[Pseudohypertriglyceridemia due to hyperglycerolemia caused by glycerol kinase deficiency].
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Biomedical subjects
Publications and source records attributed to J Rouffy.
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The authors attempted to evaluate through a literature review, lipid and lipoprotein changes induced by anti-hypertensive drugs (especially diuretics and beta-blockers). Generally, these drugs induce a modification of the lipid and lipoprotein profile towards potential atherogenicity by a moderate increase in total cholesterol, a marked increase in triglycerides and very-low-density lipoprotein triglycerides, and a decrease in high-density lipoprotein cholesterol. In theory, these changes could decrease or cancel the reduction in cardio-vascular risk obtained through the antihypertensive treatment but current data do not allow to confirm or disprove this hypothesis. However, it is certain that adherence to a suitable life-style and good dietary habits tend to reduce the associated risk factors and compensates, at least in part, for the effects of hypotensive drugs on the lipids and lipoproteins.
We report the effects of sublingual absorption of a single dose (0.5 mg) of oestradiol-17 beta (E2) in 8 post-menopausal women, on plasma E2 and oestrone (E1), urine elimination of total E2 + E1 and on plasma FSH and LH. The results show that sublingual absorption of E2 occurs and that plasma concentrations of E2 obtained (between 133.2 to 320 pmol/l) in this way were higher than those obtained after percutaneous absorption of a single dose (3 mg) of E2. The ratio E1/E2 in plasma is close to that of pre-menopausal women.
In a retrospective study about 600 hyperlipoproteinaemic patients (390 men and 210 women, mean age 47), xanthelasma palpebrarum was infrequent (25 cases or 4.16 p. 100), since it was only at the third rank of chronic extra-vascular lipid deposits after arcus corneus and tendineous xanthomas. It was twice as frequent in men (5.12 p. 100) as in women (2.38 p. 100) and of regularly increasing occurrence until the sixth decade. Among the atherosclerotic risk factors, only obesity was significantly associated. The existence of atherosclerotic diseases was observed more frequently in patients with xanthelasma palpebrarum (40 p. 100) than in total hyperlipoproteinaemic population (28 p. 100). In a second retrospective study concerning 29 patients (15 men and 14 women, mean age 50), selected from the only presence of one or several xanthelasma palpebrarum, a lipoproteinaemic abnormality was observed in 27 cases (93 p. 100): 23 patients (71 p. 100) were hyperlipoproteinaemic (type IIa, IIb or IV) and in 4 out of 6 normolipidemic patients we observed an abnormal lipoprotein cholesterol distribution (LDL + VLDL cholesterol increase and HDL cholesterol decrease). In this group, we observed in nine patients (31 p. 100) unknown atherosclerotic diseases and tendineous xanthomas in other nine patients (31 p. 100).
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The authors attempted to evaluate through a literature review, lipid and lipoprotein changes induced by anti-hypertensive drugs (especially diuretics and beta-blockers). Generally, these drugs induce a modification of the lipid and lipoprotein profile towards profile atherogenicity by a moderate increase in total cholesterol, a marked increase in triglycerides and very-low-density lipoprotein triglycerides, and a decrease in high-density lipoprotein cholesterol. In theory, these changes could decrease or cancel the reduction in cardio-vascular risk obtained through the antihypertensive treatment but current data do not allow to confirm or disprove this hypothesis. However, it is certain that adherence to a suitable life-style and good dietary habits tend to reduce the associated risk factors and compensates, at least in part, for the effects of hypotensive drugs on the lipids and lipoproteins.
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A 56-year-old woman with familial heterozygous type II hyperlipoproteinaemia and tendinous xanthomas, presented the unusual findings of acquired supravalvular aortic stenosis associated with a congenital stenosis of the orifice. Analysis of clinical, paraclinical, and particularly pathological data confirmed the existence of several associated lesions: congenital hypoplasia of the aortic ring and proximal aorta, calcified valve stenosis probably increased by the dyslipidaemia, and finally and principally, supravalvular stenosis formed of a veritable circular rim of atheromatous material, making up a second haemodynamic obstacle. It is probable that the pre-existing congenital aortic lesions in this case, apart from the hyperlipoproteinaemia, account for the observation, not previously reported in a heterozygous type II hyperlipoproteinaemia, of massive supravalvular atheromatous deposits. This latter of acquired lesion is described in 9 cases reported in the literature with a homozygous form of familial hypercholesterolaemic xanthomatosis, the atheromatous deposits in the proximal aorta appearing earlier in these patients, and in the absence of pre-existing anatomical anomalies.
In a study started in 1973 and still in progress (1100 patient-years so far) fenofibrate in daily doses of 200-400 mg consistently proved effective, without any loss of activity with time. The drug lowered serum total cholesterol levels by 17-27% in type IIa, IIb and III primary hyperlipoproteinaemia (HLP) and serum triglyceride levels by 35-51% in type IIb and III HLP and by 46-54% in type IV HLP. Clinically and biologically fenofibrate was always well tolerated, even after 5 years' treatment. Side-effects were uncommon (4%) and mild, and they obliged to discontinue treatment in only 1% of the patients. Abnormal manifestations encountered during therapy appeared to be fortuitous. The effects of the drug on cardiovascular morbidity and mortality could not be determined from this trial. In a short-term study involving 21 patients with type IIa and IIb primary HLP, fenofibrate in doses of 200-400 mg/day produced a significant decrease in total cholesterol, LDL-cholesterol and apoprotein B. It is also reduced triglycerides and VLDL-triglycerides in type IIb HLP. The increase in HDL-cholesterol observed under fenofibrate was significant in type IIa HLP but not in type IIb HLP. In both types, there was a significant rise in HDL: LDL + VLDL ratio.
Probucol was used to treat 16 patients with atherogenic hyperlipoproteinaemia. The duration of treatment varied between 30 days (16 patients) and 510 days (4 patients). The drug was found to be remarkably well tolerated, without any clinical or biological side-effects. There was an 8 to 13 p. cent reduction in plasma total cholesterol, a 4 to 17 p. cent reduction in LDL cholesterol and a very favorable increase (19 to 23 p. cent) in the HDL/LDL + VLDL cholesterol ratio. Little or no change was found in triglyceride and HDL cholesterol levels.
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Three methods of assaying the high density lipoprotein cholesterol were applied simultaneously to a hundred of human sera: ultracentrifugation, precipitation by concavalin A and phosphotungstic acid. The obtained results were compared by statistical means: it was shown that the three procedures were equivalent. Some of their analytical features are discussed.
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The authors determined in 3 populations (37 controls, 59 asymptomatic hyperlipemias, and 20 cases of arterial hyperlipemia) the total cholesterol, the HDL cholesterol and the LDL + VLDL cholesterol and their ratio by two methods : precipitation by concanavalin A and ultracentrifugation (AirFuge). These various lipid parameters were compared between 3 populations in order to determine their more or less discriminant character; furthermore, was established the degree of correlation between the results obtained by precipitation with concanavalin A and by ultracentrifugation. The results show : firstly, that the LDL + VLDL was significantly higher and the ratio HDL/LDL + VLDL significantly lower in hyperlipemia and in arterial disease compared with controls ; the HDL was significantly lower in arterial disease than in controls. No lipid parameter permitted one to differentiate between asymptomatic hyperlipemia and arterial disease. Furthermore, the correlation determined by the chi square method was very strong between the results obtained by precipitation with concanavalin A and ultracentrifugation as the coefficient of simple linear regression is very little different from the value + 1. They emphasize the interest of the method of precipitation by concanavalin A which is simple, rapid and very reliable.
Cholesterol and triglyceride content of serum lipoprotein fractions isolated by ultracentrifugation have been studied in 33 healthy subjects and in 56 subjects affected by hyperlipoproteinemia of type IIa, IIb and IV. Patients with atherosclerotic disease were characterized by a general decrease of HDL cholesterol and by a negative correlation between HDL cholesterol and VLDL triglycerides; patients with type IIb and IV showed an increase of LDL lipoproteins. The increase of triglycerides in type IIb and IV was caused by elevation of VLDL triglycerides or LDL triglycerides, and the increase of cholesterol in type IIb is sometimes caused by elevation of VLDL cholesterol. It is evident that several subtypes exist within Fredrickson's classification. Patients with cerebral arterial disease when compared with patients affected by non-ischaemic disease, showed a negative and significant correlation between HDL cholesterol and total cholesterol.
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