Family history of cardiovascular events and endothelial dysfunction in children with familial hypercholesterolemia.
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.
de Jongh
Saskia
S
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
Lilien
Marc R
MR
Bakker
Henk D
HD
Hutten
Barbara A
BA
Kastelein
John J P
JJ
Stroes
Erik S G
ES
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