Summary of Major Lipid Disorders in Children and Adolescents

Primary Lipid DisordersLipid/Lipoprotein Abnormality
Familial hypercholesterolemia
    Homozygous↑↑ LDL cholesterol
    Heterozygous↑ LDL cholesterola
Familial defective apolipoprotein B↑ LDL cholesterol
Familial combined hyperlipidemiaa
    Type IIa↑ LDL cholesterol
    Type IV↑ VLDL cholesterol, ↑ triglycerides
    Type IIb↑ LDL cholesterol, ↑ VLDL cholesterol, ↑ triglycerides
    Types IIb and IV↓ HDL cholesterol (often)
Polygenic hypercholesterolemia↑ LDL cholesterol
Familial hypertriglyceridemia (200–1000 mg/dL)↑ VLDL cholesterol, ↑ triglycerides
Severe hypertriglyceridemia (≥1000 mg/dL)↑ chylomicrons, ↑ VLDL cholesterol, ↑↑ triglycerides
Familial hypoalphalipoproteinemia↓ HDL cholesterol
Dysbetalipoproteinemia (TC: 250–500 mg/dL; triglycerides: 250–600 mg/dL)↑ IDL cholesterol, ↑ chylomicron remnants
  • ↑ indicates increased; ↓, decreased; IDL indicates intermediate-density lipoprotein; VLDL, very low density lipoprotein.

  • a These are the 2 lipid and lipoprotein disorders seen most frequently in childhood and adolescence; familial combined hyperlipidemia most often manifests with obesity.