Inflammatory and thrombotic processes are associated with vascular dysfunction in children with familial hypercholesterolemia

• Published in: Atherosclerosis 2009-06, Vol.204 (2), p.532-537
• Main Authors: Charakida, Marietta, Tousoulis, Dimitris, Skoumas, Ioannis, Pitsavos, Christos, Vasiliadou, Carmen, Stefanadi, Elli, Antoniades, Charalambos, Latsios, George, Siasos, Gerasimos, Stefanadis, Christodoulos
• Format: Article
• Language: English
• Subjects: Adolescent; Age Factors; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Atherosclerosis (general aspects, experimental research); Biological and medical sciences; Biomarkers - blood; Blood and lymphatic vessels; C-Reactive Protein - analysis; Cardiology. Vascular system; Cardiovascular; Cardiovascular Diseases - blood; Cardiovascular Diseases - genetics; Cardiovascular Diseases - physiopathology; Case-Control Studies; Cholesterol - blood; Emergency and intensive care: renal failure. Dialysis management; Endothelium; Endothelium, Vascular - drug effects; Endothelium, Vascular - physiopathology; Female; Heterozygote; Humans; Hyperemia - physiopathology; Hyperlipoproteinemia Type II - blood; Hyperlipoproteinemia Type II - genetics; Hyperlipoproteinemia Type II - physiopathology; Inflammation; Inflammation - blood; Inflammation - genetics; Inflammation - physiopathology; Inflammation Mediators - blood; Intensive care medicine; Intercellular Adhesion Molecule-1 - blood; Interleukin-1beta - blood; Interleukin-6 - blood; Linear Models; Lipids; Lipoproteins - blood; Male; Medical sciences; Nitroglycerin - pharmacology; Plasminogen Activator Inhibitor 1 - blood; Risk Assessment; Risk Factors; Thrombosis; Thrombosis - blood; Thrombosis - genetics; Thrombosis - physiopathology; Vasodilation - drug effects; Vasodilator Agents - pharmacology
• ISSN: 0021-9150; 1879-1484
• DOI: 10.1016/j.atherosclerosis.2008.09.025

Abstract Background Evidence suggests that children with familial hypercholesterolemia (FH) have endothelial dysfunction. Inflammatory and haemostatic abnormalities are associated with advanced atherosclerosis and increased cardiovascular events. However, it is unknown whether these abnormalities present in FH children and contribute to their vascular dysfunction. Methods and results We studied 38 children with FH (19 males, 19 females aged 14.8 ± 0.9 years mean ± S.E.) and 41 healthy children (controls; 22 males, 19 females aged 15.4 ± 0.7 years). Endothelium-dependent reactive hyperemia (RH%) and endothelium-independent nitrate hyperemia dilatation (NH%) were measured by strain gauge plethysmography. Inflammatory and haemostatic parameters were assessed by ELISA. RH% and NH% were significantly reduced in FH compared to controls (91.3 ± 9.3% vs. 120.4 ± 10.6% and 53.6 ± 3.8% vs. 74.5 ± 7.4%, p < 0.05 for both). Total cholesterol and lipoprotein (a) were increased in FH children compared to controls (282.3 ± 8.8 mg/dl vs. 163.8 ± 4.6 mg/dl and 11.0[4.6, 30.7] mg/dl vs. 5.24[2.63, 11.0] mg/dl median [IQR] respectively; p < 0.001 for both). Intercellular cell adhesion molecule (ICAM-1) and interleukin 1β (IL-1β) serum levels were increased in FH compared to controls ( p < 0.05 and