title: Family history of cardiovascular events and endothelial dysfunction in children with familial hypercholesterolemia.
abstract: OBJECTIVE
  in patients with familial hypercholesterolemia (FH), the propensity towards atherosclerosis may vary considerably. In the general population, a positive family history is associated with an increased risk for cardiovascular events. Since endothelial dysfunction is predictive for future cardiovascular events, we evaluated whether FH-children with a positive family history of premature cardiovascular disease have more pronounced endothelial dysfunction compared to children with a negative family history.
  
  
  METHODS
  50 FH children, 10-18 years, participated in this study. Thirty-one children had a positive family history for cardiovascular events (fh(+)) and 19 children had no events in the family (fh(-)). Nineteen matched siblings participated as controls. Endothelial function was assessed by testing the flow mediated dilatation (FMD) of the brachial artery.
  
  
  RESULTS
  baseline characteristics were comparable for fh(+), fh(-) and controls. Lipid levels were significantly higher in FH children. In FH, FMD was impaired compared to controls (11.7+/-4.4 vs. 15.6+/-6.8%, P<0.03). In addition, FMD was significantly lower in fh(+) compared to fh(-) (10.7+/-9.9 vs. 13.3+/-4.6%, P<0.05).
  
  
  CONCLUSIONS
  In FH-children, endothelial function is impaired compared to matched controls. This impairment is most pronounced in FH children with a positive family history of premature cardiovascular disease.
authors.affiliation: Department of Vascular Medicine, Academic Medical Center, University of Amsterdam, G1-146, Meibergdreef 9, P.O. Box 22660, 1100 DD, Amsterdam, The Netherlands. s.dejongh@amc.uva.nl
authors.forename: Saskia
authors.initials: S
authors.lastname: de Jongh
authors.forename: Marc R
authors.initials: MR
authors.lastname: Lilien
authors.forename: Henk D
authors.initials: HD
authors.lastname: Bakker
authors.forename: Barbara A
authors.initials: BA
authors.lastname: Hutten
authors.forename: John J P
authors.initials: JJ
authors.lastname: Kastelein
authors.forename: Erik S G
authors.initials: ES
authors.lastname: Stroes
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D001783:Blood Flow Velocity; D002318:Cardiovascular Diseases / Q000453:epidemiology* / Q000235:genetics*; D016022:Case-Control Studies; D002648:Child; D004730:Endothelium, Vascular / Q000503:physiopathology*; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000453:epidemiology* / Q000235:genetics*; D015994:Incidence; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D010375:Pedigree; D011336:Probability; D012016:Reference Values; D018570:Risk Assessment; D017678:Sex Distribution; D018709:Statistics, Nonparametric