Atherogenic risk factors in cerebrotendinous xanthomatosis

• Published in: Clinica chimica acta Vol. 200; no. 1; pp. 1 - 11
• Main Authors: Fujiyama, Jiro, Kuriyama, Masaru, Arima, Sinichi, Shibata, Yoshisada, Nagata, Kazuya, Takenaga, Satoshi, Tanaka, Hiromitsu, Osame, Mitsuhiro
• Format: Journal Article
• Language: English
• Published: Shannon Elsevier B.V 15.08.1991; Elsevier
• Subjects: Adult; Aged; Apolipoproteins - blood; Atherogenic risk factor; Atherosclerosis; Atherosclerosis (general aspects, experimental research); Biological and medical sciences; Blood and lymphatic vessels; Brain Diseases - blood; Brain Diseases - complications; Cardiology. Vascular system; Cerebrotendinous xathomatosis; Cholesterol - blood; Cholesterol, HDL - blood; Chromatography, High Pressure Liquid; Coronary artery disease; Coronary Artery Disease - etiology; Female; Humans; Lipids - blood; Lipoprotein; Lipoproteins - blood; Lipoproteins, LDL - blood; Lipoproteins, VLDL - blood; Male; Medical sciences; Middle Aged; Risk Factors; Serum lipid; Xanthomatosis - blood; Xanthomatosis - complications; ch; CTX; ECG; LCAT; LTP; Lipoprotein; Coronary artery disease; F.ch; Serum lipid; HDL; Cerebrotendinous xathomatosis; TG; LDL; NEFA; VLDL; Atherosclerosis; T.ch; PL; Atherogenic risk factor; Human; Xanthomatosis; Mitochondria; Liver; Lipids; Microsome; Coronary heart disease; Cholesterol
• ISSN: 0009-8981; 1873-3492
• DOI: 10.1016/0009-8981(91)90328-A

In a study of coronary artery disease in patients with cerebrotendinous xanthomatosis (CTX), we documented the presence or absence of atherogenic risk factors and performed detailed analyses of serum lipid and lipoprotein profiles. Four of the seven patients examined had coronary arterial narrowing and/or obstruction, but multiple atherogenic risk factors were not found in any of these patients. Total cholesterol (T.ch) levels and low density lipoprotein-cholesterol (LDL-ch) levels were lower, and high density lipoprotein 2-cholesterol (HDL 2-ch) levels were higher in CTX patients than in controls. Triglyceride and very low density lipoprotein (VLDL) levels were significantly lower in the former. Indices correlating with the risk of atherosclerosis, such as the atherogenic index, and the ratios of apolipoprotein B/apolipoprotein AI, HDL 2-ch/LDL-ch, HDL 2-ch/HDL 3-ch, indicated that CTX serum was, in fact, ‘anti-atherogenic’. However, coronary artery disease is frequently seen in patients with CTX. This discrepancy suggests the existence of a unique mechanism by which atherosclerosis is induced in patients with CTX. We discuss a mechanism of disturbed lipoprotein metabolism which might be responsible for the deposition of sterols in the tissues of patients with CTX.