Different doses of 3-hydroxy-3-methyl-glutaric acid (HMG) on plasma lipids were studied in a double-blind trial in 36 patients with familial hypercholesterolemia (type IIa or hyper-beta-lipoproteinemia). The patients were randomly assigned to five groups, and each group received one of the following treatments: placebo, or HMG 750 mg, 1500 mg, 2250 mg, or 3000 mg per day. As compared to placebo, the mean plasma cholesterol levels during the eight-week treatment period were 11 and 13 per cent lower in the 2250-mg and 3000-mg HMG-treated groups (P less than 0.034 and less than 0.021, respectively). At the same dosage levels LDL cholesterol was decreased by 8 per cent. HMG had no significant effect on plasma triglycerides as compared to placebo. Discontinuation of the medication did not result in a rebound of plasma cholesterol. There were no clinical or biologic adverse effects due to the administration of HMG, and all patients maintained excellent compliance to the medication. Because of its lack of toxicity, HMG may be useful as an adjunct to diet in the treatment of familial hypercholesterolemia.

Adult↗

PubMedDate not supplied

Homozygous familial hypercholesterolemia among French Canadians in Québec Province.

Nineteen patients with homozygous familial hypercholesterolemia (FH) living at the time of the 1981 Canada census are the subject of this report. Their mean age at that time was 15, with a range of 1 to 26 years. All patients had extensive xanthomatosis but showed variable clinical manifestations of coronary heart disease (CHD); five (mean age, 21; range, 11 to 27 years) died from sudden death due to CHD. Plasma cholesterol levels varied more than twofold (557 to 1532 mg/dl). Variation in the concentrations of both plasma and low density lipoprotein cholesterol, as well as apolipoprotein B, were related neither to age at death from CHD nor to the clinical course of CHD. The mean high density lipoprotein cholesterol concentration (37 mg/dl) was lower than the mean value (49 mg/dl) in the control population (p less than 0.001). Both the clinical and biochemical features of this cohort are typical of homozygous FH. The prevalence of homozygotes among French Canadians in Québec was approximately 1:275,000, and the minimum estimated frequency of heterozygotes was 1:270. In northeastern Québec, the frequency of homozygotes was approximately 1:100,000, and the minimum estimated frequency of heterozygotes was 1:154. Only Afrikaaners in South Africa have correspondingly higher frequencies.

Adolescent↗

PubMedDate not supplied

Gemfibrozil therapy in primary type II hyperlipoproteinemia: effects on lipids, lipoproteins and apolipoproteins.

Gemfibrozil lowers triglycerides, low density lipoprotein (LDL) and very low density lipoprotein (VLDL) cholesterol. It also promotes a significant increase of high density lipoprotein (HDL) cholesterol. It has been established that normalization of apolipoproteins is an important protective factor against atherosclerosis. The present report examines the effectiveness of 12 months of gemfibrozil treatment on plasma lipids and apolipoproteins in types IIa (VLDL 18 +/- 2 mg cholesterol/dL) and IIb (VLDL 58 +/- 7 mg cholesterol/dL) hypercholesterolemic patients. Gemfibrozil lowered plasma triglycerides, VLDL cholesterol and apolipoprotein B (apoB), increased HDL cholesterol and apoAI levels in both groups, and induced a very substantial reduction in LDL cholesterol in type IIa patients only. Even though HDL particles were enriched in cholesterol, indicating improvement in the reverse cholesterol transport and lower risk of atherosclerosis in both groups, it is important to note that production of cholesterol-poor LDL particles and reduction in LDL cholesterol and the LDL/HDL cholesterol ratio were observed only in the normotriglyceride group (type IIa). Due to the initially elevated concentration of plasma triglycerides and VLDL in type IIb patients and the increased catabolism of VLDL to LDL during gemfibrozil therapy, this drug has a more efficient regulating effect on LDL particles in type IIa compared with type IIb hyperlipidemia.

Adult↗

PubMedDate not supplied

[Enoximone and the therapeutic strategy in patients awaiting emergency cardiac graft].

Enoximone is a positive inotropic agent belonging to the group of phosphodiesterase F-III inhibitors. The drug was tested in 34 patients uncontrolled by sympathomimetic drugs and referred to our department for urgent heart transplantation or circulatory assistance. After insertion of a Swan-Ganzgatheter and a radial artery catheter for haemodynamic monitoring, enoximone was administered as a 15-minute intravenous bolus injection of 1 to 2.5 mg/kf every 8 hours, in addition to sympathomimetic agents. Clinical and haemodynamic improvement was observed after thirty minutes in 30 patients. The cardiac index rose from 1.82 to 2.67 l/min/m2 and the pulmonary wedge pressure fell from 30.8 to 18.9 mmHg. Systemic arterial resistance decreased from 2170 to 1520 dyn. s. cm-5, and pulmonary resistance from 5.5 to 4.6 Wood units (p less than 0.01 for all values). Four patients had no haemodynamic improvement and were put on circulatory assistance, using a Jarvik 7 total artificial heart in 3 of them and heterotopic circulatory assistance in one. After clinical investigation for contra-indication to heart transplantation, and as their improved haemodynamic status permitted, 12 of the 30 patients were considered suitable (group B) for heart transplantation. Transplantation was performed within a week of admission in 11 patients without any need for mechanical assistance. One of the group B patients who required implantation of a Jarvik 7 artificial heart died after 12 hours of assistance. Eighteen patients were considered unsuitable for transplantation (group A) and treated medically.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗