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Lipid apheresis

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https://www.readbyqxmd.com/read/28329241/lipoprotein-a-the-revenant
#1
Baris Gencer, Florian Kronenberg, Erik S Stroes, François Mach
In the mid-1990s, the days of lipoprotein(a) [Lp(a)] were numbered and many people would not have placed a bet on this lipid particle making it to the next century. However, genetic studies brought Lp(a) back to the front-stage after a Mendelian randomization approach used for the first time provided strong support for a causal role of high Lp(a) concentrations in cardiovascular disease and later also for aortic valve stenosis. This encouraged the use of therapeutic interventions to lower Lp(a) as well numerous drug developments, although these approaches mainly targeted LDL cholesterol, while the Lp(a)-lowering effect was only a 'side-effect'...
February 17, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28279833/effect-of-mipomersen-on-ldl-cholesterol-in-patients-with-severe-ldl-hypercholesterolaemia-and-atherosclerosis-treated-by-lipoprotein-apheresis-the-mica-study
#2
Elisa Waldmann, Anja Vogt, Alexander Crispin, Julia Altenhofer, Ina Riks, Klaus G Parhofer
BACKGROUND AND AIMS: In this study, we evaluated the effect of mipomersen in patients with severe LDL-hypercholesterolaemia and atherosclerosis, treated by lipid lowering drugs and regular lipoprotein apheresis. METHODS: This prospective, randomized, controlled phase II single center trial enrolled 15 patients (9 males, 6 females; 59 ± 9 y, BMI 27 ± 4 kg/m(2)) with established atherosclerosis, LDL-cholesterol ≥130 mg/dL (3.4 mmol/L) despite maximal possible drug therapy, and fulfilling German criteria for regular lipoprotein apheresis...
February 24, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28255870/efficacy-and-safety-of-lomitapide-in-hypercholesterolemia
#3
REVIEW
Xin Liu, Peng Men, Yuhui Wang, Suodi Zhai, Zhigang Zhao, George Liu
BACKGROUND: Despite extensive use of statins, patients with hypercholesterolemia, especially homozygous familial hypercholesterolemia (HoFH), do not achieve recommended targets of low-density lipoprotein cholesterol (LDL-C). There is an urgent need for novel options that could reduce proatherogenic lipoprotein cholesterol levels. Lomitapide, a microsomal triglyceride transport protein (MTP) inhibitor, was approved three years ago as an orphan drug for the treatment of patients with HoFH...
March 2, 2017: American Journal of Cardiovascular Drugs: Drugs, Devices, and Other Interventions
https://www.readbyqxmd.com/read/28215937/long-term-treatment-with-evolocumab-added-to-conventional-drug-therapy-with-or-without-apheresis-in-patients-with-homozygous-familial-hypercholesterolaemia-an-interim-subset-analysis-of-the-open-label-taussig-study
#4
Frederick J Raal, G Kees Hovingh, Dirk Blom, Raul D Santos, Mariko Harada-Shiba, Eric Bruckert, Patrick Couture, Handrean Soran, Gerald F Watts, Christopher Kurtz, Narimon Honarpour, Lihua Tang, Sree Kasichayanula, Scott M Wasserman, Evan A Stein
BACKGROUND: Homozygous familial hypercholesterolaemia is a genetic disorder characterised by substantially raised LDL cholesterol, reduced LDL receptor function, xanthomas, and cardiovascular disease before age 20 years. Conventional therapy is with statins, ezetimibe, and apheresis. We aimed to assess the long-term safety and efficacy of the proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor evolocumab in a subset of patients with homozygous familial hypercholesterolaemia enrolled in an open-label, non-randomised phase 3 trial...
February 16, 2017: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/28185213/hyperlipoproteinaemia-a-apheresis-and-emerging-therapies
#5
REVIEW
Anja Vogt
A high level of lipoprotein(a) (Lp(a)) is recognized as an independent and additional cardiovascular risk factor contributing to the risk of early onset and progressive course of cardiovascular disease (CVD). All lipid lowering medications in use mainly lower low density lipoprotein-cholesterol (LDL-c) with no or limited effect on levels of Lp(a). Niacin, the only component lowering Lp(a), is firstly often poorly tolerated and secondly not available anymore in many countries. A level of <50 mg/dl was recommended recently as the cut off level for clinical use and decision making...
March 2017: Clinical Research in Cardiology Supplements
https://www.readbyqxmd.com/read/28154305/efficacy-and-safety-of-lomitapide-in-japanese-patients-with-homozygous-familial-hypercholesterolemia
#6
Mariko Harada-Shiba, Katsunori Ikewaki, Atsushi Nohara, Yoshihiko Otsubo, Koji Yanagi, Masayuki Yoshida, Qing Chang, Pamela Foulds
AIM: There is an unmet need in Japan for more optimal lipid-lowering therapy (LLT) for patients with homozygous familial hypercholesterolemia (HoFH) who respond inadequately to available drug therapies and/or apheresis, to achieve goals of low-density lipoprotein cholesterol (LDL-C) reduction by 50% or to <100 mg/dL. METHODS: In this study, Japanese patients with HoFH on stable LLT and diet were treated with lomitapide, initiated at 5 mg/day and escalated to maximum tolerated dose (up to 60 mg/day) over 14 weeks...
February 2, 2017: Journal of Atherosclerosis and Thrombosis
https://www.readbyqxmd.com/read/28128058/apolipoprotein-a-antisense-oligonucleotides-a-new-treatment-option-for-lowering-elevated-lipoprotein-a
#7
Julia Schreml, Ioanna Gouni-Berthold
BACKGROUND: Lipoprotein(a) [Lp(a)] is a particle similar to LDL that contains an additional protein called apolipoprotein(a) [apo(a)]. Recent epidemiologic and Mendelian randomization studies have provided evidence that Lp(a) may be causally related to the pathogenesis of atherosclerosis and cardiovascular disease (CVD). While the risk association between Lp(a) concentrations and CVD is weak it seems to be continuous in shape and without an obvious threshold for Lp(a) levels. METHODS: Circulating concentrations of Lp(a) are genetically determined and desirable levels are &amp;amp;amp;amp;amp;amp;lt; 50 mg/dl...
January 25, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28078998/lipoprotein-a-management-pharmacological-and-apheretic-treatment
#8
Ruth Hanssen, Ioanna Gouni-Berthold
Lipoprotein (a) [Lp(a)] is an low-density lipoprotein (LDL)-like particle with an additional apolipoprotein, apolipoprotein (a), [apo(a)] attached to apolipoprotein B. Recent epidemiologic and Mendelian randomization studies have provided evidence that Lp(a) is causally related to the pathogenesis of atherosclerosis and cardiovascular disease (CVD). The risk association between Lp(a) concentrations and CVD is still controversial but seems to be continuous and without an obvious threshold Lp(a) level. Circulating concentrations of Lp(a) are genetically determined; desirable levels are amplt; 50 mg/dl...
January 12, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/27940769/progressive-aortic-stenosis-in-homozygous-familial-hypercholesterolemia-after-liver-transplant
#9
Margaret Greco, Joshua D Robinson, Osama Eltayeb, Irwin Benuck
Early onset coronary artery disease and aortic calcifications are characteristic features of patients with homozygous familial hypercholesterolemia. Standard medical therapy includes dietary modification, pharmacotherapy, and lipoprotein apheresis to lower serum low-density lipoprotein cholesterol (LDL-C). Liver transplant is a surgical option for the treatment of homozygous familial hypercholesterolemia and can lead to normal cholesterol levels. Vascular calcifications are known to progress despite standard medical therapy and have been reported after liver transplant in the setting of rejection...
November 2016: Pediatrics
https://www.readbyqxmd.com/read/27919346/disease-control-via-intensified-lipoprotein-apheresis-in-three-siblings-with-familial-hypercholesterolemia
#10
Christina Taylan, Andrea Schlune, Thomas Meissner, Karolis Ažukaitis, Floris E A Udink Ten Cate, Lutz T Weber
BACKGROUND: Familial hypercholesterolemia (FH), the prevalent monogenic form of hypercholesterolemia, carries the risk of premature coronary heart disease. Lipoprotein-apheresis is established in children with severe dyslipidemia. We present 3 siblings with a negative/negative residual low-density lipoprotein (LDL) receptor mutation (p.Trp577Arg), unresponsive to drug treatment. OBJECTIVE: Intensified lipoprotein-apheresis is well tolerated and results in permanently low lipid values without harming the health-related quality of life in children...
November 2016: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/27878139/compound-heterozygous-ldlr-variant-in-severely-affected-familial-hypercholesterolemia-patient
#11
Faisal A Al-Allaf, Abdullah Alashwal, Zainularifeen Abduljaleel, Mohiuddin M Taher, Abdellatif Bouazzaoui, Hala Abalkhail, Ahmad F Al-Allaf, Mohammad Athar
Familial hypercholesterolemia (FH) is most commonly caused by mutations in the LDL receptor (LDLR), which is responsible for hepatic clearance of LDL from the blood circulation. We described a severely affected FH proband and their first-degree blood relatives; the proband was resistant to statin therapy and was managed on an LDL apheresis program. In order to find the causative genetic variant in this family, direct exon sequencing of the LDLR, APOB and PCSK9 genes was performed. We identified a compound heterozygous mutation in the proband with missense p...
2017: Acta Biochimica Polonica
https://www.readbyqxmd.com/read/27859540/the-impact-of-citrate-concentration-on-adhesion-of-platelets-and-leukocytes-to-adsorbents-in-whole-blood-lipoprotein-apheresis
#12
René Weiss, Michael B Fischer, Viktoria Weber
Lipoprotein apheresis is applied to deplete low density lipoprotein and other apolipoprotein B containing lipoproteins in patients with severe familial hypercholesterolemia, hypertriglyceridemia associated pancreatitis, or lipoprotein (a)-hyperlipoproteinemia. Anticoagulation of the extracorporeal circuit may influence cellular activation, as evidenced by a reduction of inflammatory parameters during regional citrate anticoagulation with acid citrate dextrose A (ACD-A) commonly used in whole blood lipid apheresis...
November 17, 2016: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/27761705/emerging-therapeutic-options-for-lowering-of-lipoprotein-a-implications-for-prevention-of-cardiovascular-disease
#13
REVIEW
Michael B Boffa
PURPOSE OF REVIEW: Elevated plasma concentrations of lipoprotein(a) (Lp(a)) are an independent and causal risk factor for cardiovascular diseases including coronary artery disease, ischemic stroke, and calcific aortic valve stenosis. This review summarizes the rationale for Lp(a) lowering and surveys relevant clinical trial data using a variety of agents capable of lowering Lp(a). RECENT FINDINGS: Contemporary guidelines and recommendations outline populations of patients who should be screened for elevated Lp(a) and who might benefit from Lp(a) lowering...
December 2016: Current Atherosclerosis Reports
https://www.readbyqxmd.com/read/27756331/impact-of-selective-ldl-apheresis-on-serum-chemerin-levels-in-patients-with-hypercholesterolemia
#14
Viktória E Varga, Hajnalka Lőrincz, Noémi Zsíros, Péter Fülöp, Ildikó Seres, György Paragh, József Balla, Mariann Harangi
BACKGROUND: Selective low-density lipoprotein (LDL) apheresis is commonly used to treat patients with familial hypercholesterolemia (FH). Chemerin is an adipokine with putative roles in the regulation of lipid metabolism. METHODS: In our pilot study, we measured serum chemerin levels by enzyme-linked immunosorbent assay in six severe heterozygous FH patients before and after their first LDL apheresis treatments using the technique of direct adsorption of lipoproteins (DALI)...
October 18, 2016: Lipids in Health and Disease
https://www.readbyqxmd.com/read/27690713/targeting-microsomal-triglyceride-transfer-protein-and-lipoprotein-assembly-to-treat-homozygous-familial-hypercholesterolemia
#15
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...
January 2017: Critical Reviews in Clinical Laboratory Sciences
https://www.readbyqxmd.com/read/27578108/management-of-homozygous-familial-hypercholesterolemia-in-real-world-clinical-practice-a-report-of-7-italian-patients-treated%C3%A2-in%C3%A2-rome-with-lomitapide-and-lipoprotein%C3%A2-apheresis
#16
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
https://www.readbyqxmd.com/read/27435024/liver-transplantation-for-homozygote-familial-hypercholesterolemia-the-only-curative-treatment
#17
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...
December 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27429525/transfusion-related-acute-lung-injury-trali-a-single-institution-experience-of-15%C3%A2-years
#18
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
https://www.readbyqxmd.com/read/27416576/the-1-st-and-the-2-nd-italian-consensus-conferences-on-low-density-lipoprotein-apheresis-a-practical-synopsis-and-update
#19
REVIEW
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
https://www.readbyqxmd.com/read/27318831/transition-from-ldl-apheresis-to-evolocumab-in-heterozygous-fh-is-equally-effective-in-lowering-ldl-without-lowering-hdl-cholesterol
#20
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
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