Kaposi's sarcoma and HTLV-I infection.
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Biomedical subjects
Publications and source records attributed to F Dairou.
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The serum lipoprotein Lp(a) concentration was measured in 1065 individuals in order to assess whether there was a relation between the type of dyslipidemia and the level of Lp(a). Males and females, aged between 2 and 83 years old, were included in the study. Quantification was performed by an immunonephelometric technique. The whole population was divided into normolipidemic (NL), type IIa without xanthoma (type IIa), type IIa with xanthoma (FH), type IIb and type IV phenotypes. Lp(a) level was arbitrarily divided into 5 subclasses in each group of dyslipidemia and in the normolipidemic group. In addition each group was divided according to sex and whether or not they were under treatment. We observed a significant difference between the median Lp(a) level of the normolipidemic group (NL) and of the dyslipidemic group as a whole. Median Lp(a) levels in the 4 dyslipidemic groups did not differ significantly. Sex, age and treatment did not influence the distribution of Lp(a) values distribution. Only weak correlations (Spearman's rank test) were observed between Lp(a) and other lipid parameters (total cholesterol, LDL, apo B, HDL, triglycerides): the highest correlation (r' = 0.15) was between Lp(a) and apo B. We conclude that Lp(a) level is not influenced by the type of dyslipidemia, sex or hypolipidemic drugs.
Three groups of drugs can be used in hypercholesterolemia: 1) Drugs chelating bile acids (resins) which stimulate LDL receptors. 2) HMG R inhibitors which competitively inhibit the enzyme and increase the synthesis of LDL receptors. 3) Fibrate derivatives which are second line drugs in pure hypercholesterolemia. They should be used in case of intolerance of resins and may be preferred to inhibitors in moderate forms for economic reasons of lower cost. However fibrates are the treatment of choice in hypertriglyceridemia and mixed hyperlipidemias. All fibrates increase HDL cholesterol. 4) Probucol, neomycin and nicotinic acid are adjuvant hypolipidemic agents. The following steps must precede the prescription of a hypolipidemic drug: 1) Classification of the disorder. 2) Elimination of secondary dyslipidemias. 3) Family assessment. 4) An absolutely essential dietary phase. 5) This is permanent treatment without gaps. 6) No other atherogenic risk factor should be allowed to persist. 7) Iatrogenic effects must be avoided.
In a prospective study of 20 patients with primary hyperparathyroidism the diagnostic value of the intact parathormone assay was compared with that of intermediate fragment measurement and that of nephrogenic cyclic AMP determination. In the basal state, measuring the intact parathormone is the best way of separating hyperparathyroid patients form normal subjects. During calcium infusion tests, there is good concordance between changes in intact parathormone and in nephrogenic cyclic AMP. In addition, the intact parathormone assay enables a thorough study of the relationship between ionized calcaemia and parathyroid secretion to be performed and confirms the existence, in hyperparathyroid patients, of two types of secretory response to a rise in calcaemia. Being more reliable than the measurement of a C-terminal or intermediate fragment and easier to perform than nephrogenic cyclic AMP determination, the intact parathormone assay seems to be particularly suitable for the study of primary hyperparathyroidism.
High circulating levels of coagulation factor VII (FVII) are known to be associated with elevated concentrations of blood lipids. More specifically, hypertriglyceridemia is correlated with raised FVII coagulant activity (FVIIc). Recently, evidence has been described which suggests that elevation in FVIIc might reflect an increase in the total concentration of FVII, as evaluated by quantitation of FVII antigen (FVIIag). It is also known that FVIIc represents a risk factor for cardiovascular death, but the prediction of cardiovascular risk based on triglyceride estimation is the subject of conflicting results. Since dyslipidemia featuring abnormal triglyceride metabolism is pathophysiologically heterogeneous, we analyzed the possible relationships between FVII and triglyceride further and under standardized postprandial conditions. For this purpose we studied FVIIc and FVIIag in relation to triglyceridemia after a standardized test meal. Our results confirm that FVIIc and FVIIag levels are strongly correlated with each other (r = 0.714; p = 0.01). In addition, both were significantly increased (p less than 0.02) in patients who exhibited abnormal triglyceride levels 8 h after a standardized test meal, as compared to those who displayed normal triglyceridemia. Furthermore, we found that apolipoprotein B levels were also increased in such patients. The deficient postprandial catabolism of triglycerides, therefore, appears to be related to an increase in total FVII concentration, suggesting that some association between the metabolism of triglyceride-rich lipoproteins and FVII might underlie the mechanism of the elevation in FVII. Given the important contribution of FVII to hypercoagulability, then our results may be relevant to the understanding of the role of postprandial triglyceride in atherogenesis and in consideration of the circadian prevalence of cardiovascular thrombosis.
A plasma exchange program for familial hypercholesterolaemia was started in 1982. Ten patients aged from 7 to 58 years were progressively included: 3 had an heterozygous form of the disease with ischaemic heart disease; 3 had an homozygous form with defective low density lipoprotein receptor activity, 4 had a receptor-negative homozygous familial hypercholesterolaemia and had previously undergone portacaval shunt. During total plasma exchange against human albumin (470 sessions in 9 patients) low density lipoprotein cholesterol values, but also high density lipoprotein cholesterol values, decreased by 40 per cent. More recently, 5 patients had selective low density lipoprotein absorption on dextran sulfate column (Liposorber); 90 exchanges were performed. High density lipoprotein cholesterol values decreased by 55 per cent and high density lipoprotein cholesterol values by only 27 per cent. The patients' attitude to treatment was excellent, with less fatigue and better compliance.
High density lipoproteins (HDL), also called alpha lipoprotein, are small high protein content particles that are present in the vascular and extravascular systems. Two main classes of HDL, HDL2 and HDL3, have been identified and found to have different structures and different metabolisms. Their principal function is to ensure that tissue cholesterol returns to the liver. HDL, and notably HDL2, are factors that protect against cardiovascular diseases. HDL2 are subjected to the influence of numerous dietetic, genetic and hormonal factors, while HDL3 seem to be more independent of all variations, except those of alcohol.
We studied the progression of coronary angiographic findings in a series of 10 patients with homozygous familial hypercholesterolaemia. All patients had medical treatment; six had additional surgical treatment. They were followed over a period of 2-10 years (mean follow-up five years). Three types of evolution were observed: (a) normal coronary angiography with no development of any lesion over a period of eight years in two young patients; (b) regression of proximal coronary stenoses in three patients, and (c) progression of coronary angiographic status in five patients. A different evolution of proximal and distal segment lesions was observed; while more than two-thirds of proximal segments stenoses improved and no proximal lesion increased with a 45% decrease in plasma cholesterol level, no improvement was obtained at the distal level. Distal lesions tended to increase and new lesions appeared in spite of a major plasma cholesterol level reduction. When early treatment results in a cholesterol plasma level decrease of more than 45% and a level of about 4 g l-1 over a very long period of time, the coronary prognosis may be good.
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The authors report three observations of cerebro-tendinous xanthomatosis (CTX). The three patients presented tendinous xanthoma and cataract. The neurologic disorders were different in each case. The first one, a 43 years old woman suffered from dementia, ataxia and pseudobulbar palsy: CT scan showed cerebellar hypodense lesions. After the apparition of bulbar signs ans cachexia she died at 45. The second patient, a 39 years old man had an ataxia and mild psychiatric disorders. He was stabilized with a treatment of chenodesoxycholic acid. The third one, a 49 years old women suffered only from tendinous xanthoma, cataract, and had no neurological disorder. His plasmatic cholestanol level was high. CTX is a recessive deficit of the hepatic 26 hydroxylase with deposits of abnormal metabolites in tendons, crystalline lenses and central nervous system. Reviewing the 44 observations of CTX in the literature, the authors define the genetical, clinical, biochemical and therapeutical aspects of CTX, and underline the necessity of a early diagnosis with cholestanol dosage, before the apparition of neurological disorders and the short terminal phase. CTX is a rare but fortunately treatable neurolipidosis.
Atherogenic hyperlipidemias are the commonest types of primary hyperlipidemia and include pure hypercholesterolemia, endogenous hypertriglyceridemia and mixed hyperlipidemia. They affect almost 4% of the population.
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All types of hyperlipoproteinaemia must be corrected to prevent or delay the atheroma they produce, or even bring about its regression. A dietetic regimen is prescribed first to be completed, if necessary, by a medicinal treatment. Once instituted, these treatments must be pursued indefinitely, the return to normal lipid levels being a sign of their effectiveness and not a reason for their discontinuation. Conversely, failure to obtain normal lipid levels indicates that the treatment should be modified, especially the dietetic regimen which, when not regularly adhered to, often is the cause of resistance of hyperlipoproteinaemia. One must watch for iatrogenic complications in order to detect and prevent them. The drugs most frequently prescribed for hypercholesterolaemia are resins that absorb biliary acids, fibrates, probucol and, very soon, HMG coareductase inhibitors, and for mixed hyperlipidaemia and hypertriglyceridaemia, fibrates and, accessorily, nicotinic acid.
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Compared with other hyperlipoproteinaemias, minor hypertriglyceridaemia of type IV in Fredrickson's classification (serum concentrations below 5 g/l) has been the object of few studies. From a study of 235 patients presenting with this metabolic abnormality, 64 were isolated who developed vascular lesions. Our patients' recruitment system makes it impossible for us to provide figures concerning the frequency of vascular lesions, but it enables us to present two important qualitative assessments. Firstly, a different profile seems to differentiate patients with atheromatous lesions from those with accidents of arterial thrombosis. The latter are heavier, consume significantly greater amounts of tobacco and alcohol and are significantly younger when vascular complications occur. Metabolically, they have a lower blood cholesterol/triglycerides ratio and a lower serum concentration of apolipoprotein B. In those who develop myocardial infarction compared with those who suffer from angina the cholesterol/triglyceride ratios are 1.38 and 0.91 respectively, and the serum levels of apolipoprotein B are 1.57 g/l and 1.20 g/l respectively (normal value: 1.30 g/l). Secondly, the very high frequency in our population of inaugural myocardial infarction is an original clinical finding. It provides and additional argument in support of the theory that hypertriglyceridaemia may be a contributing factor of arterial thrombosis. This study emphasizes the point that metabolically as well as clinically pure hypertriglyceridaemia is a very heterogeneous metabolic abnormality, as shown by its repercussions on the vascular system.
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