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- 저자 : 이명묵(Myong Mook Lee) (검색결과 1 건)
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박영배(Yong Bae Park),한기훈(Ki Hoon Han),최영진(Young Jin Choi),최성주(Sung Joo Choi),손대원(Dae Won Sohn),오병희(Byung Hee Oh),이명묵(Myong Mook Lee),최윤식(Yun Shik Choi),서정돈(Jung Don Seo),이영우(Young Woo Lee) 대한내과학회 1994 대한내과학회지 Vol.47 No.1
N/A Background: Familial hypercholesterolemia (FH) is a frequent dyslipemic disorder (one in 500 in Caucasian) inherited by autosomal dominant trait and constitutes about 59 of all cases who have myocardial infarction. While this disorder is of clinical importance, little is reported about familial hypercholesterolemia in Korea. Method: We analysed the lipid profiles, Achilles tendon thickness, body mass index, lipoprotein levels and lipoprotein (a)(Lp(a)) in patients (N=26) with FH and their first degree relatives (N = 92) who visited the Seoul National University Hospital between January 1991 and August 1992. Clnical characteristics and coronary angio-graphic findings were also analysed in probands and their first degree relatives affected with familial hypercholesterolemia. 1) Twenty seven out of 92 first degree relatives were affected with familial hypercholesterolemia, The overall morbidity was 42.39(53 out of 118) and both sexes were equally affected (male: female=26:27), suggesting that familial hypercholesterolemia is autosomally inherited disorder. 2) The mean cholesterol level of the affected was abnormally very high (mean 345. 6 mg/dl) and was similar to that of Japanese studies on familial hypercholesterolemia. Lp (a), another risk factor of coronary artery disease, was higher (mean 24, 5 mg/dl) in the affected than in normal controls. 3) Achilles tendon thickness was significantly increased in all the cases with familial hypercholesterolemia (N =30, mean l3.7 mm, mean age 46.2 years: 23~75 years), compared with that of those who were not affected with familial hypercholesteolemia (N =20 mena 6. 9 mm, mean age 46 8 years), suggesting that thickening of Achilles tendon is a specific clinical finding of familial hypercholesterolemia (FH). 4) Patients with FH and coronary artery disease (CAD) were significantly older than FH patients without CAD (p<0,01). The coronary atherosclerosis score (CAS: Jenkins 1978) was positively correlate with age (R=0.91), which suggested that developement of CAD and the degree of coronary atherosclerosis was associated with the duration of exposure to, hypercholesterolemia. 5) The significant stenosis of coronary artery develped on proximal segments (14 out of 28 lesions N = 1) according to the findings of coronary arteriography, suggesting that CAD in FH can produee more fatal outcomes. Conclusion: Familial hypercholesterolemia is autosomal dominant inherited disorder of lipid mctabolism and is closely associated with coronary artery disease. The affected are almost totally asymptomatic until they experience angina or myocardial infarction, To prevent the fatal outcome due to coronary artery disease, more intense clinical attention sould be paid to detect familial hypercholesterolemia proband and its affected asymptomatic first degree relatives through pedigree study.
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