Effect of Long Term Treatment of Four Kinds of Cholesterol Lowering Drugs for Familial Hypercholesterolemia

We studied effects of long term treatment of four cholesterol lowering drugs; compactin, cholestylamine, probucol and niceritorol on lipid and lipoprotein concentration and on the incidence of coronary heart disease in 111 patients with familial hypercholesterolemia (FH).1. In 4 patients with homozygous FH, mean serum cholesterol levels decreased by 24 per cent with drug treatment of large dose of compactin in one patient and of 1-1.5g/day probucol in the other three patients. One patient who was suffered from myocardial infarction before treatment died of heart attack during follow-up period. Other three patients were performed AC bypass operation and their anginal attacks were controled well.2. In heterozygous patients, mean serum cholesterol levels decreased by 19 per cent. Three drugs which are known to effective for FH decreased cholesterol levels by -26±11%, -13±8% and -15±7%, resspectively. In addition, niceritorol was also effective for heterozygous FH when the sufficient doses could be given without side effect such as flashing. More than 5 per cent decrease in Achilles tendon thickness (ATT) was found in one-third patients. Although the regression of ATT required the reduction of serum cholesterol level, the reduction of ATT and that of serum cholesterol level was not closely correlated. In the patients treated with probucol, the regression of ATT was correlated with the reduction of HDL-cholesterol level.3. During follow-up period, three patients suffered of fatal or nonfatal myocardial infarction. All three patients had had manifest coronary artery disease at entry. The patients without coronary artery disease at entry did not suffered from new cardiac events during the period. Even if the patients had coronary artery disease at entry, combination therapy of surgery with hypolipidemic drugs could improve their prognosis.In conclusion, assessment of coronary arterial lesion and treatment with cholesterol-lowering drugs might be needed before the manifestation of coronary heart disease in patients with FH.