Treatment of refractory familial hypercholesterolemia by low-density lipoprotein apheresis using an automated dextran sulfate cellulose adsorption system

Gordon, Bruce R.; Kelsey, Sheryl F.; Bilheimer, David W.; Brown, David C.; Dau, Peter C.; Gotto, Antonio M.; Illingworth, D. Roger; Jones, Peter H.; Leitman, Susan F.; Prihoda, James S.; Stein, Evan A.; Stern, Thomas N.; Zavoral, James H.; Zwiener, Robert J.

doi:10.1016/0002-9149(92)90352-y

Cited by 103 publications

(62 citation statements)

References 34 publications

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“…Therefore, the mean cholesterol value was b + 3.78* (a-b)/x, where a is the postapheresis cholesterol level, b is the preapheresis cholesterol level of subsequent procedures, and x is the treatment interval (days). [17][18][19] Coronary angiography: Coronary angiography (CAG) was performed before and after the combination therapy with intracoronary administration of 5 mg isosorbide dinitrate immediately before the injection of contrast medium. The degree of stenosis in 15 segments of the coronary artery for each patient was analyzed by 2 experienced cardiologists at different dates blinded to the clinical history of the patient.…”

Section: Methodsmentioning

confidence: 99%

Ten-year Follow-up of Familial Hypercholesterolemia Patients After Intensive Cholesterol-lowering Therapy

Masaki

Tatami²,

Kumamoto³

et al. 2005

Int. Heart J.

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SUMMARYTo prevent coronary artery disease, it is necessary for patients with familial hypercholesterolemia (FH) to maintain a low cholesterol level. Recently a combination therapy of low-density lipoprotein (LDL) apheresis and statins has been used for FH patients, but their long-term prognosis over 10 years is unknown.In this single center prospective report, 18 FH patients with severe coronary stenosis received LDL apheresis every 2 or 4 weeks and statin therapy for 9.8 ± 3.0 years. Probucol was given to 17 of the 18 patients. We observed their clinical events as well as coronary stenosis findings and ejection fractions for 10.7 ± 2.6 years.Total and LDL cholesterol levels before therapy were 345 ± 46 and 277 ± 48 mg/ dL, respectively. Immediately following LDL-apheresis, these levels decreased to 104 ± 7.5 and 66 ± 16 mg/dL, respectively. There were no cardiac deaths and 4 patients were free from any coronary events. There was one noncardiac death. Nonfatal myocardial infarction occurred in 2 patients and coronary bypass surgery was required in one patient. Twelve patients received additional coronary angioplasty. There was little change in coronary stenosis and ejection fraction following 10 years of the combination therapy. Univariate Cox regression analysis revealed that the calculated mean LDL cholesterol level was the predictive value of treatment efficacy (mean LDL cholesterol < 140 mg/dL, hazard ratio 0.23, P = 0.028). The combination therapy of LDL-apheresis and antilipid drugs delayed the progression of coronary atherosclerosis and prevented a major cardiac event, although complete inhibition was limited to a small group. Additional coronary angioplasty is likely to be required for a favorable clinical outcome in FH patients. (Int Heart J 2005; 46: 833-843)

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Section: Methodsmentioning

confidence: 99%

Ten-year Follow-up of Familial Hypercholesterolemia Patients After Intensive Cholesterol-lowering Therapy

Masaki

Tatami²,

Kumamoto³

et al. 2005

Int. Heart J.

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“…Portacaval shunt (decreases hepatic cholesterol biosynthesis) or orthotopic liver transplantation (donation of LDL receptors to the patients) are in the experimental stage. 3 The late development of coronary arterial disease in the present case may be due to the protective effect of female hormones in spite of altered lipid metabolism. The sudden death of four siblings in early childhood was assumed to be due to stenotic coronary or aortic valvular lesion.…”

Section: Figure 2: Nodular Swellings Of Pip and Mcp Jointsmentioning

confidence: 66%

Answer to What's Your Diagnosis?

Das

2002

Ann Saudi Med

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“…New tech niques have been developed to replace plasmaphere sis, which selectively remove apolipoprotein-B-► containing lipoproteins from plasma [65][66][67][68][69]. The performance of regular LDL-apheresis permits the achievement of lower levels of LDL-cholesterol, which are usually not possible to attain with drug therapy alone [70][71][72][73]. Adverse events from LDLapheresis are infrequent and similar to any extracorporeal treatment.…”

Section: Ldl-apheresismentioning

confidence: 99%

LDL-cholesterol lowering and atherosclerosis - clinical benefit and possible mechanisms: an update

Stalenhoef

1997

The Netherlands Journal of Medicine

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The results of several lipid-lowering randomized trials have been released during the past years and have confirmed the lipid hypothesis. Reduction of cholesterol by potent drugs in clinically symptomatic or asymptomatic patients with above-average cholesterol levels will substantially reduce the risk of coronary events. The present article gives a review of potent low-density lipoprotein cholesterol-lowering treatments and discusses developments in hypolipidaemic therapy in relation to recent primary and secondary prevention studies. In addition, possible mechanisms of cholesterol lowering in retardation of the atherosclerotic process are summarised. © 1997 Elsevier Science B.V.

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Treatment of refractory familial hypercholesterolemia by low-density lipoprotein apheresis using an automated dextran sulfate cellulose adsorption system

Cited by 103 publications

References 34 publications

Ten-year Follow-up of Familial Hypercholesterolemia Patients After Intensive Cholesterol-lowering Therapy

Ten-year Follow-up of Familial Hypercholesterolemia Patients After Intensive Cholesterol-lowering Therapy

Answer to What's Your Diagnosis?

LDL-cholesterol lowering and atherosclerosis - clinical benefit and possible mechanisms: an update

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