Primary hyperlipoproteinemia in childhood and adolescence: identification and treatment of persons at risk for premature atherosclerosis.
Determination of serum cholesterol values in three populations of children and adolescents, totalling 4013 subjects aged 1 month to 20 years, revealed 16 cases of primary hyperbetalipoproteinemia (overall frequency, 1:251) and led to the detection of the disorder in 12 asymptomatic siblings. The upper limit of normal for serum cholesterol concentration was approximately 200 mg/dl at all ages studied. Dietary treatment was instituted in patients whose serum cholesterol value exceeded this limit and in whom a primary lipid defect was confirmed; the serum cholesterol value decreased in all patients who adhered to the diet. However, since the potential hazards and long-term results of dietary treatment, with or without drug therapy, in growing children are not known, such treatment should be reserved for affected children with a family history of premature atherosclerosis, and follow-up is essential.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D002791:Cholesterol, Dietary / Q000009:adverse effects; D002792:Cholestyramine Resin / Q000627:therapeutic use; D003327:Coronary Disease / Q000235:genetics*; D005247:Feeding Behavior; D005260:Female; D006330:Heart Defects, Congenital / Q000097:blood; D006801:Humans; D006949:Hyperlipidemias / Q000097:blood / Q000175:diagnosis* / Q000178:diet therapy* / Q000235:genetics*; D007223:Infant; D008077:Lipoproteins, LDL / Q000097:blood; D008297:Male; D010375:Pedigree; D011336:Probability; D012306:Risk
D002791:Cholesterol, Dietary; D008077:Lipoproteins, LDL; D002792:Cholestyramine Resin; D002784:Cholesterol