Plasma Exchange in the Management of Homozygous Familial Hypercholesterolæmia

Thompson, Gilbert R.; Lowenthal, RM; Myant, N. B.

doi:10.1016/s0140-6736(75)92193-5

Cited by 352 publications

(101 citation statements)

References 23 publications

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“…Similar effects were noted in patient 2 except that the posttreatment cholesterol levels never reached the pretreatment value throughout the study. This difference may be attributed to the additional beneficiary effect of hypolipidemic drugs in this patient as also previously reported by Thompson et al (24,25).…”

Section: Discussionsupporting

confidence: 83%

Removal of Cholesterol from Blood by Affinity Binding to Heparin-Agarose: Evaluation on Treatment in Homozygous Familial Hypercholesterolemia

et al. 1980

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SummaryExtracorporeal removal of cholesterol from blood with heparinagarose conjugates was studied as a means of reducing the plasma cholesterol concentration in two patients with homozygous familial. hypercholesterolemia. Both patients were treated in an outpatient clinic. Patient 1 underwent two separate courses of treatment;. during the first course of eight treatments, the plasma cholesterol concentration decreased by a maximum of 54% (from 811-370 mg/dl). The six treatments in the second course resulted in a 35% reduction of plasma cholesterol (from 939-632 mg/dl). In patient two, 12 consecutive treatments resulted in a 56% decrease in the concentration of plasma cholesterol (from 768-298 mg/dl). Repetitive treatments in both patients resulted in a new "steady state" with circulating cholesterol levels lower than the pretreatment baseline value. The decrease in plasma cholesterol is mainly due to the removal of low density lipoproteins. The circulating level of high density lipoproteins is unaffected by the treatment. The treatment has no effect on the chemical composition of both low and high density lipoproteins. The extracorporeal treatment of blood with heparin-agarose was well tolerated by both patients and there were no undesirable effects. The effectiveness and simplicity of the present approach makes it attractive as a possible mode of therapy for homozygous familial hypercholesterolemia.

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Section: Discussionsupporting

confidence: 83%

Removal of Cholesterol from Blood by Affinity Binding to Heparin-Agarose: Evaluation on Treatment in Homozygous Familial Hypercholesterolemia

et al. 1980

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“…Some studies have reported that LDL apheresis therapy was safe in children without influencing development/growth, and that this therapy led to the reduction/disappearance of skin xanthoma, inhibiting exacerbation of aortic valve stenosis/supravalvular stenosis, which are characteristic of homozygous FH, and coronary lesions, or resulting in improvement [73][74][75][76][77][78][79][80] . Irondeficiency anemia is the most frequent side effect.…”

Section: Effects Of Ldl Apheresismentioning

confidence: 99%

Guidelines for the Management of Familial Hypercholesterolemia

Harada‐Shiba

Arai

Oikawa

et al. 2012

JAT

167

164

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Familial hypercholesterolemia (FH) is a highly prevalent autosomal dominant hereditary disease, generally characterized by three major signs, hyper-low-density-lipoprotein (LDL) cholesterolemia, tendon/skin xanthomas and premature coronary artery disease (CAD). Because the risk of CAD is very high in these patients, they should be identified at an early stage of their lives and started on intensive treatment to control LDL-cholesterol. We here introduce a new guideline for the management of FH patients in Japan intending to achieve better control to prevent CAD. Diagnostic criteria for heterozygous FH are 2 or more of 1) LDL-cholesterol ≥ 180 mg/dL, 2) tendon/skin xanthoma(s), and 3) family history of FH or premature CAD within second degree relatives, for adults; and to have both 1) LDL-cholesterol ≥ 140 mg/dL and 2) family history of FH or premature CAD within second degree relatives, for children. For the treatment of adult heterozygous FH, intensive lipid control with statins and other drugs is necessary. Other risks of CAD, such as smoking, diabetes mellitus, hypertension etc., should also be controlled strictly. Atherosclerosis in coronary, carotid, or peripheral arteries, the aorta and aortic valve should be screened periodically. FH in children, pregnant women, and women who wish to bear a child should be referred to specialists. For homozygotes and severe heterozygotes resistant to drug therapies, LDL apheresis should be performed. The treatment cost of homozygous FH is authorized to be covered under the program of Research on Measures against Intractable Diseases by the Japanese Ministry of Health, Labour, and Welfare.

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“…In the young female patient groups, those with angina pectoris or myocardial ischemia showed significantly higher CSI than those without symptoms and signs (p<0.05) ( Figure 2D). Midpoint and standard deviation of CSI in five age groups (20)(21)(22)(23)(24)(25)(26)(27)(28)(29)30-39, 40-49, 50-59, and 60-70 years) were shown in Figure 3. As might be predicted from the regression equations in Figures 2D and 3 Figure 2D).…”

Section: Coronary Angiographic Studymentioning

confidence: 99%

Development of coronary heart disease in familial hypercholesterolemia.

et al. 1989

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From these data, we can assume that coronary artery stenosis detectable by angiography will occur after 17 and 25 years of age in male and female heterozygotes, respectively, and the treatment of heterozygotes with lipid-lowering drugs can be delayed until late adolescence. (Circulation 1989;79:225-332) F amilial hypercholesterolemia (FH) is a common autosomal dominant disorder caused by a mutation of the gene for the low-density lipoprotein (LDL) receptor.1 FH is frequently associated with premature coronary heart disease (CHD), and the rate of death from CHD among heterozygotes is several times higher than that among the general population.1-4 Homozygous patients do not usually survive to reach the age of 30,1,5 whereas the mean age at death for male and female heterozygotes is 54 and 65,4,6,7 respectively. There are no data, however, reporting when FH patients develop coronary heart disease documented by coronary angiography. The National Institutes of Health Consensus Development Conference on lowering cholesterol to prevent heart disease has recommended that strict dietary intervention is indicated for children with a blood cholesterol level above 200 mg/dl and that nonresponders should be considered for treatment with a lipid-lowering agent.

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Plasma Exchange in the Management of Homozygous Familial Hypercholesterolæmia

Cited by 352 publications

References 23 publications

Removal of Cholesterol from Blood by Affinity Binding to Heparin-Agarose: Evaluation on Treatment in Homozygous Familial Hypercholesterolemia

Removal of Cholesterol from Blood by Affinity Binding to Heparin-Agarose: Evaluation on Treatment in Homozygous Familial Hypercholesterolemia

Guidelines for the Management of Familial Hypercholesterolemia

Development of coronary heart disease in familial hypercholesterolemia.

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