Section 11. Inherited Disorders of Metabolism >
Part 8. Disorders of Lipid and Bile Acid Metabolism >
Chapter 166. Disorders of Lipid and Lipoprotein MetabolismPeter O. Kwiterovich Jr.
Topics Discussed: apolipoproteins; atherosclerosis; cardiovascular disease; cholesterol transport; chylomicrons; dyslipidemias; fat metabolism; genetics and dysmorphology; high density lipoproteins; lipid disorders; lipid transport; lipids; lipoprotein metabolism; lipoprotein metabolism disorders; lipoproteins; lipoproteins, vldl; low-density lipoproteins.
Sections: Screening for Dyslipidemia in Youth, Guidelines for Treating Dyslipidemia in Children and Adolescents, Metabolic Disorders of Dyslipidemia in Youth, Familial Metabolic Disorders of TG-Rich Lipoproteins, Elevated Levels of LP (A) Lipoprotein, Deficiencies in ApoB-Containing Lipoproteins, References.
Excerpt:"Disorders of lipid and lipoprotein metabolism are characterized
by dyslipidemia, which is defined as either elevated or low levels
of one or more of the major lipoprotein classes: chylomicrons, very-low-density
lipoproteins (VLDL), low-density lipoproteins (LDL), and high-density
lipoproteins (HDL). Dyslipidemia can result from the expression
of a mutation in a single gene that plays a paramount role in lipoprotein
metabolism. More often, dyslipidemia reflects the influence of multiple
genes. Environmental influences such as excessive dietary intake
of fat and calories and limited physical activity, particularly
when associated with overweight or obesity, can also contribute
significantly to dyslipidemia. This chapter presents a theoretical
and practical approach to the diagnosis and treatment of dyslipidemia
in infants, children, and adolescents. The major clinical complication
of dyslipidemia is a predilection to atherosclerosis starting early
in life and leading to cardiovascular disease (CVD) in adulthood.
At the extremes of dyslipidemia, where inherited disorders of lipid
and lipoprotein metabolism are more likely to occur, premature CVD
is more frequent and can be accompanied by deposition of lipid in
various tissues. Children with profound hypertriglyceridemia are
at high risk of pancreatitis.A number of clinical, epidemiological, metabolic, genetic, and
randomized clinical trials strongly support the tenet that the origins
of atherosclerosis and CVD risk factors begin in..."
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