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Lipid apheresis

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https://www.readbyqxmd.com/read/29785886/treatment-of-homozygous-familial-hypercholesterolaemia-in-paediatric-patients-a-monocentric-experience
#1
Paola S Buonuomo, Marina Macchiaiolo, Giovanna Leone, Paola Valente, Gerarda Mastrogiorgio, Maria Gnazzo, Ippolita Rana, Michaela V Gonfiantini, Maria G Gagliardi, Francesca Romano, Andrea Bartuli
Background Homozygous familial hypercholesterolaemia is a rare life-threatening disease characterized by markedly elevated low-density lipoprotein cholesterol (LDL-C) concentrations and accelerated atherosclerosis. The presence of double gene defects in the LDL-Receptor, either the same defect (homozygous) or two different LDL-raising mutations (compound heterozygotes) or other variants, identify the homozygous phenotype (HopFH). Apheresis is a procedure in which plasma is separated from red blood cells before the physical removal of LDL-C or the LDL-C is directly removed from whole blood...
January 1, 2018: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/29702000/the-effect-of-intravenous-insulin-apheresis-and-oral-lipid-lowering-agents-on-non-fasting-hypertriglyceridemia-and-associated-pancreatitis
#2
Daniela Frankova, Kristin Melissa Olson, Brian Joseph Whyms, Maria Alejandra Guevara Hernandez, Jan Franko
OBJECTIVES: There is evidence that increasing severity of hypertriglyceridemia increases the risk of acute pancreatitis. There is a debate about superiority of treatment methods and previous works have specifically called for direct comparison between IV insulin and apheresis techniques. Identify patient characteristics predictive of lipid-lowering therapy selection in a large community hospital for treatment of hypertriglyceridemia; evaluate for a concentration-dependent relationship between hypertriglyceridemia severity and risk of acute pancreatitis; assess for differences in clinical outcomes between patients treated with IV insulin versus apheresis...
May 4, 2018: Postgraduate Medicine
https://www.readbyqxmd.com/read/29540222/lipoprotein-turnover-and-possible-remnant-accumulation-in-preeclampsia-insights-from-the-freiburg-preeclampsia-h-e-l-p-apheresis-study
#3
Christine Contini, Martin Jansen, Brigitte König, Filiz Markfeld-Erol, Mirjam Kunze, Stefan Zschiedrich, Ulrich Massing, Irmgard Merfort, Heinrich Prömpeler, Ulrich Pecks, Karl Winkler, Gerhard Pütz
BACKGROUND: Preeclampsia is a life-threatening disease in pregnancy, and its complex pathomechanisms are poorly understood. In preeclampsia, lipid metabolism is substantially altered. In late onset preeclampsia, remnant removal disease like lipoprotein profiles have been observed. Lipid apheresis is currently being explored as a possible therapeutic approach to prolong preeclamptic pregnancies. Here, apheresis-induced changes in serum lipid parameters are analyzed in detail and their implications for preeclamptic lipid metabolism are discussed...
March 14, 2018: Lipids in Health and Disease
https://www.readbyqxmd.com/read/29502162/multimodal-lipid-lowering-treatment-in-pediatric-patients-with-homozygous-familial-hypercholesterolemia-target-attainment-requires-further-increase-of-intensity
#4
Günter Klaus, Christina Taylan, Rainer Büscher, Claus Peter Schmitt, Lars Pape, Jun Oh, Joenna Driemeyer, Matthias Galiano, Jens König, Carsten Schürfeld, Ralf Spitthöver, Juergen R Schaefer, Lutz T Weber, Andreas Heibges, Reinhard Klingel
BACKGROUND: Familial hypercholesterolemia (FH) causes premature cardiovascular disease (CVD). Lipoprotein apheresis (LA) is recommended as first-line lipid-lowering treatment (LLT) for homozygous (ho) FH. METHODS: Efficacy of multimodal LLT including lifestyle counseling, drug treatment, and LA was analyzed in 17 pediatric hoFH or compound heterozygous (c-het) FH patients, who commenced chronic LA in Germany before the age of 18. RESULTS: At time of diagnosis, mean low-density lipoprotein cholesterol (LDL-C) concentration was 19...
March 3, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29498875/-the-role-of-pcsk9-inhibitors-and-of-lipoprotein-apheresis-in-the-treatment-of-homozygous-and-severe-heterozygous-familial-hypercholesterolemia-a-rivalry-or-are-things-quite-different
#5
Vladimír Bláha, Milan Bláha, Miriam Lánská, Eduard Havel, Pavel Vyroubal, Zdeněk Zadák, Pavel Žák
PCSK9-inhibitors belong to the new class of hypolipidemic agents. They enhance catabolism of low density lipoprotein cholesterol (LDL-C) through inhibiting activity of proprotein convertase subtilisin/kexin type 9 (PCSK9). They are monoclonal antibodies (alirocumab, evolocumab etc). Under clinical development are also other types of PCSK9-inhibitors which act at a subcellular level. The treatment with PCSK9-inhibitors can be beneficially combined with lipoprotein apheresis (LA). If such treatment using PCSK9-inhibitors is possible with regard to an individual patients genotype, the combination of LA and PCSK9-inhibitors leads to slowing the space of LDL-C increase between individual procedures of apheresis and enables attaining of the lowest possible values of LDL-cholesterolemia for the longest possible period of time...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29473701/antibody-epitope-of-human-%C3%AE-galactosidase-a-revealed-by-affinity-mass-spectrometry-a-basis-for-reversing-immunoreactivity-in-enzyme-replacement-therapy-of-fabry-disease
#6
Zdenek Kukacka, Marius Iurascu, Loredana Lupu, Hendrik Rusche, Mary Murphy, Lorenzo Altamore, Fabio Borri, Stefan Maeser, Anna Maria Papini, Julia Hennermann, Michael Przybylski
α-Galactosidase (αGal) is a lysosomal enzyme that hydrolyses the terminal α-galactosyl moiety from glycosphingolipids. Mutations in the encoding genes for αGal lead to defective or misfolded enzyme, which results in substrate accumulation and subsequent organ dysfunction. The metabolic disease caused by a deficiency of human α-galactosidase A is known as Fabry disease or Fabry-Anderson disease, and it belongs to a larger group known as lysosomal storage diseases. An effective treatment for Fabry disease has been developed by enzyme replacement therapy (ERT), which involves infusions of purified recombinant enzyme in order to increase enzyme levels and decrease the amounts of accumulated substrate...
May 8, 2018: ChemMedChem
https://www.readbyqxmd.com/read/29389816/lomitapide-in-homozygous-familial-hypercholesterolemia-cardiology-perspective-from-a-single-center-experience
#7
Simona Sperlongano, Felice Gragnano, Francesco Natale, Laura D'Erasmo, Claudia Concilio, Arturo Cesaro, Enrica Golia, Mario Crisci, Rossella Sperlongano, Fabio Fimiani, Mariagiovanna Russo, Marcello Arca, Giuseppe Limongelli, Paolo Calabrò
AIMS: Homozygous familial hypercholesterolemia (HoFH) is a genetic dyslipidemia characterized by elevated levels of low-density lipoprotein cholesterol (LDL-C) and accelerated atherosclerosis. Frequently, traditional lipid-lowering therapy is ineffective in these patients, and lipoprotein apheresis is required. Lomitapide has been recently approved for HoFH. We reported our experience in HoFH patients treated with lomitapide, evaluating its efficacy and safety profile. METHODS: Probands suspected for familial hypercholesterolemia were extrapolated from the registry of patients admitted to our cardiology department...
March 2018: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/29365934/impact-of-lipoprotein-apheresis-on-the-content-of-alpha-tocopherol-in-cell-membranes-and-lipid-peroxidation
#8
Vladimir Blaha, Dagmar Solichova, Milan Blaha, Miriam Lanska, Eduard Havel, Pavel Vyroubal, Lenka Kujovska Krcmova, Pavel Zak, Lubos Sobotka, Joao Fortunato
No abstract text is available yet for this article.
August 2017: Atherosclerosis
https://www.readbyqxmd.com/read/29348020/autosomal-recessive-hypercholesterolemia-long-term-cardiovascular-outcomes
#9
Laura D'Erasmo, Ilenia Minicocci, Antonio Nicolucci, Paolo Pintus, Janine E Roeters Van Lennep, Luis Masana, Pedro Mata, Rosa Maria Sánchez-Hernández, Pablo Prieto-Matos, Josè T Real, Juan F Ascaso, Eduardo Esteve Lafuente, Miguel Pocovi, Francisco J Fuentes, Sandro Muntoni, Stefano Bertolini, Cesare Sirtori, Laura Calabresi, Chiara Pavanello, Maurizio Averna, Angelo Baldassare Cefalu, Davide Noto, Adolfo Arturo Pacifico, Giovanni Mario Pes, Mariko Harada-Shiba, Enzo Manzato, Sabina Zambon, Alberto Zambon, Anja Vogt, Marco Scardapane, Barbara Sjouke, Renato Fellin, Marcello Arca
BACKGROUND: Autosomal recessive hypercholesterolemia (ARH) is a rare lipid disorder characterized by premature atherosclerotic cardiovascular disease (ASCVD). There are sparse data for clinical management and cardiovascular outcomes in ARH. OBJECTIVES: Evaluation of changes in lipid management, achievement of low-density lipoprotein cholesterol (LDL-C) goals and cardiovascular outcomes in ARH. METHODS: Published ARH cases were identified by electronic search...
January 23, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29316258/characterization-of-fractionation-membranes-in-an-animal-model-of-double-filtration-lipoprotein-apheresis
#10
Detlef H Krieter, Florian Lange, Horst-Dieter Lemke, Florian Bonn, Christoph Wanner
Technical problems during clinical lipid apheresis interfere with fractionator performance. Therefore, a large animal model was established to characterize a new plasma fractionation membrane. Four sheep were randomized, controlled, and crossover subjected to double ofiltration lipoprotein apheresis with three specimens of FractioPESR having slightly different HDL sieving coefficients (SK ) (FPESa, 0.30, FPESb, 0.26, and FPESc, 0.22) versus a control fractionator (EVAL). SK and reduction ratios were determined for LDL, HDL, fibrinogen, IgG, and albumin...
April 2018: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/29286309/lowering-low-density-lipoprotein-particles-in-plasma-using-dextran-sulphate-co-precipitates-procoagulant-extracellular-vesicles
#11
Jiong-Wei Wang, Ya-Nan Zhang, Siu Kwan Sze, Sander M van de Weg, Flora Vernooij, Arjan H Schoneveld, Sock-Hwee Tan, Henri H Versteeg, Leo Timmers, Carolyn S P Lam, Dominique P V de Kleijn
Plasma extracellular vesicles (EVs) are lipid membrane vesicles involved in several biological processes including coagulation. Both coagulation and lipid metabolism are strongly associated with cardiovascular events. Lowering very-low- and low-density lipoprotein ((V)LDL) particles via dextran sulphate LDL apheresis also removes coagulation proteins. It remains unknown, however, how coagulation proteins are removed in apheresis. We hypothesize that plasma EVs that contain high levels of coagulation proteins are concomitantly removed with (V)LDL particles by dextran sulphate apheresis...
December 29, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29176657/dyslipidaemia-in-nephrotic-syndrome-mechanisms-and-treatment
#12
REVIEW
Shipra Agrawal, Joshua J Zaritsky, Alessia Fornoni, William E Smoyer
Nephrotic syndrome is a highly prevalent disease that is associated with high morbidity despite notable advances in its treatment. Many of the complications of nephrotic syndrome, including the increased risk of atherosclerosis and thromboembolism, can be linked to dysregulated lipid metabolism and dyslipidaemia. These abnormalities include elevated plasma levels of cholesterol, triglycerides and the apolipoprotein B-containing lipoproteins VLDL and IDL; decreased lipoprotein lipase activity in the endothelium, muscle and adipose tissues; decreased hepatic lipase activity; and increased levels of the enzyme PCSK9...
January 2018: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/29114919/circulating-progenitor-cells-in-patients-with-familial-hypercholesterolemia
#13
P B Sandesara, V Ramjee, N Ghasemzadeh, Y Guo, N Bhatia, Q Li, L Vaughn, C Nell-Dybdahl, E K Waller, E A Mahar, K Brigham, P W F Wilson, A Quyyumi, N-A Le, L S Sperling
OBJECTIVE: Familial hypercholesterolemia (FH) is a genetic disease with very high levels of circulating low density lipoprotein cholesterol (LDL-C) levels that leads to accelerated atherosclerosis. Lipoprotein apheresis is an effective treatment option for patients with FH and results in reduced cardiovascular morbidity and mortality. Circulating progenitor cells (CPCs) are markers of overall vascular health and diminished levels have been associated with decreased reparative potential and worse outcomes...
November 8, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/29106543/survival-in-homozygous-familial-hypercholesterolaemia-is-determined-by-the-on-treatment-level-of-serum-cholesterol
#14
Gilbert R Thompson, Dirk J Blom, A David Marais, Mary Seed, Gillian J Pilcher, Frederick J Raal
Aims: Homozygous familial hypercholesterolaemia (FH) is a rare inherited disorder characterized by extreme hypercholesterolaemia from birth, accelerated atherosclerosis, and premature death. Many forms of lipid-lowering therapies have been used in the past, but definitive evidence of benefit has been lacking. We therefore undertook a retrospective survey of lipid levels and clinical outcomes of FH homozygotes treated with a combination of lipid-lowering measures between 1990 and 2014 in South Africa and the UK...
April 7, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29096861/current-insights-into-the-german-lipoprotein-apheresis-registry-glar-almost-5-years-on
#15
V J J Schettler, C L Neumann, C Peter, T Zimmermann, U Julius, E Roeseler, F Heigl, P Grützmacher, H Blume
BACKGROUND: In recent years the Federal Joint Committee (G-BA), a paramount decision-making body of the German health care system required a reassessment of the approval of chronic lipoprotein apheresis therapy for regular reimbursement. Since 2005 an interdisciplinary German apheresis working group has been established by members of both German Societies of Nephrology. In 2009 the working group completed the indication for lipoprotein apheresis with respect to current cardiovascular guidelines and current scientific knowledge for the registry...
November 2017: Atherosclerosis. Supplements
https://www.readbyqxmd.com/read/29096860/current-insights-into-the-german-lipoprotein-apheresis-standard-pcsk9-inhibitors-lipoprotein-apheresis-or-both
#16
V J J Schettler, J Ringel, S Jacob, U Julius, R Klingel, F Heigl, E Roeseler, P Grützmacher
According to current European guidelines, lipid lowering therapy for progressive cardiovascular disease including cardiovascular events has to be focused on a target level for LDL-C. In contrast for Lp(a) a threshold has to be defined with respect to the method of measurement. However, due to new lipid lowering drug developments like PCSK9-inhibitors (PCSK-9-I) a therapeutic algorithm for patients with severe hypercholesterolemia or isolated Lipoprotein(a)-hyperlipoproteinemia with progressive cardiovascular disease may be necessary to manage the use of PCSK9-I, lipoprotein apheresis (LA) or both...
November 2017: Atherosclerosis. Supplements
https://www.readbyqxmd.com/read/29096859/lipoprotein-a-apheresis-in-the-light-of-new-drug-developments
#17
Anja Vogt
Elevated levels of lipoprotein(a) (Lp(a)) contribute to the risk of early and severe cardiovascular disease (CVD). Recently <50 mg/dl was recommended as the desirable level for clinical use and decision making. All established medical therapies to lower cholesterol levels have no impact on lowering Lp(a) except niacin which is all too often poorly tolerated and not obtainable everywhere. Lipoprotein apheresis is an extracorporeal treatment to lower levels of Lp(a) significantly by > 60%. In some countries it is recommended in very high risk patients with early or progressive CVD...
November 2017: Atherosclerosis. Supplements
https://www.readbyqxmd.com/read/29096858/management-of-patients-with-statin-intolerance
#18
Sabine Fischer, Ulrich Julius
In recent years statins have become an established option in lipid-lowering pharmacotherapy despite the fact that statin intolerance is fairly common. When muscle pains and/or an elevation of the creatine kinase appear, the dose must be lowered in patients with slight symptoms or stopped altogether if the symptoms are more severe. When the symptoms are alleviated and creatine kinase is normalized, re-exposition can be considered. If symptoms recur, treatment with another statin should be attempted - in these cases pravastatin or fluvastatin are recommended, although they are less effective in reducing LDL cholesterol...
November 2017: Atherosclerosis. Supplements
https://www.readbyqxmd.com/read/29096841/efficacy-of-lipid-reduction-with-dali-and-monet
#19
MULTICENTER STUDY
Wolfgang Ramlow, Eberhard Röseler, Franz Heigl, Ralf Spitthöver, Jens Ringel, Gerd Schmitz, Rainer Heinzler, Nadim Abdul-Rahman, Frank Leistikow, Frido Himmelsbach, Volker Schettler, Jenny Pham, Justyna Kozik-Jaromin
BACKGROUND: Lipidapheresis techniques are increasingly used to treat drug-resistant hyperlipidemia but few efficacy studies under routine application are available. In this multicenter observational study we investigated direct adsorption of lipoproteins (DALI) and lipoprotein filtration (MONET) for the short and the long-term effects on lipid-lowering effects. METHODS: Data of 122 apheresis patients from 11 centers (DALI: n = 78, MONET: n = 44) were prospectively collected for a period of 2 years...
November 2017: Atherosclerosis. Supplements
https://www.readbyqxmd.com/read/29096840/kinetics-of-lipoprotein-a-in-patients-undergoing-weekly-lipoprotein-apheresis-for-lp-a-hyperlipoproteinemia
#20
S Tselmin, G Müller, U Schatz, U Julius, S R Bornstein, B Hohenstein
INTRODUCTION: Lipoprotein apheresis (LA) represents the only effective therapeutic option for patients with elevated Lipoprotein(a) (Lp(a)) levels. We aimed at analyzing the Lp(a) reduction, rebound rates as well as mean interval values between two weekly apheresis sessions, since this might be important for the prediction of the residual cardiovascular risk and development of individualized approaches for this special therapeutic strategy. MATERIALS AND METHODS: 20 patients under weekly and 2 patients under twice weekly apheresis were included...
November 2017: Atherosclerosis. Supplements
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