FAMILIAL LIPOPROTEIN DISORDERS AND PREMATURE CORONARY-ARTERY DISEASE

Significant risk factors for premature coronary heart disease include: (1) family history, (2) elevated low density lipoprotein (LDL) choles terol level greater than or equal to 160 mg/dl, 1, (3) decreased high density lipoprotein (HDL) cholesterol level < 35 mg/dl, 1, (4) cigaret te 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 dise ase include familial lipoprotein(a) excess, familial dyslipidemia (ele vated triglycerides and decreased HDL cholesterol), familial combined hyperlipidemia (elevations of LDL cholesterol and triglycerides, and o ften decreased HDL cholesterol), familial hypoapobetalipoproteinemia ( elevated apolipoprotein B levels), familial hypoalphalipoproteinemia ( low HDL cholesterol levels), and familial hypercholesterolemia (elevat ed LDL cholesterol levels). All these disorders have been characterize d using age and gender specific 90th and 10th percentile values from t he normal population. The diagnosis and potential management of these disorders is reviewed.