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https://www.readbyqxmd.com/read/28913682/-therapeutic-options-in-hyperlipidemia
#1
Robert Berent, Theresa Berent, Helmut Sinzinger
Treatment of lipid disorders (dyslipidemia) is the cornerstone of atherosclerosis prevention and reduction of progression. Lifestyle modification is the first step to improve the plasma lipid profile. Statins play a central role in the reduction of LDL cholesterol. Whether and to what extent other lipids such as triglycerides or lipoprotein(a) should also be treated depends on the extent of atherosclerotic disease and its progression over time. Especially in residential cardiac rehabilitation we have the opportunity to encourage adherence and adapt medication as necessary due to a face to face contact over 4 weeks...
September 14, 2017: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28856989/from-vulnerable-plaque-to-blood-healthy-therapy
#2
Sun Yuhua, Wang Baoping
Cardiovascular atherosclerotic disease is the leading cause of death in China and in Western nations. People with plaque or stenosis in the coronary artery or the carotid artery are the most susceptible population to suffer from acute events. Current investigations showed that plaque with the characteristics of intra-plaque hemorrhage or a thin cap with a large lipid core was causally associated with vulnerable plaque and plaque rupture. Of the many plaque ruptures occurring in patients with atherosclerotic disease, very few will trigger symptomatic events, rendering it exceedingly difficult to predict adverse outcomes...
August 1, 2017: Perfusion
https://www.readbyqxmd.com/read/28838366/efficacy-of-rosuvastatin-in-children%C3%A2-with%C3%A2-homozygous-familial-hypercholesterolemia-and-association-with-underlying-genetic-mutations
#3
RANDOMIZED CONTROLLED TRIAL
Evan A Stein, Eldad J Dann, Albert Wiegman, Flemming Skovby, Daniel Gaudet, Etienne Sokal, Min-Ji Charng, Mafauzy Mohamed, Ilse Luirink, Joel S Raichlen, Mattias Sundén, Stefan C Carlsson, Frederick J Raal, John J P Kastelein
BACKGROUND: Homozygous familial hypercholesterolemia (HoFH), a rare genetic disorder, is characterized by extremely elevated levels of low-density lipoprotein cholesterol (LDL-C) and accelerated atherosclerotic cardiovascular disease. Statin treatment starts at diagnosis, but no statin has been formally evaluated in, or approved for, HoFH children. OBJECTIVES: The authors sought to assess the LDL-C efficacy of rosuvastatin versus placebo in HoFH children, and the relationship with underlying genetic mutations...
August 29, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28761763/premature-valvular-heart-disease-in-homozygous-familial-hypercholesterolemia
#4
Akl C Fahed, Kamel Shibbani, Rabih R Andary, Mariam T Arabi, Robert H Habib, Denis D Nguyen, Fady F Haddad, Elie Moubarak, Georges Nemer, Sami T Azar, Fadi F Bitar
Valvular heart disease frequently occurs as a consequence of premature atherosclerosis in individuals with familial hypercholesterolemia (FH). Studies have primarily focused on aortic valve calcification in heterozygous FH, but there is paucity of data on the incidence of valvular disease in homozygous FH. We performed echocardiographic studies in 33 relatively young patients (mean age: 26 years) with homozygous FH (mean LDL of 447 mg/dL, 73% on LDL apheresis) to look for subclinical valvulopathy. Twenty-one patients had evidence of valvulopathy of the aortic or mitral valves, while seven subjects showed notable mitral regurgitation...
2017: Cholesterol
https://www.readbyqxmd.com/read/28725307/acute-pancreatitis-secondary-to-severe-hypertriglyceridemia-management-of-severe-hypertriglyceridemia-in-emergency-setting
#5
Ahmad Chaudhary, Umair Iqbal, Hafsa Anwar, Hafiz Umair Siddiqui, Madiha Alvi
Hypertriglyceridemia (HTG) is the third most common cause of acute pancreatitis (AP). The incidence of AP is around 10-20% with levels > 2,000 mg/dL. We present here a case of a 44-year-old male with history of uncontrolled diabetes mellitus and HTG admitted with severe abdominal pain. Labs revealed elevated lipase and amylase. CT of abdomen with contrast showed AP. He was found to have a triglyceride (TG) level of 6,672 mg/dL. Besides conventional treatment for AP with intravenous (IV) hydration, he was started on IV regular insulin along with dextrose saline...
June 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28572002/toward-an-international-consensus-integrating-lipoprotein-apheresis-and-new-lipid-lowering-drugs
#6
REVIEW
Claudia Stefanutti, Ulrich Julius, Gerald F Watts, Mariko Harada-Shiba, Maria Cossu, Volker J Schettler, Giustina De Silvestro, Handrean Soran, Jeanine Roeters Van Lennep, Livia Pisciotta, Hans U Klör, Kurt Widhalm, Patrick M Moriarty
BACKGROUND: Despite advances in pharmacotherapy of lipid disorders, many dyslipidemic patients do not attain sufficient lipid lowering to mitigate risk of atherosclerotic cardiovascular disease. Several classes of novel lipid-lowering agents are being evaluated to reduce atherosclerotic cardiovascular disease risk. Lipoprotein apheresis (LA) is effective in acutely lowering the plasma concentrations of atherogenic lipoproteins including low-density lipoprotein cholesterol and lipoprotein(a), and novel lipid-lowering drugs may dampen the lipid rebound effect of LA, with the possibility that LA frequency may be decreased, in some cases even be discontinued...
April 25, 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28555526/statin-intolerance-in-heterozygous-familial-hypercolesterolemia-with-cardiovascular-disease-after-pcsk-9-antibodies-what-else
#7
Francesco Sbrana, Beatrice Dal Pino, Federico Bigazzi, Andrea Ripoli, Claudio Passino, Alessandra Gabutti, Emilio M Pasanisi, Christina Petersen, Alessandro Valleggi, Giancarlo Todiere, Andrea Barison, Alberto Giannoni, Luca Panchetti, Francesco Becherini, Mascia Pianelli, Roberta Luciani, Tiziana Sampietro
Background Familial hypercholesterolemia is the elective clinical condition that deserves the maximal personalisation in lipid-lowering therapy, especially in the presence of statin intolerance. Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors represent a promising approach to lower low-density lipoprotein (LDL) cholesterol. Methods We enrolled 18 patients (mean age 62 ± 8 years, 72% men) affected by heterozygous familial hypercholesterolemia and cardiovascular disease, with a history of statin intolerance assigned to PCSK9 inhibitors...
January 1, 2017: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/28506388/discordant-response-of-low-density-lipoprotein-cholesterol-and-lipoprotein-a-levels-to-monoclonal-antibodies-targeting-proprotein-convertase-subtilisin-kexin-type-9
#8
Jonathan B Edmiston, Nathan Brooks, Hagai Tavori, Jessica Minnier, Bart Duell, Jonathan Q Purnell, Tina Kaufman, Cezary Wojcik, Szilard Voros, Sergio Fazio, Michael D Shapiro
BACKGROUND: Clinical trials testing proprotein convertase subtilisin/kexin type 9 inhibitors (PCSK9i) have demonstrated an unanticipated but significant lipoprotein (a) (Lp(a))-lowering effect, on the order of 25% to 30%. Although the 50% to 60% reduction in low-density lipoprotein (LDL)-cholesterol (LDL-C) achieved by PCSK9i is mediated through its effect on LDL receptor (LDLR) preservation, the mechanism for Lp(a) lowering is unknown. OBJECTIVE: We sought to characterize the degree of concordance between LDL-C and Lp(a) lowering because of PCSK9i in a standard of care patient cohort...
May 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28506384/managing-the-challenging-homozygous-familial-hypercholesterolemia-patient-academic-insights-and-practical-approaches-for-a-severe-dyslipidemia-a-national-lipid-association-masters-summit
#9
EDITORIAL
G Kees Hovingh, Anne C Goldberg, Patrick M Moriarty
The following article represents material presented and discussed at a symposium hosted by the National Lipid Association hosted entitled "Managing the Challenging Homozygous Familial Hypercholesterolemia Patient-Academic Insights and Practical Approaches for a Severe Dyslipidemia" on November 7, 2015 in Orlando, FL. Presenters included G.K.H., A.C.G, and P.M.M. The diagnosis and genetic causes of extremely high low-density lipoprotein (LDL) cholesterol, which has become known as homozygous familial hypercholesterolemia, were discussed...
May 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28432645/efficacy-of-lomitapide-in-the-treatment-of-familial-homozygous-hypercholesterolemia-results-of-a-real-world-clinical-experience-in-italy
#10
Laura D'Erasmo, Angelo Baldassare Cefalù, Davide Noto, Antonina Giammanco, Maurizio Averna, Paolo Pintus, Paolo Medde, Giovanni Battista Vigna, Cesare Sirtori, Laura Calabresi, Chiara Pavanello, Marco Bucci, Carlo Sabbà, Patrizia Suppressa, Francesco Natale, Paolo Calabrò, Tiziana Sampietro, Federico Bigazzi, Francesco Sbrana, Katia Bonomo, Fulvio Sileo, Marcello Arca
INTRODUCTION: Homozygous familial hypercholesterolaemia (HoFH) is a rare form of inherited dyslipidemia resistant to conventional cholesterol-lowering medications so that lipoprotein apheresis (LA) is usually required. Lomitapide has been approved for the treatment of HoFH. The aim of this study was to evaluate the benefits of lomitapide in HoFH patients followed with the usual clinical care. METHODS: Clinical and biochemical data were retrospectively collected in 15 HoFH patients (10 with mutations in the LDLR gene and 5 in the LDLRAP1 gene) treated for at least 6 months with lomitapide in addition to lipid-lowering therapies (LLT) in different Lipid Clinics across Italy...
May 2017: Advances in Therapy
https://www.readbyqxmd.com/read/28429694/the-rationale-and-design-of-the-national-familial-hypercholesterolemia-registries-in-turkey-a-hit1-and-a-hit2-studies
#11
Meral Kayıkçıoğlu, Lale Tokgözoğlu
OBJECTIVE: Familial hypercholesterolemia (FH) is a genetic disease characterized by extremely high levels of cholesterol, leading to premature atherosclerosis. Although many countries have already addressed the burden of FH by means of national registries, Turkey has no national FH registry or national screening program to detect FH. Creation of a series of FH registries is planned as part of Turkish FH Initiative endorsed by the Turkish Society of Cardiology to meet this need. This article provides detailed information on the rationale and design of the first 2 FH registries (A-HIT1 and A-HIT2)...
April 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28391901/low-density-lipoprotein-receptor-negative-compound-heterozygous-familial-hypercholesterolemia-two-lifetime-journeys-of-lipid-lowering-therapy
#12
Reyhana Yahya, Monique T Mulder, Eric J G Sijbrands, Monique Williams, Jeanine E Roeters van Lennep
We present the case history of 2 patients with low-density lipoprotein receptor-negative compound heterozygous familial hypercholesterolemia who did not receive lipoprotein apheresis. We describe the subsequent effect of all lipid-lowering medications during their life course including resins, statins, ezetimibe, nicotinic acid/laropiprant, mipomersen, and lomitapide. These cases tell the story of siblings affected with this rare disease, who are free of symptoms but still are at a very high cardiovascular disease risk, and their treatment from childhood...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28377633/vldl-ldl-acts-as-a-drug-carrier-and-regulates-the-transport-and-metabolism-of-drugs-in-the-body
#13
Hideaki Yamamoto, Tappei Takada, Yoshihide Yamanashi, Masatsune Ogura, Yusuke Masuo, Mariko Harada-Shiba, Hiroshi Suzuki
Only free drugs have been believed to be carried into tissues through active or passive transport. However, considering that lipoproteins function as carriers of serum lipids such as cholesterol and triglycerides, we hypothesized that lipoproteins can associate with certain drugs and mediate their transport into tissues in lipid-associated form. Here, in vitro and in vivo studies with low density lipoprotein receptor (LDLR)-overexpressing or -knockdown cells and wild-type or LDLR-mutant mice were used to show the association of various drugs with lipoproteins and the uptake of lipoprotein-associated drugs through a lipoprotein receptor-mediated process...
April 4, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28329241/lipoprotein-a-the-revenant
#14
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'...
May 21, 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
#15
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...
April 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28255870/efficacy-and-safety-of-lomitapide-in-hypercholesterolemia
#16
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...
August 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
#17
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
#18
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
#19
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...
April 3, 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
#20
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
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