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Lipid apheresis

Meghan T Walsh, M Mahmood Hussain
Homozygous familial hypercholesterolemia (HoFH) is a polygenic disease arising from defects in the clearance of plasma low-density lipoprotein (LDL), which results in extremely elevated plasma LDL cholesterol (LDL-C) and increased risk of atherosclerosis, coronary heart disease, and premature death. Conventional lipid-lowering therapies, such as statins and ezetimibe, are ineffective at lowering plasma cholesterol to safe levels in these patients. Other therapeutic options, such as LDL apheresis and liver transplantation, are inconvenient, costly, and not readily available...
October 1, 2016: Critical Reviews in Clinical Laboratory Sciences
Claudia Stefanutti, Claudia Morozzi, Serafina Di Giacomo, Barbara Sovrano, Dario Mesce, Alberto Grossi
BACKGROUND: Homozygous familial hypercholesterolemia (HoFH) is a rare, genetically determined condition of highly elevated low-density lipoprotein cholesterol (LDLC) levels. If untreated, patients do not typically survive beyond the second decade of life. Traditional lipid-lowering therapies (statins and ezetimibe) are largely ineffective in HoFH patients, and extracorporeal lipoprotein apheresis (LA) forms the mainstay of treatment. Lomitapide is a microsomal triglyceride transfer protein inhibitor approved for the treatment of HoFH as an adjunct to LA...
July 2016: Journal of Clinical Lipidology
Altan Alim, Yaman Tokat, Yalcin Erdogan, Zafer Gokkaya, Murat Dayangac, Yildiray Yuzer, Arzu Oezcelik
FH is an autosomal dominant genetic disorder characterized by increased TC and LDL level, which leads to xanthomas, atherosclerosis, and cardiac complications even in childhood. The treatment options are diet, medical treatment, lipid apheresis, and LT. The aim of our study was to analyze our data of patients with FH. Between 2004 and 2015, there were 51 patients who underwent pediatric LT at our center. All patients with FH were identified, and the data were retrospectively analyzed. There were eight patients with homozygous FH in the median age of 10 years (IQR 6-12) who underwent LT...
July 20, 2016: Pediatric Transplantation
Ramesh Kumar, Mohammed Jaber Sedky, Sunny Joseph Varghese, Osama Ebrahim Sharawy
Transfusion related acute Lung injury (TRALI) though a serious blood transfusion reaction with a fatality rate of 5-25 % presents with acute respiratory distress with hypoxaemia and noncardiac pulmonary oedema within 6 h of transfusion. In non fatal cases, it may resolve within 72 h or earlier. Although reported with an incidence of 1:5000, its true occurrence is rather unknown. Pathogenesis is believed to be related to sequestration and adhesion of neutrophils to the pulmonary capillary endothelium and its activation leading to its destruction and leaks...
September 2016: Indian Journal of Hematology & Blood Transfusion
Claudia Stefanutti
The clinical indications and guidelines for low-density lipoprotein (LDL)-apheresis set by the 1(st) Italian Consensus Conference held in Ostuni in 1990 and completed in 1992, but never published, are reported schematically. In 1994, within the Project "Prevention and control of the factors of the disease (FATMA)" by the Italian National Research Council, subproject 8 "Control of cardiovascular disease", a "Hearing on therapeutic apheresis: need for a target-oriented project" was organised. The meeting was the last scientific initiative on LDL-apheresis supported by public funds in Italy...
July 7, 2016: Blood Transfusion, Trasfusione del Sangue
Knut Tore Lappegård, Terje Enebakk, Hilde Thunhaug, Anders Hovland
BACKGROUND AND AIMS: LDL apheresis is effective in reducing low-density lipoprotein (LDL) cholesterol (LDL-C) and clinical endpoints, however, the treatment is invasive and time consuming. In the present study, we explored lipid profiles and quality of life in patients with heterozygous familial hypercholesterolemia (FH) when altering the treatment regimen from weekly LDL apheresis to bi-weekly evolocumab treatment. METHODS: Three patients with FH and coronary artery disease, established in LDL apheresis for 135 ± 13(SD) months, participated...
August 2016: Atherosclerosis
Maya S Safarova, Iftikhar J Kullo
Familial hypercholesterolemia (FH), a relatively common Mendelian genetic disorder, is associated with a dramatically increased lifetime risk of premature atherosclerotic cardiovascular disease due to elevated plasma low-density lipoprotein cholesterol (LDL-C) levels. The diagnosis of FH is based on clinical presentation or genetic testing. Early identification of patients with FH is of great public health importance because preventive strategies can lower the absolute lifetime cardiovascular risk and screening can detect affected relatives...
June 2016: Mayo Clinic Proceedings
D Müller-Wieland, N Marx
Proprotein convertase subtilisin/kexin type 9 (PCSK9) binds to low-density lipoprotein (LDL) receptors leading to their degradation in the liver. Inhibition of PCSK9 leads to an increase in LDL receptors and as a result to a reduction of LDL cholesterol in blood. Currently, two antibodies against PCSK9 are available for clinical treatment in Germany, evolocumab (Repatha®) and alirocumab (Praluent®). Clinical studies have shown that treatment with these antibodies, which must be subcutaneously injected by patients every 2 or 4 weeks, in addition to an already existing lipid therapy can lower the LDL cholesterol level in blood by an average of 50-60 %...
June 2016: Herz
Oliver Weingärtner, Dieter Lütjohann, Torsten Plösch, Albrecht Elsässer
Hypercholesterolemia is a major risk factor for cardiovascular diseases. Serum cholesterol concentrations are regulated by enteral absorption, biliary secretion, and hepatic synthesis. Statins inhibit the rate-limiting enzyme of cholesterol synthesis, HMG-CoA-reductase, and reduce serum cholesterol concentrations as well as cardiovascular morbidity and mortality in general. Evidence from smaller studies indicates that patients with high baseline cholesterol absorption may show only a small response to statin treatment in terms of cholesterol lowering...
May 20, 2016: Journal of Steroid Biochemistry and Molecular Biology
Patrick M Moriarty, Klaus G Parhofer, Stephan P Babirak, Emil deGoma, P Barton Duell, Bernd Hohenstein, Wolfgang Ramlow, Vinaya Simha, Elisabeth Steinhagen-Thiessen, Paul D Thompson, Anja Vogt, Berndt von Stritzky, Yunling Du, Garen Manvelian
BACKGROUND: Many patients with heterozygous familial hypercholesterolemia (HeFH) fail to reach optimal low-density lipoprotein cholesterol (LDL-C) levels with available lipid-lowering medications, including statins, and require treatment using alternative methods such as lipoprotein apheresis. OBJECTIVE: To evaluate the efficacy of alirocumab 150 mg every 2 weeks (Q2W) compared with placebo in reducing the frequency of lipoprotein apheresis treatments in patients with HeFH...
May 2016: Journal of Clinical Lipidology
Calvin Yeang, Ming-Yow Hung, Young-Sup Byun, Paul Clopton, Xiaohong Yang, Joseph L Witztum, Sotirios Tsimikas
BACKGROUND: Oxidized phospholipids (OxPL) on apolipoprotein B-100 (OxPL-apoB) reflect the biological activity of lipoprotein(a) (Lp[a]) and predict cardiovascular disease events. However, studies with statins and low-fat diets show increases in OxPL-apoB and Lp(a). OBJECTIVE: This study evaluated changes in OxPL-apoB and Lp(a) with extended-release niacin (N), ezetimibe/simvastatin (E/S) and combination E/S/N. A systematic literature review of previously published trials, measuring both OxPL-apoB and Lp(a) after therapeutic interventions, was also performed...
May 2016: Journal of Clinical Lipidology
Genovefa D Kolovou, Vana Kolovou, Anna Papadopoulou, Gerald F Watts
Homozygous familial hypercholesterolemia (HoFH) is a rare genetic disorder, which leads to premature cardiovascular diseases. Microsomal triglyceride transport protein (MTP) inhibitors, such as lomitapide, offer a new therapeutic approach for treating these patients. We evaluated the lipid lowering (LL) efficacy of lomitapide according to several gene variants in MTP. Four clinically and/or molecularly defined HoFH patients were treated with lomitapide in addition to conventional high intensity LL therapy and regular lipoprotein apheresis...
July 1, 2016: Journal of Atherosclerosis and Thrombosis
C Schmöcker, U Kassner, S Kiesler, M Bismarck, M Rothe, E Steinhagen-Thiessen, K H Weylandt
Lipoprotein apheresis such as heparin-induced extracorporal LowDensityLipoprotein (LDL) Cholesterol precipitation (HELP) reduces apolipoprotein B-containing lipoproteins, most importantly low-density-lipoprotein (LDL), and lipoprotein (a) [Lp(a)]. It is used in patients with atherosclerotic disease and therapy-refractory hypercholesterolemia or progressive atherosclerotic disease in patients with elevated Lp(a). While lipid-lowering effects of lipoprotein apheresis are well-established, there are only sparse data regarding the effect of apheresis on individual omega-6 and omega-3 polyunsaturated fatty acids (n-6 PUFA and n-3 PUFA), such as arachidonic acid (AA), eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA), which could increase (AA) or decrease (EPA and DHA) cardiovascular risk...
June 2016: Atherosclerosis
Kevin D Ballard, Eunice Mah, Yi Guo, Richard S Bruno, Beth A Taylor, Jo Ellen Beam, Donna M Polk, Paul D Thompson
Objective. To investigate vascular endothelial function (VEF) responses to a single low-density lipoprotein (LDL) apheresis session in hypercholesterolemic patients undergoing chronic treatment. Methods. We measured brachial artery flow-mediated dilation (FMD), plasma lipids, vitamin E (α- and γ-tocopherol), markers of oxidative/nitrative stress (malondialdehyde (MDA) and nitro-γ-tocopherol (NGT)), and regulators of NO metabolism (arginine (ARG) and asymmetric dimethylarginine (ADMA)) prior to (Pre) and immediately following (Post) LDL apheresis and at 1, 3, 7, and 14 d Post in 5 hypercholesterolemic patients (52 ± 11 y)...
2016: International Journal of Vascular Medicine
Claudia Stefanutti, Fabio Mazza, Michael Steiner, Gerald F Watts, Joel De Nève, Daniela Pasqualetti, Juergen Paal
The effect of lipoprotein apheresis (Direct Adsorption of Lipids, DALI) (LA) on plasma levels of pentraxin 3 (PTX3), an inflammatory marker that reflects coronary plaque vulnerability, and expression of PTX3 mRNA was evaluated in patients with hyperLp(a)lipoproteinemia and angiographically defined atherosclerosis/coronary artery disease. Eleven patients, aged 55 ± 9.3 years (mean ± SD), were enrolled in the study. PTX3 soluble protein levels in plasma were unchanged by 2 sessions of LA; however, a downregulation of mRNA expression for PTX3 was observed, starting with the first session of LA (p < 0...
2016: Mediators of Inflammation
Joerg Klepper, Baerbel Leiendecker, Nicole Heussinger, Ekkehart Lausch, Friedrich Bosch
High-fat ketogenic diets are the only treatment available for Glut1 deficiency (Glut1D). Here, we describe an 8-year-old girl with classical Glut1D responsive to a 3:1 ketogenic diet and ethosuximide. After 3 years on the diet a gradual increase of blood lipids was followed by rapid, severe asymptomatic hypertriglyceridemia (1,910 mg/dL). Serum lipid apheresis was required to determine liver, renal, and pancreatic function. A combination of medium chain triglyceride-oil and a reduction of the ketogenic diet to 1:1 ratio normalized triglyceride levels within days but triggered severe myoclonic seizures requiring comedication with sultiam...
April 2016: Neuropediatrics
Patrick M Moriarty, Linda Hemphill
Patients with familial hypercholesterolemia (FH) have early development of atherosclerosis and cardiovascular disease (CVD). Lipid level-lowering medications are not always successful in reducing increased low-density lipoprotein C (LDL-C) levels. Lipoprotein apheresis (LA) therapy has proven its clinical benefit in reducing CVD events for patients with FH with hypercholesterolemia. LA reduces LDL-C levels by more than 60% in patients with FH and reduces CVD events. LA also reduces Lp(a) levels and CVD events...
March 2016: Endocrinology and Metabolism Clinics of North America
Hala Hussein, Samir Saheb, Martine Couturier, Marielle Atassi, Alexina Orsoni, Alain Carrié, Patrice Therond, Sandrine Chantepie, Paul Robillard, Eric Bruckert, M John Chapman, Anatol Kontush
BACKGROUND: Familial hypercholesterolemia (FH) features elevated oxidative stress and accelerated atherosclerosis driven by elevated levels of atherogenic lipoproteins relative to subnormal levels of atheroprotective high-density lipoprotein (HDL). Small, dense HDL3 potently protects low-density lipoprotein (LDL) against proinflammatory oxidative damage. OBJECTIVE: To determine whether antioxidative and/or anti-inflammatory activities of HDL are defective in FH and whether such defects are corrected by LDL apheresis...
January 2016: Journal of Clinical Lipidology
Harold E Bays, Peter H Jones, Carl E Orringer, W Virgil Brown, Terry A Jacobson
The National Lipid Association (NLA) Annual Summary of Clinical Lipidology is a yearly updated summary of principles important to the patient-centered evaluation, management, and care of patients with dyslipidemia. This summary is intended to be a "living document," with future annual updates based on emerging science, clinical considerations, and new NLA Position, Consensus, and Scientific Statements, thus providing an ongoing resource that applies the latest in medical science towards the clinical management of patients with dyslipidemia...
January 2016: Journal of Clinical Lipidology
Elisa Waldmann, Klaus G Parhofer
An elevated plasma concentration of lipoprotein(a) (Lp(a)) is an independent risk factor for cardiovascular disease. Life style modification and currently available drugs either fail to effectively lower plasma Lp(a) levels or do not result in clinical benefit. However lipoprotein apheresis is very efficient in decreasing Lp(a) concentrations. A single apheresis session can acutely decrease Lp(a) by approximately 60-75% and weekly or biweekly performed apheresis results in considerably decreased mean interval concentrations (approximately 25-40% reduction)...
February 17, 2016: Journal of Lipid Research
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