Clinical features of type III hyperlipoproteinemia: analysis of 64 patients

Clin Investig. 1993 May;71(5):362-6. doi: 10.1007/BF00186624.

Abstract

The clinical and biochemical characteristics of type III hyperlipoproteinemia are described in 64 patients (35 males and 29 females). Homozygosity for apolipoprotein E2, the presence of an abnormally cholesterol-rich very low density lipoprotein fraction (beta-VLDL) and an elevated ratio of very low density lipoprotein cholesterol to plasma triglycerides (> 0.3; normal ratio about 0.2) were the basis for the diagnosis. Mean serum cholesterol and triglyceride concentrations at the first visit in the clinic were 426 +/- 221 and 719 +/- 996 mg/dl, respectively. The mean age at diagnosis of the disorder was 49 years in males and 53 years in females. There was a high prevalence of obesity (72%), xanthomas (42%), and atherosclerosis (39%), especially peripheral vascular disease (31%). Early and correct diagnosis of this familial lipoprotein disorder seems necessary because of the prompt and beneficial response to therapeutic interventions.

MeSH terms

  • Adult
  • Apolipoprotein E2
  • Apolipoproteins E / genetics
  • Arteriosclerosis / complications
  • Cholesterol / blood
  • Female
  • Homozygote
  • Humans
  • Hyperlipoproteinemia Type III / blood
  • Hyperlipoproteinemia Type III / complications
  • Hyperlipoproteinemia Type III / pathology*
  • Lipoproteins, VLDL / blood
  • Male
  • Middle Aged
  • Obesity / complications
  • Peripheral Vascular Diseases / complications
  • Triglycerides / blood
  • Xanthomatosis / complications

Substances

  • Apolipoprotein E2
  • Apolipoproteins E
  • Lipoproteins, VLDL
  • Triglycerides
  • Cholesterol