Abstract:
:Homozygous familial hypercholesterolemia (HFH) is a very rare autosomal dominant disease characterized by accelerated severe atherosclerosis. We examined 18 patients from 9 families with HFH. The age range was 6-30 years (mean = 16 years). Male to female ratio was equal. All patients had huge, multiple tuberous xanthomas on the skin and tendons. Mean +/- standard deviation of plasma cholesterol, triglycerides, low-density lipoproteins (LDL), and high-density lipoproteins (HDL) cholesterol levels were 608 +/- 89, 122 +/- 39, 550 +/- 88, and 26 +/- 8 mg/dl, respectively. Five patients (28%) had angina pectoris, two sustained a myocardial infarction, and one died at the age of 15 years. Two-dimensional echocardiography demonstrated supravalvular aortic stenosis in 3 of the 13 patients (23%). Coronary arteriography performed in 11 patients demonstrated significant obstruction in 6 patients, 2 each with single-, double-, and triple-vessel disease. Left main stenosis was present in 3 patients (27%). Supravalvular aortic narrowing was demonstrated in 6 patients (54%) and was associated with a gradient in 2 (25 and 35 mmHg, respectively). Segmental contraction abnormalities were detected in 2 of the 11 patients (18%). It is concluded that coronary artery disease is prevalent in patients with HFH and, based on the data presented, we recommend the performance of noninvasive technique, coronary arteriography and supravalvular aortography at an early age to detect and to follow the progression of the disease.
journal_name
Clin Cardioljournal_title
Clinical cardiologyauthors
Brook GJ,Keidar S,Boulos M,Grenadier E,Wiener A,Shehada N,Markiewicz W,Benderli A,Aviram Mdoi
10.1002/clc.4960120608subject
Has Abstractpub_date
1989-06-01 00:00:00pages
333-8issue
6eissn
0160-9289issn
1932-8737journal_volume
12pub_type
杂志文章abstract::Fourteen morbidly obese patients scheduled for gastric bypass surgery were diagnosed preoperatively as suffering from sleep apnea syndrome. There were 13 males and 1 female aged 24 to 59 years. Mean preoperative excessive body weight was 222 +/- 38%; mean apnea index prior to surgery was 84 +/- 44. A whole night lead ...
journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960081005
更新日期:1985-10-01 00:00:00
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journal_title:Clinical cardiology
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journal_title:Clinical cardiology
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更新日期:1994-04-01 00:00:00
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更新日期:2021-01-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960070111
更新日期:1984-01-01 00:00:00
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journal_title:Clinical cardiology
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更新日期:1987-04-01 00:00:00
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journal_title:Clinical cardiology
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更新日期:2000-08-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章,评审
doi:10.1002/clc.4960190307
更新日期:1996-03-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.22738
更新日期:2017-10-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章,评审
doi:10.1002/clc.4960110705
更新日期:1988-07-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960060605
更新日期:1983-06-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960210220
更新日期:1998-02-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/clc.22838
更新日期:2017-12-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.20340
更新日期:2008-10-01 00:00:00
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pub_type: 杂志文章
doi:10.1002/clc.20171
更新日期:2007-08-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960190605
更新日期:1996-06-01 00:00:00
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pub_type: 杂志文章
doi:10.1002/clc.4960290708
更新日期:2006-07-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960220706
更新日期:1999-07-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.20241
更新日期:2008-08-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.20462
更新日期:2009-11-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960200207
更新日期:1997-02-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960050707
更新日期:1982-07-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.20546
更新日期:2010-02-01 00:00:00
abstract:BACKGROUND:Separately, electrophysiologic study (EPS) and placement of a transvenous implantable cardioverter-defibrillator (ICD) can be performed safely in the majority of patients. The safety and potential cost savings of same-setting procedures have not been evaluated. HYPOTHESIS:Electrophysiologic study and placem...
journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960240905
更新日期:2001-09-01 00:00:00
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pub_type: 杂志文章
doi:10.1002/clc.22963
更新日期:2018-06-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章
doi:10.1002/clc.4960260412
更新日期:2003-04-01 00:00:00