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1/283. Angiographic and pathological studies on regression of coronary atherosclerosis of FH patients who received LDL-apheresis treatment.

    We report on the most recent data confirming the angiographic and pathological efficacy of LDL-apheresis for coronary atherosclerosis of a familial hypercholesterolemia (FH) patient using collected data of a large number of cases and especially of one autopsy case. Changes in coronary artery stenosis have been assessed angiographically in 37 FH patients in the LDL-Apheresis Regression Study (LARS) group of 13 institutions in japan. Definite regression was observed in 21 segments by visual and computer analysis. Fourteen of 37 patients (37.8%) who had received LDL-apheresis treatment in combination with cholesterol-lowering drugs had at least one regressed segment without any progressed segment. We pathologically examined at autopsy the coronary arteries in one FH patient who had received long-term LDL-apheresis therapy before death. The results revealed the process of scarring of atheromatous plaque, suggesting pathological regression correlated with the angiographic regression shown in serial angiograms taken during LDL-apheresis treatment. It was further suggested that the formation of an eccentric thickened wall lesion rich in collagen fiber prevented atheromatous plaque from tearing off. Such tearing off might lead to an acute coronary event. Aggressive cholesterol-lowering therapy based on LDL-apheresis can induce both angiographic and pathological regression in coronary atherosclerosis of FH patients. ( info)

2/283. Case study: analysis of an acute anterior-lateral myocardial infarction in a 16-year-old patient with familial hypercholesterolemia.

    This article presents a case study of a 16-year-old male patient with a significant family history for hypercholesterolemia and coronary artery disease, who suffered an anterior lateral myocardial infarction. On admission, his electrocardiograms revealed the classic pattern of an anterior lateral acute myocardial infarction plus a left anterior hemiblock. His cholesterol level was 750 mg/dL, and his low-density lipoprotein was 650 mg/dL. He underwent a cardiac catheterization that revealed an occluded left anterior descending artery requiring a percutaneous transluminal angioplasty and three coronary stents. The 12-lead electrocardiograms on admission and before discharge are analyzed. This article discusses the electrocardiogram characteristics of anterior lateral wall myocardial infarction coupled with a left anterior hemiblock. ( info)

3/283. Weekly versus biweekly lipid removal and effect of statins in severe hypercholesterolemia.

    Lipid removal using a continuous-flow extracorporeal system is of proven efficacy in severe hypercholesterolemia. Because of the inconveniences and expenses of extracorporeal removal of lipids, the effects of two treatment intervals (weekly versus biweekly) were assessed in two adolescents with circulating cholesterol higher than 20.0 mmol/L. In both patients, circulating levels were largely lower on a weekly lipid removal interval when compared with a biweekly interval. A rapid reaccumulation of cholesterol was noted after lipid removal. Treatment with simvastatin decreased the rapid reappearance of total cholesterol noted during the first 2 days after lipid removal but without any major effect on the subsequent reaccumulation of cholesterol. Aggressive treatment of severe hypercholesterolemia with statins and especially with extracorporeal lipid removal is now possible and of proven efficacy. The minimal practical lipid removal treatment interval should be used. ( info)

4/283. LDL-apheresis: clinical experience and indications in the treatment of severe hypercholesterolemia.

    LDL-cholesterol is the leading risk factor which influences the clinical outcome of patients with preexisting coronary heart disease. Clinical trials show that plasma LDL-cholesterol below 100 mg/dL decrease the rate of recurrent myocardial infarction and can induce regression in patients with coronary heart disease. However, in most cases of severe hypercholesterolemia with plasma LDL-cholesterol concentrations above 220 mg/dL LDL cannot be sufficiently decreased by maximal dietary and pharmacological therapy alone. Today this group of high risk CHD patients can be treated in addition with an extracorporeal procedure to eliminate LDL from the plasma circulation, the H.E.L.P.--LDL-apheresis. This method for selective removal of LDL, lipoprotein(a) and fibrinogen from plasma has been shown to be a clinically safe and very efficient method for the treatment of patients with homozygous familial hypercholesterolemia or CHD patients with severe hypercholesterolemia. Treatments with 1 week H.E.L.P. intervals revealed a mean reduction of minus 51% for LDL, of minus 45% for Lp(a) and of minus 46% for apo B, while HDL was increased by 12%. fibrinogen was decreased by minus 46%. Besides the marked reduction of LDL and fibrinogen plasma concentrations the H.E.L.P. treatment significantly improves hemorheological parameters and increases the oxygen tension in the tissue. We have also investigated the efficiency of a combined therapy, using HMG-CoA reductase inhibitors together with the H.E.L.P.--apheresis. Under this combined treatment, a reduction of the interval LDL-cholesterol levels of 70-80% has been achieved, while Lp(a) and fibrinogen were not further affected. We now report about our long-term clinical experience with the H.E.L.P. system in treating patients with different lipoprotein disorders: (1) Homozygous form of familial hypercholesterolemia; (2) CHD patients with familial and non-familial hypercholesterolemia; (3) CHD patients with very high concentrations of lipoprotein(a); and (4) Hypercholesterolemic patients after heart transplantation. Based on present experience guidelines for secondary prevention of coronary heart disease indications for the H.E.L.P.--LDL-apheresis treatment are discussed. ( info)

5/283. Treatment of homozygous familial hypercholesterolemia by plasma exchange and LDL-apheresis.

    Two girls with familial hypercholesterolemia were treated for 7 years by plasma exchanges (PE) or LDL-apheresis (LA). We compared different methods of treatment; PE with or without reuse of the plasma separator, LA of varying frequency, and LA with or without oral administration of simvastatin. We assessed the long-term results by measuring the blood levels of the biochemical parameters before sessions, and determined the effectiveness of each session by the percentage of decrease in the blood levels between the beginning and the end of the sessions. LA led to a more selective treatment (lowering of LDL cholesterol and maintenance of HDL cholesterol), but the blood levels of total cholesterol before sessions were the same as those obtained by PE. IgG and haemoglobin levels decreased little with LA. The rhythm of one session a week gave better results in LA. Although reuse of the plasma separator represents a financial saving it produced poorer results. The oral administration of simvastatin improved the results of LA. ( info)

6/283. LDL-apheresis in treatment of two patients with heterozygous familial hypercholesterolemia and extremely elevated lipoprotein (a) levels.

    In hyperlipidemia and, in particular, elevated lipoprotein (a) [Lp(a)] levels there appears to be pronounced linkage between the development and progression of atherosclerosis. Our study concerned two Caucasian male patients with heterozygous forms of familial hypercholesterolemia and extremely high Lp(a) concentrations. Maximal diet regimens and the use of lipid lowering drugs achieved a serum total-, LDL-cholesterol and triglyceride reduction of up to 30%, but no reduction of the Lp(a) level was discernible. Both patients suffered three myocardial infarctions and several coronary angiographies with percutaneous transluminal angioplasties (PTCA) were necessary. In 1989, we commenced treatment with LDL-apheresis. At present, after 78 LDL-aphereses in the case of the 41-year-old patient (48 months, dextran sulfate adsorption, KANEKA, japan) and 38 aphereses in the case of the 35-year old patient (8 months, immunoadsorption, special Lp[a] columns, LIPOPAK, POCARD, russia), the Lp(a) has dropped an average of 53%, total cholesterol 31%, LDL-cholesterol 40% and triglycerides 42%. During this period neither mycardial infarctions nor cardiac complaints were observed. In the course of treatment, both patients experienced an improvement in general well-being and increased performance. These results are very encouraging: LDL-apheresis may be effective in the treatment of patients, the only risk factor for premature atherosclerosis being an extremely high Lp(a) concentration. ( info)

7/283. Impact of different low-density lipoprotein (LDL) receptor mutations on the ability of LDL to support lymphocyte proliferation.

    Based on the demand for cholesterol for membrane formation, we determined the ability of low-density lipoprotein (LDL) to support proliferation in lymphocytes bearing different LDL receptor mutations, which were treated "in vitro" with lovastatin to inhibit endogenous cholesterol synthesis. Peripheral lymphocytes were isolated from two patients with homozygous familial hypercholesterolemia (FH), one homozygote for the mutation N804K (FH(Colmenar)) in exon 17, herein described for the first time, and a compound heterozygote carrying the mutations D280G and G528V, which determine a transport-defective biochemical phenotype. Flow cytometric analysis with 1,1'-dioctadecyl-3,3,3,3'-tetramethylindocarbocyanineperchlorate (Dil)-LDL showed normal LDL binding but defective internalization in lymphocytes from case 1, whereas in lymphocytes from case 2 both LDL binding and internalization were affected. Studies with mitogen-stimulated lymphocytes demonstrated that despite the different phenotype, the ability of LDL to support proliferation was impaired in both cases to a similar extent. These results indicate that internalization of the LDL particle is required for expression of the mitogenic effect of LDL. ( info)

8/283. Efficacy of statin therapy: possible effect of phenytoin.

    Statins are currently the most widely prescribed lipid-lowering drugs. Individual statins are known to be metabolised by the CYP3A4 isoform of the cytochrome P450 system. The effect of CYP3A4 inducers such as phenytoin on the metabolism and efficacy of these agents is unknown. We report a patient with familial hypercholesterolaemia and epilepsy in whom the introduction and subsequent discontinuation of phenytoin were associated with marked changes in the lipid response to treatment with simvastatin and atorvastatin. The serum activity of gamma-glutamyl transpeptidase may have acted as a marker of microsomal induction by phenytoin, since it rose markedly when phenytoin was introduced and returned to normal after it was discontinued. ( info)

9/283. Surgical excision of the tendon xanthoma in familial hypercholesterolemia--a case report.

    Familial hypercholesterolemia is an autosomal dominant disorder characterized by increased low-density lipoprotein cholesterol, premature atherosclerosis and tendon xanthomas. Genetic studies reveal familial hypercholesterolemia to be a dysfunction of LDL receptor gene on cell surface. Recently various mutations in the LDL receptor gene have been reported. When dna method is not available, the occurrence of tendon xanthomas, an isolated elevation of plasma cholesterol, with a normal concentration of plasma triglycerides virtually establishes the diagnosis of familial hypercholesterolemia. In this report, a 42-year-old male had tendon xanthoma at extensor surface of metacarpophalangeal joint of his right hand, olecranon of the left elbow and both knees, and Achilles tendons. The tendon xanthoma was excised for cosmetic reasons, and the wound healing was slower than average in this case. We suggest that before suture removal, wound healing must be complete. It is important that the hand surgeon recognize that tendon xanthoma is a physical sign of a potentially life-threatening disorder to the patient as well as his family, and that this disorder may respond favorably to early examination and management. ( info)

10/283. Integrin mediated adhesion of mononuclear cells from patients with familial hypercholesterolemia.

    BACKGROUND: Low-density lipoproteins (LDL) can induce the adhesion of monocytes to endothelial cells. monocytes of patients with familial hypercholesterolemia (FH) are exposed to high concentrations of LDL, and it has been reported that adhesiveness of these cells in hypercholesterolemic patients is enhanced. We investigated whether LFA-1 or VLA-4 mediated adhesion is altered in FH patients and whether HMG-CoA reductase inhibitors influence this adhesion. patients AND methods: LFA-1 and VLA-4 mediated adhesion to ICAM-1 and VCAM-1 coated beads was investigated using freshly isolated monocytes and t-lymphocytes from patients with homozygous FH, heterozygous FH (before and after cholesterol lowering treatment), and from controls. In addition, the expression of beta1- and beta2-integrins on these cells was determined. RESULTS: Both LFA-1 and VLA-4 mediated adhesion and integrin expression of monocytes and CD3 cells from patients with homozygous FH and heterozygous FH was similar to that of monocytes from a control population. Treatment with HMG-CoA reductase inhibitors did not affect the adherence to ICAM-1 or VCAM-1, and did not influence the expression of integrins. CONCLUSIONS: In contrast to studies by others, we demonstrated in the present study that the actual LFA-1 and VLA-4 mediated adhesion of t-lymphocytes and monocytes is not altered in patients with FH. ( info)
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