Abstract
Familial hypercholesterolemia (FH) is a dominantly inherited defect of low density lipoprotein (LDL) catabolism due to a defect in the LDL receptor activity. The disorder affects approximately one in 200 to one in 500 persons in North America (1). Thus, between 500,000 and 1,000,000 Americans are likely to be affected. The great majority of affected subjects are heterozygotes in whom the disorder is characterized by elevated LDL cholesterol, frequent appearance of xanthomata in early adult life, and the premature onset of coronary heart disease. In some studies, the risks of developing clinically evident coronary artery disease (CAD) in men with FH were 5% by age 30, while 80% of men were symptomatic by age 60 (2). The risks for CAD in females with the disorder is less but still very much higher than in the non-affected population.
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Stein, E.A., Kirkeeide, R.L., Gould, L., Nishikawa, A., Lamkin, G. (1990). The Repetitive Plasma Exchange/Drug Trial in Familial Hypercholesterolemia: Very Early Results from Two-year Coronary Angiograms. In: Glagov, S., Newman, W.P., Schaffer, S.A. (eds) Pathobiology of the Human Atherosclerotic Plaque. Springer, New York, NY. https://doi.org/10.1007/978-1-4612-3326-8_37
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DOI: https://doi.org/10.1007/978-1-4612-3326-8_37
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