title: The biochemical, clinical, and genetic features of type III hyperlipoproteinemia.
abstract: The clinical and biochemical features of type III hyperlipoproteinemia are described in 49 patients from 23 to 70 years of age. An increase in very low-density lipoproteins (VLDL) of abnormal chemical composition was the basis for diagnosis. The untreated patients all had hypercholesterolemia and hyperglyceridemia, and, on the average, decreased concentrations of both low- and high-density lipoproteins. Seventy-four percent had xanthomas, and classic "xanthoma striata palmaris" was found in more than half. Twenty-seven percent had ischemic heart disease, detected earlier in men than in women. Twenty-seven percent had peripheral vascular disease (compared to 4% of subjects with type II hyperlipoproteinemia). Twenty-five of 35 subjects achieved normal lipid levels with dietary therapy alone. Analysis of 29 kindred showed hyperlipidemia in half of adult blood relatives; half of these had type III, the remainder usually had sample endogenous hyperglyceridemia (type IV). Only 2 of 55 children less than 20 years of age were affected, both with type IV.
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D002784:Cholesterol / Q000097:blood; D003327:Coronary Disease / Q000453:epidemiology / Q000209:etiology; D005260:Female; D005951:Glucose Tolerance Test; D006801:Humans; D006937:Hypercholesterolemia / Q000150:complications; D006949:Hyperlipidemias / Q000097:blood / Q000145:classification / Q000175:diagnosis / Q000235:genetics; D008074:Lipoproteins / Q000097:blood*; D008075:Lipoproteins, HDL / Q000097:blood; D008077:Lipoproteins, LDL / Q000097:blood; D008079:Lipoproteins, VLDL / Q000097:blood; D008297:Male; D008875:Middle Aged; D014280:Triglycerides / Q000097:blood; D014527:Uric Acid / Q000097:blood; D014652:Vascular Diseases / Q000453:epidemiology / Q000209:etiology; D014973:Xanthomatosis / Q000209:etiology
chemical_list: D008074:Lipoproteins; D008075:Lipoproteins, HDL; D008077:Lipoproteins, LDL; D008079:Lipoproteins, VLDL; D014280:Triglycerides; D014527:Uric Acid; D002784:Cholesterol