Heterozygous familial hypercholesterolaemia in childhood: Cardiovascular risk prevention

  • A. van der Graaf
  • J. J. P. Kastelein
  • A. Wiegman
REVIEW

DOI: 10.1007/s10545-009-1165-1

Cite this article as:
van der Graaf, A., Kastelein, J.J.P. & Wiegman, A. J Inherit Metab Dis (2009) 32: 699. doi:10.1007/s10545-009-1165-1

Summary

Children with familial hypercholesterolaemia (FH) have severely increased low-density lipoprotein cholesterol (LDL-C) levels that strongly predispose to premature cardiovascular disease (CVD) later in life. Early identification makes it possible to start lipid-lowering therapy at young age to prevent CVD. The atherosclerotic process can be inhibited by potent lipid-lowering therapy. The cornerstone of lipid-lowering therapy is a healthy lifestyle, but most of the time this is insufficient to reach adequate LDL-C goals. Subsequently, pharmacological therapy is initiated with increasing frequency. In the past decade numerous studies have assessed the efficacy and safety of statins in children with FH. Those studies demonstrate that statins are well tolerated, safe and effective. Therefore, these agents have a pivotal role in the treatment of children with FH.

Abbreviations

AAP

American Academy of Pediatrics

apoB

apolipoprotein B100

cIMT

carotid intima media thickness

CVD

cardiovascular disease

FDB

familial defective apolipoprotein B

FH

familial hypercholesterolaemia

FMD

flow-mediated dilatation

HDL-C

high-density lipoprotein cholesterol

LDL-C

low-density lipoprotein cholesterol

LDL-R

low-density lipoprotein receptor

PCSK9

proprotein convertase subtilisin/kexin 9

Copyright information

© SSIEM and Springer 2009

Authors and Affiliations

  • A. van der Graaf
    • 1
  • J. J. P. Kastelein
    • 1
  • A. Wiegman
    • 2
    • 3
  1. 1.Department of Vascular Medicine, Academic Medical CentreUniversity of AmsterdamAmsterdamThe Netherlands
  2. 2.Department of Paediatrics, Academic Medical CentreUniversity of AmsterdamAmsterdamThe Netherlands
  3. 3.Department of PaediatricsAcademic Medical CentreAmsterdamThe Netherlands