Lp(a) in Childhood
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PEDIATRICS (S. GIDDING, SECTION EDITOR)
Lp(a) in Childhood Christopher J. Prendergast 1 & Jennifer C. Kelley 1 & Edward F. Linton 2 & MacRae F. Linton 3
Published online: 8 August 2017 # Springer Science+Business Media, LLC 2017
Abstract Elevated plasma concentration of lipoprotein (a) (Lp(a)) is an independent risk factor for the development of atherosclerotic coronary vascular disease. There is a causal relationship between Lp(a) elevation and myocardial infarction. In the pediatric population, Lp(a) has been associated with risk for ischemic stroke. However, a consensus regarding the clinical utility of Lp(a) measurement in children has not been established. In this article, we review recent literature regarding Lp(a) metabolism, its role in disease states such as in pediatric thrombosis and familial hypercholesterolemia (FH), and therapy directed at Lp(a) levels. Our findings show that Lp(a) remains a controversial but emerging risk factor for cardiovascular disease, especially in children. However, new and important research continues to contribute to our understanding of Lp(a) metabolism in children and to cardiovascular risk in diseases such as FH. What is clear is that Lp(a) has the potential to play a role in the management of cardiovascular risk in children and adults. Keywords Lipoprotein(a) . Cardiovascular risk . Thrombosis . Familial hypercholesterolemia This article is part of the Topical Collection on Pediatrics * MacRae F. Linton [email protected] 1
Departments of Pediatrics, Atherosclerosis Research Unit, Cardiovascular Medicine, Vanderbilt University Medical Center, Nashville, TN 37232-6300, USA
2
Perelman School of Medicine, Jordan Medical Education Center, University of Pennsylvania, 6th floor, 3400 Civic Center Blvd, Philadelphia, PA 19104-6055, USA
3
Department of Medicine, Department of Pharmacology, Vanderbilt University Medical Center, Vanderbilt University School of Medicine, 2220 Pierce Avenue, Nashville, TN 37232-6300, USA
Introduction: Background and Structure Lipoprotein (a) (Lp(a)) consists of an low-density lipoprotein (LDL) particle connected to apoprotein (a) (apo(a)), a glycoprotein with repeating Kringle units that have homology to plasminogen [1••]. Apo(a) binds covalently to LDL through a disulfide bond with apoB-100, the main structural apoprotein in LDL [2••]. ApoB-100 is required for the synthesis of very low density lipoprotein (VLDL) particles by the liver, and LDL is produced in the plasma through the hydrolysis of triglycerides in VLDL by lipoprotein lipase and hepatic lipase [3]. Although the main precursors of Lp(a), VLDL and apo(a), are synthesized in the liver, Lp(a) particles are thought to be formed in the bloodstream or on the hepatocyte surface. Apo(a) has homology to plasminogen and is composed of variable numbers of repeating units known as Kringles, named due to their resemblance to the Danish pastry, consisting of three loops stabilized by three disulfide bonds [1••]. The number of repeating Kringle units is genetically determined, and th
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