Familial hypercholesterolemia (FH) is a genetic disease characterized by high serum cholesterol levels and premature coronary atherosclerosis. Hyper cholesterolemia is one of the factors promoting the arteriosclerotic process and is a major cause of aortic aneurysm. Few data are available, however, about abdominal aortic aneurysms (AAAs) in patients with FH. In this study, the clini cal and angiographic characteristics of AAAs found in patients with FH were investigated. Thirty-one cases (23 men, 8 women, aged fifty ± fourteen years) were examined by coronary angiography, thoracic and abdominal aortography, and clinical data. Abdominal aortography detected abdominal aneurysms in 8 cases (26%), all of whom were men, including 4 cases (50%) that were compli cated by diabetes mellitus. The abdominal aneurysm patients manifested severe coronary atherosclerosis, severe abdominal aortic irregularity, and higher blood pressure than the nonaneurysm FH patients. These findings suggest that AAAs are an important and prevalent feature in FH, especially in men with diabetes mellitus and high blood pressure.
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References
1.
Goldstein JL, Brown MS: Familial hypercholesterolemia. In: The Metabolic Basis of Inherited Disease, ed. 5, ed. by Stanbury JB, Wyngaaden JB, Fredrickson DS, et al. New York: McGraw-Hill , 1983, pp 672-684
2.
Brown MS, Goldstein JL: Familial hypercholesterolemia ; genetic, biochemical, and pathophysiological consideration. Adv Intern Med20:273-282, 1975.
3.
Ross R.: The pathogenesis of atherosclerosis: An update. N Engl J Med314:488-500, 1986.
4.
Mabuchi H., Koizumi J., Shimizu M., et al: Development of coronary heart disease in familial hypercholesterolemia . Circulation79:225-232, 1989.
5.
Jensen J., Levy RI, Kornerup V.: Coronary disease in familial hypercholesterolemia. Circulation36:77-89, 1967.
6.
Stone NJ, Levy RI, Fredrickson DS, et al: Coronary artery disease in 116 kindred with familial type II hyperlipoproteinemia. Circulation49:476-488, 1974.
7.
Mabuchi H., Miyamoto S., Ueda K., et al: Causes of death in patients with familial hypercholesterolemia . Atherosclerosis61:1-6, 1986.
8.
Mabuchi H., Ito S., Haba T., et al: Discrimination of familial hypercholesterolemia and secondary hypercholesterolemia by Achilles' tendon thickness. Atherosclerosis28:61-67, 1977.
9.
Mabuchi H., Kamon N., Fujita H., et al: Effects of CS-514 on serum lipoprotein lipid and apolipoprotein levels in patients with familial hypercholesterolemia. Metabolism36:475-479, 1987.
10.
World Health Organization (WHO)Expert Committee on Diabetes Mellitus: Second Report. Technical Report Series 646, Geneva: WHO, 1980.
11.
Austen WG, Edwards JE, et al: AHA Committee Report: A reporting system on patients evaluated for Grading of Coronary Artery Disease, Council on Cardiovascular Surgery, American Heart Association. Circulation51:5-12, 1975.
12.
Tatami R., Mabuchi H., Ueda K., et al: Intermediate-density lipoprotein and cholesterol-rich very low density lipoprotein in angiographically determined coronary artery disease . Circulation64:1174-1184, 1981 .
13.
Gore I., Hirst AE: Arteriosclerotic aneurysms of the abdominal aorta: A review. Prog Cardiovasc Dis16:1174-1184, 1981 .
Evans PH: Relationship of longstanding blood pressure levels to atherosclerosis . Lancet1:516-519, 1965.
16.
Giertsen JC : Atherosclerosis in an autopsy series. VII. Relation of hypertension to atherosclerosis. Acta Path Microbiol Scand66:331-340, 1966.
17.
Rao Bpc, Wagner WD, Hitchins PM, et al: Spontaneous and diet-aggravated atherosclerosis in White Carneau pigeons genetically selected for discordant blood pressure. Atherosclerosis65:29-35, 1987.
18.
Schwartz CJ , Stenhouse NS, Taylor AE, et al: Coronary disease severity at necropsy . Br Heart J27:731-739, 1965.
19.
Cabin HS, Roberts WC: Relation of serum total cholesterol and triglyceride levels to the amount and extent of coronary arterial narrowing by atherosclerotic plaque in coronary heart disease. Am J Med73:227-234, 1982.
20.
Kannel WB, McGee DL: Diabetes and cardiovascular risk factors: The Framingham study. Circulation59:8-16, 1979.
21.
Stout RW: Blood glucose and atherosclerosis. Arteriosclerosis1:227-234, 1981.
22.
Gottfrie M. , Yao J., Glagov S.: Vasa vasorum, mural tension and the ultrastructural organization of human atherosclerotic arteries. Fed Proc37:933-950, 1978.
23.
Hertzer NR, Beven EG, Young J., et al: Coronary artery disease in peripheral vascular patients. Ann Surg199:223-233, 1984.
24.
Genda A., Nakayama A., Shimizu M., et al: Coronary angiographic characteristics in Japanese patients with heterozygous familial hypercholesterolemia. Atherosclerosis66:29-36, 1987.
