholesterol level at or above the 90th percentile, triglyceride level at or above the 90th percentile, and/or high-density lipoprotein cholesterol (HDL-C) level at or less than the 10th percentile). The HDL-C and triglyceride abnormalities were twice as common as low-density lipoprotein-cholesterol abnormalities. The most common syndromes found were familial combined hyperlipidemia (36% to 48% of families with CHD), familial dyslipidemic hypertension (21% to 54% of families with CHD), and isolated low levels of HDL-C (15%), with overlapping familial dyslipidemic hypertension with familial combined hyperlipidemia and low-level HDL-C. Well-defined monogenic syndromes were uncommon: familial hypercholesterolemia being 3% and familial type III hyperlipidemia, 3%. Another 15% of families with CHD had no lipid abnormalities at the 90th percentile. Physicians should learn to recognize and treat these common familial syndromes before the onset of CHD by evaluating family history and all three standard blood lipid determinations. Failure to recognize and treat them leaves affected family members at high risk of premature CHD.
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PMID:Population-based frequency of dyslipidemia syndromes in coronary-prone families in Utah. 231 Feb 76

In autoimmune hyper- or dislipidemia secondary to a monoclonal antilipoprotein gammapathy, immunoglobulin-lipoprotein (Ig-Lp) complexes are found in the circulating blood. In order to determine their possible significance in common types of hyperlipidemia we compared the Ig-Lp content of sera from 98 healthy blood donors and 155 outpatients from a Lipid Clinic, including 91 cases of hypercholesterolemia (55 familial and 36 non-familial), 15 cases of hypertriglyceridemia, 20 cases of mixed hyperlipidemia and 29 miscellaneous cases. Detection of the Ig-Lp was performed by an ELISA technique with polyclonal affinity purified anti-LDL + HDL as capture antibodies and peroxidase-labeled anti-Ig antibodies specific for IgA, IgG, IgM heavy chains as indicators. Two cases of monoclonal gammapathy (one IgA K and one IgG L) with dislipidemia served as positive controls for the test. IgG, IgA and IgM Lp were found in the sera of the blood donors, in very small quantities when compared with the monoclonal gammapathy cases. All three types of Ig-Lp were also found in the different hyperlipidemic populations studied. When blood donors were compared to hyperlipidemic patients, no difference was observed for IgG Lp. A significant increase in IgM Lp was found in patients with familial hypercholesterolemia (P less than 0.01). An increase in IgA Lp was also found in hypercholesterolemia, familial or not (P less than 0.01), and in patients with corneal arcus (P less than 0.0001), ischaemic disease (P less than 0.01), tendon xanthomas (P less than 0.05) or xanthelasma (P less than 0.05). Furthermore, in a group of 18 paired parents from 9 different families, positive interparent correlations were found for IgM Lp (r = 0.78; P = 0.013) and IgG Lp (r = 0.69; P = 0.038). Therefore IgM Lp may be markers for subpopulations of familial hypercholesterolemia, and IgA Lp markers for the risk of atherosclerotic ischemic disease and deposition of lipids in the cornea. It may be (1) that natural clones of autoanti-lipoprotein antibodies are responsible for the minute quantities of Ig-Lp found in normal people; (2) that the marked development of one of these clones is the cause of autoimmune hyper- or dyslipidemia and xanthomatosis associated with monoclonal gammapathy; (3) that the limited development of a clone produces the Ig-Lp particles found in hypercholesterolemic patients; (4) that there are types of Ig-Lp particles (IgA Lp) that may be harmful for tissues independently of hypercholesterolemia.
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PMID:Immunoglobulin-bound lipoproteins (Ig-Lp) as markers of familial hypercholesterolemia, xanthomatosis and atherosclerosis. 324 Mar 31

A Cambodian male (aged 5 years and 9 months) presented with subcutaneous and tendon xanthomas in association with hypercholesterolemia. He was erroneously diagnosed as having familial hypercholesterolemia and treated with a low cholesterol diet (+/- cholestyramine) to which he did not respond. A determination of plasma total lipid profile by high-temperature gas chromatography revealed elevated plasma levels of free and esterified plant sterols along with the hypercholesterolemia. Introduction and maintenance of a diet low in cholesterol and plant sterols resulted in significant reduction in the blood concentration of these sterols, which returned to pretreatment level upon discontinuation of the low sterol regimen. The rapid identification and quantitation of the plant sterols by high-temperature gas chromatography provides a sensitive means of distinguishing phytosterolemia, which might be more common than previously suspected, from other forms of dyslipidemia, and for following the course of any treatment.
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PMID:Usefulness of gas chromatographic profiles of plasma total lipids in diagnosis of phytosterolemia. 377 8

A previously healthy 9-month-old girl, obese (12,500 gm) with sudden onset of hyperthermia (40 degrees C), generalized tonic-clonic seizures, followed by focal seizures, drowsiness, left facial nerve palsy, left lagophthalmos and mydriasis is presented. CT-scan and MRI suggested temporal-parietal infarction due to Sylvian artery occlusion in a 9-month-old infant with familial hypercholesterolemia (type 2 A dyslipidemia). The possibility of a cerebral abscess or herpetic encephalitis was considered. Negative clinic and serologic results excluded this diagnosis. The management of the symptomatology was made with parenteral antibiotics, anticonvulsive and antioedematous cerebral therapy. Favourable evolution with residual left hemiparesis after 30 days, when the child was discharged. CT-scan reevaluation (after 5 months of evolution) showed a hypodense temporal-parietal area abnormality due to a right ischemic infarction.
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PMID:Acute hemiplegia in one infant. 777 44

Significant risk factors for premature coronary heart disease include: (1) family history, (2) elevated low density lipoprotein (LDL) cholesterol level > or = 160 mg/dl, l, (3) decreased high density lipoprotein (HDL) cholesterol level < 35 mg/dl, l, (4) cigarette smoking, (5) high blood pressure and (6) diabetes mellitus. All of these risk factors are common in patients with premature heart disease. Common familial lipid disorders associated with premature heart disease include familial lipoprotein(a) excess, familial dyslipidemia (elevated triglycerides and decreased HDL cholesterol), familial combined hyperlipidemia (elevations of LDL cholesterol and triglycerides, and often decreased HDL cholesterol), familial hypoapobetalipoproteinemia (elevated apolipoprotein B levels), familial hypoalphalipoproteinemia (low HDL cholesterol levels), and familial hypercholesterolemia (elevated LDL cholesterol levels). All these disorders have been characterized using age and gender specific 90th and 10th percentile values from the normal population. The diagnosis and potential management of these disorders is reviewed.
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PMID:Familial lipoprotein disorders and premature coronary artery disease. 780 28

Atherosclerosis develops rapidly in patients with diabetes or renal insufficiency. Plasma lipoprotein profiles are frequently abnormal in these conditions and reflect an elevation in the level of the apoprotein B (ApoB)-containing components very low density lipoprotein (VLDL) and low density lipoprotein (LDL). High levels of circulating advanced glycation end products (AGEs) also occur in diabetes and end-stage renal disease (ESRD). These products arise from glucose-derived Amadori products and include AGE-modified peptides (AGE-peptides) which result from the catabolism of AGE-modified tissue proteins. AGE-peptides have been shown to crosslink protein amino groups and to accumulate in plasma as a consequence of renal insufficiency. To address potential mechanisms for the dyslipidemia of diabetes and ESRD, we investigated the possibility that circulating AGEs react directly with plasma lipoproteins to prevent their recognition by tissue LDL receptors. AGE-specific ELISA showed a significantly increased level of AGE-modified LDL in the plasma of diabetic or ESRD patients compared with normal controls. AGE-LDL formed readily in vitro when native LDL was incubated with either synthetic AGE-peptides or AGE-peptides isolated directly from patient plasma. LDL which had been modified by AGE-peptides in vitro to the same level of modification as that present in the plasma of diabetics with renal insufficiency exhibited markedly impaired clearance kinetics when injected into transgenic mice expressing the human LDL receptor. These data indicate that AGE modification significantly impairs LDL-receptor-mediated clearance mechanisms and may contribute to elevated LDL levels in patients with diabetes or renal insufficiency. This hypothesis was further supported by the observation that the administration of the advanced glycation inhibitor aminoguanidine to diabetic patients decreased circulating LDL levels by 28%.
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PMID:Modification of low density lipoprotein by advanced glycation end products contributes to the dyslipidemia of diabetes and renal insufficiency. 793 86

Total cholesterol levels, obesity index and blood pressure were measured in 6,278 school-age children living in Ibaraki Prefecture in 1991, and children with high risk for atherosclerosis were identified. The frequencies of the school-age children with hypercholesterolemia (total cholesterol > or = 200 mg/dl), obesity (obesity index > or = 40%) or hypertension were 7%, 5%, 1%, respectively. In half of the area where the children lived, lipid measurements were also obtained in the parents of hypercholesterolemic children. Twenty-nine out of ninety fathers (32%) and 22 out of 140 mothers (16%) were hypercholesterolemia (total cholesterol > or = 240 mg/dl). Among them five families of familial hypercholesterolemia were diagnosed. Seventy children with hypercholesterolemia and 81 obese children, who were screened and received health counseling, were re-examined after one year. The levels of LDL-cholesterol, triglyceride and atherogenic index were significantly decreased and HDL cholesterol level was significantly increased in the children with hypercholesterolemia. Obesity index, triglyceride level and atherogenic index were significantly decreased and HDL cholesterol level was significantly increased in the children with obesity. In addition, the frequencies of the children with dyslipidemia or liver dysfunction were significantly decreased in the obese children after one year. These data suggested that the screening system and the plans after the examinations described here were effective in reducing risk factors for atherosclerosis in children.
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PMID:[Cardiovascular risk factors among Japanese school-age children: a screening system for children with high risk for atherosclerosis in Ibaraki, Japan]. 811 Oct 84

Although there is consensus that lipid variables, especially lipoprotein(a), are heritable and that elevated LDL cholesterol levels should be treated, there are no clear definitions of the common familial lipid disorders associated with premature CHD (lipoprotein(a) excess, FCH, familial dyslipidemia, familial hypoalphalipoproteinemia, familial hypercholesterolemia), nor do we have clear guidelines for the treatment of most of these disorders. Implementation of therapy for elevated LDL cholesterol in familial lipid disorders often has not occurred even in the United States. Before recommendations can be made for subjects with lipoprotein(a) excess and HDL deficiency (who often have combined hyperlipidemia or hypertriglyceridemia), prospective studies documenting benefit of CHD risk reduction must be carried out in subjects with lipoprotein(a) excess and HDL deficiency. One such study is being carried out with gemfibrozil in CHD patients with HDL deficiency. Current data do justify treatment of CHD patients with lipoprotein(a) excess with niacin because niacin has been shown to lower lipoprotein(a) levels as well as lower CHD risk mortality in random CHD patients. With regard to CHD patients with or without HDL cholesterol levels less than 35 mg/dL (0.9 mmol/L), efforts should be made to optimize their lipid profile and reduce their LDL cholesterol levels to less than 100 mg/dL (2.6 mmol/L).
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PMID:Familial lipoprotein disorders and premature coronary artery disease. 828 32

The review examines the evidence that the supply of cholesterol available for incorporation into nascent lipoprotein particles exerts a regulatory influence on apolipoprotein (apo) B secretion by the liver. Support for this hypothesis comes both from in vitro experiments and from recent observations in normal subjects and patients with dyslipidemia associated with familial hypercholesterolemia, obesity, noninsulin dependent diabetes mellitus, growth hormone deficiency and cholesteryl ester storage disease. The findings do not negate a role for triglyceride synthesis in determining apoB secretion in very low density lipoprotein, but the inhibitory effects on the latter process of pharmacological blockade of cholesterol synthesis or esterification suggest that it is conditional upon an adequate supply of cholesteryl ester.
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PMID:Role of cholesterol in regulating apolipoprotein B secretion by the liver. 872 9


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