keyword
https://read.qxmd.com/read/38598969/clinical-practice-recommendations-on-lipoprotein-apheresis-for-children-with-homozygous-familial-hypercholesterolaemia-an-expert-consensus-statement-from-erknet-and-espn
#1
REVIEW
M Doortje Reijman, D Meeike Kusters, Jaap W Groothoff, Klaus Arbeiter, Eldad J Dann, Lotte M de Boer, Sarah D de Ferranti, Antonio Gallo, Susanne Greber-Platzer, Jacob Hartz, Lisa C Hudgins, Daiana Ibarretxe, Meral Kayikcioglu, Reinhard Klingel, Genovefa D Kolovou, Jun Oh, R Nils Planken, Claudia Stefanutti, Christina Taylan, Albert Wiegman, Claus Peter Schmitt
Homozygous familial hypercholesterolaemia is a life-threatening genetic condition, which causes extremely elevated LDL-C levels and atherosclerotic cardiovascular disease very early in life. It is vital to start effective lipid-lowering treatment from diagnosis onwards. Even with dietary and current multimodal pharmaceutical lipid-lowering therapies, LDL-C treatment goals cannot be achieved in many children. Lipoprotein apheresis is an extracorporeal lipid-lowering treatment, which is used for decades, lowering serum LDL-C levels by more than 70% directly after the treatment...
March 27, 2024: Atherosclerosis
https://read.qxmd.com/read/38565461/a-focused-update-to-the-2019-nla-scientific-statement-on-use-of-lipoprotein-a-in-clinical-practice
#2
JOURNAL ARTICLE
Marlys L Koschinsky, Archna Bajaj, Michael B Boffa, Dave L Dixon, Keith C Ferdinand, Samuel S Gidding, Edward A Gill, Terry A Jacobson, Erin D Michos, Maya S Safarova, Daniel E Soffer, Pam R Taub, Michael J Wilkinson, Don P Wilson, Christie M Ballantyne
Since the 2019 National Lipid Association (NLA) Scientific Statement on Use of Lipoprotein(a) in Clinical Practice was issued, accumulating epidemiological data have clarified the relationship between lipoprotein(a) [Lp(a)] level and cardiovascular disease risk and risk reduction. Therefore, the NLA developed this focused update to guide clinicians in applying this emerging evidence in clinical practice. We now have sufficient evidence to support the recommendation to measure Lp(a) levels at least once in every adult for risk stratification...
March 29, 2024: Journal of Clinical Lipidology
https://read.qxmd.com/read/38555232/impact-of-therapeutic-plasma-exchange-on-intact-protein-s-apolipoproteins-and-thrombin-generation
#3
JOURNAL ARTICLE
Hanna H Pitkänen, Mikko Haapio, Mika Saarela, Marja-Riitta Taskinen, Herm-Jan Brinkman, Riitta Lassila
INTRODUCTION: Therapeutic plasma exchange (TPE), with solvent/detergent (S/D)-treated plasma as replacement fluid, is an extracorporeal blood purification technique with major impact on both coagulation and lipids. Our previous in vitro study showed that S/D-plasma enhances thrombin generation by lowering intact protein S (PS) levels. AIMS: To evaluate the impact of altered lipid balance on coagulation phenotype during heparin-anticoagulated TPE with S/D-plasma, and to investigate whether the lowered intact PS levels with concomitant procoagulant phenotype, are recapitulated in vivo...
March 27, 2024: Transfusion and Apheresis Science
https://read.qxmd.com/read/38545781/evolocumab-treatment-in-pediatric-patients-with-homozygous-familial-hypercholesterolemia-pooled-data-from-three-open-label-studies
#4
JOURNAL ARTICLE
Frederick J Raal, Robert A Hegele, Andrea Ruzza, J Antonio G López, Ajay K Bhatia, Johnny Wu, Huei Wang, Daniel Gaudet, Albert Wiegman, Jian Wang, Raul D Santos
BACKGROUND: Pediatric patients with homozygous familial hypercholesterolemia (HoFH) have an increased risk of atherosclerotic cardiovascular disease and difficulty meeting low-density lipoprotein cholesterol (LDL-C) goals. In this post hoc analysis, we evaluated pooled safety and efficacy data from 3 studies in pediatric patients with HoFH treated with the PCSK9 (proprotein convertase subtilisin/kexin type 9) monoclonal antibody inhibitor evolocumab. METHODS: Patients with HoFH aged 10 to 17 years received treatment with open-label evolocumab 420 mg subcutaneously monthly or biweekly in the TAUSSIG, RAMAN, or HAUSER-OLE clinical studies...
March 28, 2024: Arteriosclerosis, Thrombosis, and Vascular Biology
https://read.qxmd.com/read/38541699/novel-therapeutic-approaches-for-the-management-of-elevated-lipoprotein-a-from-traditional-agents-to-future-treatment-options
#5
REVIEW
György Paragh, Péter Zilahi, László Róbert Kolozsvári, Hajnalka Lőrincz, Péter Fülöp, Mariann Harangi
Cardiovascular disease is the leading cause of mortality worldwide. Despite the availability of effective low-density lipoprotein cholesterol (LDL-C) lowering agents, an increased cardiovascular risk is still observed in individuals with therapeutic LDL-C levels. One of these cardiovascular risk factors is elevated plasma lipoprotein(a) (Lp(a)) concentration, which maintains chronic inflammation through the increased presence of oxidized phospholipids on its surface. In addition, due to its 90 percent homology with the fibrinolytic proenzyme plasminogen, Lp(a) exhibits atherothrombotic effects...
March 12, 2024: Life
https://read.qxmd.com/read/38523000/improved-lipid-lowering-treatment-and-reduction-in-cardiovascular-disease-burden-in-homozygous-familial-hypercholesterolemia-the-safeheart-follow-up-study
#6
JOURNAL ARTICLE
Rodrigo Alonso, Raquel Arroyo-Olivares, Jose Luis Díaz-Díaz, Francisco Fuentes-Jiménez, Francisco Arrieta, Raimundo de Andrés, Pablo Gonzalez-Bustos, Rosa Argueso, Mercedes Martin-Ordiales, Ceferino Martinez-Faedo, Fátima Illán, Pedro Saenz, José María Donate, Juan F Sanchez Muñoz-Torrero, Sergio Martinez-Hervas, Pedro Mata
AIM: We aimed to describe clinical and genetic characteristics, lipid-lowering treatment and atherosclerotic cardiovascular disease (ASCVD) outcomes over a long-term follow-up in homozygous familial hypercholesterolemia (HoFH). METHODS: SAFEHEART (Spanish Familial Hypercholesterolaemia Cohort Study) is a long-term study in molecularly diagnosed FH. Data analyzed in HoFH were prospectively obtained from 2004 until 2022. ASCVD events, lipid profile and lipid-lowering treatment were determined...
March 16, 2024: Atherosclerosis
https://read.qxmd.com/read/38510381/delipid-extracorporeal-lipoprotein-filter-from-plasma-system-a-new-intensive-lipid-lowering-therapy-for-patients-with-acute-ischemic-stroke
#7
JOURNAL ARTICLE
Yuqiong Jiao, Qi Yang, Ting Ye, Jun Zhu, Qunyi Li, Xiang Han, Qiang Dong
OBJECTIVES: To investigate the safety and efficacy of the delipid extracorporeal lipoprotein filter from plasma (DELP) system, a new low-density lipoprotein cholesterol (LDL-C) adsorption system, in acute ischemic stroke (AIS) patients. PATIENTS AND METHODS: In the present study, a total of 180 AIS patients were enrolled during March 2019 to February 2021. They were divided into DELP group ( n 1  = 90) and the control group ( n 2  = 90)...
2024: Frontiers in Neurology
https://read.qxmd.com/read/38407694/-eve-is-not-adam-differences-in-efficacy-safety-and-clinical-outcomes-with-lipoprotein-apheresis-between-sexes
#8
JOURNAL ARTICLE
Beatrice Dal Pino, Francesco Sbrana
PURPOSE: In Familial Hypercholesterolemia (FH), female atherosclerotic cardiovascular disease occurs 20 years earlier than in women without FH. The aim of this study is to describe the differences in lipoprotein apheresis (LA), a last therapeutic option, in terms of efficacy, safety and clinical outcomes between the two sexes. MATERIALS AND METHODS: Sex related differences were analysed in 31 subjects in on LA treatment with FH and not achieving LDL-cholesterol and/or Lp(a) target values on maximum lipid-lowering therapies...
February 26, 2024: Endocrine
https://read.qxmd.com/read/38390468/changing-from-lipoprotein-apheresis-to-evolocumab-treatment-lowers-circulating-levels-of-arachidonic-acid-and-oxylipins
#9
JOURNAL ARTICLE
Chaoxuan Wang, Anne Kaufmann, Nadja Kampschulte, Ulf Elbelt, Ursula Kassner, Elisabeth Steinhagen-Thiessen, Anne Pietzner, Christoph Schmöcker, Dev Datta, Tiziana Sanpietro, Nils Helge Schebb, Karsten-H Weylandt, Nadine Rohwer
BACKGROUND AND AIMS: Previous studies have shown that lipoprotein apheresis can modify the plasma lipidome and pro-inflammatory and pro-thrombotic lipid mediators. This has not been examined for treatment with protein convertase subtilisin/kexin type 9 inhibitors such as evolocumab, which are increasingly used instead of lipoprotein apheresis in treatment-resistant familial hypercholesterolemia. The aim of this study was to compare the effects of evolocumab treatment and lipoprotein apheresis on the fatty acid profile and on formation of lipid mediators in blood samples...
March 2024: Atheroscler Plus
https://read.qxmd.com/read/38353972/sex-differences-in-diagnosis-treatment-and-cardiovascular-outcomes-in-homozygous-familial-hypercholesterolemia
#10
JOURNAL ARTICLE
Janneke W C M Mulder, Tycho R Tromp, Mutaz Al-Khnifsawi, Dirk J Blom, Krysztof Chlebus, Marina Cuchel, Laura D'Erasmo, Antonio Gallo, G Kees Hovingh, Ngoc Thanh Kim, Jiang Long, Frederick J Raal, Willemijn A M Schonck, Handrean Soran, Thanh-Huong Truong, Eric Boersma, Jeanine E Roeters van Lennep
IMPORTANCE: Homozygous familial hypercholesterolemia (HoFH) is a rare genetic condition characterized by extremely increased low-density lipoprotein (LDL) cholesterol levels and premature atherosclerotic cardiovascular disease (ASCVD). Heterozygous familial hypercholesterolemia (HeFH) is more common than HoFH, and women with HeFH are diagnosed later and undertreated compared to men; it is unknown whether these sex differences also apply to HoFH. OBJECTIVE: To investigate sex differences in age at diagnosis, risk factors, lipid-lowering treatment, and ASCVD morbidity and mortality in patients with HoFH...
February 14, 2024: JAMA Cardiology
https://read.qxmd.com/read/38336556/impact-of-production-methods-and-storage-conditions-on-extracellular-vesicles-in-packed-red-blood-cells-and-platelet-concentrates
#11
REVIEW
Marie Ebeyer-Masotta, Tanja Eichhorn, Michael B Fischer, Viktoria Weber
The use of blood and blood products can be life-saving, but there are also certain risks associated with their administration and use. Packed red blood cells (pRBCs) and platelet concentrates are the most commonly used blood products in transfusion medicine to treat anemia or acute and chronic bleeding disorders, respectively. During the production and storage of blood products, red blood cells and platelets release extracellular vesicles (EVs) as a result of the storage lesion, which may affect product quality...
February 5, 2024: Transfusion and Apheresis Science
https://read.qxmd.com/read/38281851/sex-differences-in-the-presentation-treatment-and-outcomes-of-patients-with-homozygous-familial-hypercholesterolemia
#12
JOURNAL ARTICLE
Zobaida Al-Baldawi, Leslie Brown, Isabelle Ruel, Alexis Baass, Jean Bergeron, Lubomira Cermakova, Patrick Couture, Daniel Gaudet, Gordon A Francis, Robert A Hegele, Iulia Iatan, G B John Mancini, Brian W McCrindle, Thomas Ransom, Mark H Sherman, Ruth McPherson, Jacques Genest, Liam R Brunham
BACKGROUND: Homozygous familial hypercholesterolemia (HoFH) is a rare, autosomal semi-dominant lipid metabolism disorder characterized by extremely high LDL-C levels and premature cardiovascular disease. The objective of this study was to investigate sex-differences in the treatment and outcomes of patients with HoFH. METHODS: We examined clinical characteristics, lipid-lowering therapy (LLT), and cardiovascular events using descriptive statistics of patients in the Canadian HoFH registry...
January 14, 2024: Journal of Clinical Lipidology
https://read.qxmd.com/read/38228097/application-of-lipid-apheresis-in-acute-lipogenic-pancreatitis
#13
JOURNAL ARTICLE
Yan Yu, Fang Yuan, Pan'ai Song, Cuifang Sun, Hao Xiong, Jie Song
INTRODUCTION: Recently, the incidence of hypertriglyceridemia-associated pancreatitis(HTG-AP) has been increasing. The pathogenesis of lipogenic pancreatitis is not fully understood.This study aimed to retrospectively analyze the laboratory data, clinical manifestations, and prognosis of patients with lipid-derived pancreatitis who received lipid purification,to explore whether lipid purification is a better treatment for acute hyperlipidemic pancreatitis . METHODS: In this study, we enrolled five subjects diagnosed with HTG-AP at the Second Xiangya Hospital of Central South University between 2021 and 2022...
January 16, 2024: Blood Purification
https://read.qxmd.com/read/38158247/modern-approaches-to-the-management-of-homozygous-familial-hypercholesterolemia-in-the-middle-east-and-north-africa
#14
REVIEW
Abdullah Al-Ashwal, Afaf Alsagheir, Mohammed Al Dubayee, Mutaz Al-Khnifsawi, Ahmed Al-Sarraf, Zuhier Awan, Tawfeg Ben-Omran, Saif Al-Yaarubi, Angham Almutair, Abdelhadi Habeb, Faouzi Maatouk, Manal Alshareef, Naji Kholaif, Dirk Blom
Homozygous familial hypercholesterolaemia (HoFH) is a severe form of FH in which inheritance of two defective or null mutations in genes associated with metabolism of low-density lipoprotein cholesterol (LDL-C) results in extremely high LDL-C, premature atherosclerotic cardiovascular disease (ASCVD) and mortality. Treatment of HoFH comprises a multi-modal approach of statins, ezetimibe, lipoprotein apheresis; and inhibitors of proprotein convertase subtilisin/kexin type, angiopoietin-like protein 3 (ANGPTL3) and microsomal triglyceride transfer protein...
December 15, 2023: Journal of Clinical Lipidology
https://read.qxmd.com/read/38155796/clinical-characteristics-of-homozygous-familial-hypercholesterolemia-in-japan-a-survey-using-a-national-database
#15
JOURNAL ARTICLE
Yasuaki Takeji, Hayato Tada, Masatsune Ogura, Atsushi Nohara, Masa-Aki Kawashiri, Shizuya Yamashita, Mariko Harada-Shiba
BACKGROUND: The studies evaluating patients' characteristics and lipid-lowering therapy for patients with homozygous familial hypercholesterolemia (HoFH) are scarce. OBJECTIVES: This study aims to evaluate the characteristics of and treatments for patients with HoFH. METHODS: This study included 201 patients who were diagnosed with definite or probable HoFH from the National Database of the Japanese Ministry of Health, Labour, and Welfare...
December 2023: JACC Asia
https://read.qxmd.com/read/38094739/peripartum-lipid-apheresis-novel-management-of-familial-hyperlipidemia-in-pregnancy
#16
Kayle Shapero, Malamo Countouris, Irina Chibisov, Arun Jeyabalan, Kathryn Berlacher
Familial hypercholesterolemia (FH) is a genetic lipid disorder associated with early-onset severe cardiovascular disease. Many FH therapeutics have not been studied in pregnancy, and management of patients with FH through pregnancy is limited. We present a patient with FH who was safely treated through pregnancy with combination therapy.
December 6, 2023: JACC. Case reports
https://read.qxmd.com/read/38092592/fatty-acid-analysis-in-serum-of-patients-with-elevated-lipoprotein-a-and-cardiovascular-disease-undergoing-lipoprotein-apheresis
#17
JOURNAL ARTICLE
Agnieszka Mickiewicz, Joanna Marlęga-Linert, Monika Czapiewska, Marta Marcinkowska, Aleksandra Krzesińska, Agnieszka Kuchta, Marcin Fijałkowski, Marcin Gruchała, Adriana Mika
BACKGROUND: Lipoprotein apheresis (LA) is an extracorporeal treatment that transiently reduces lipoprotein (a) by 60% and leads to an 80-92% reduction in major adverse cardiovascular events. LA has a significant impact on lipid profile in serum of patients with atherosclerotic cardiovascular disease. OBJECTIVE: To investigate the effects of LA on the composition of serum fatty acids (FAs), focusing on those which could have an impact on cardiovascular disease (CVD)...
November 28, 2023: Journal of Clinical Lipidology
https://read.qxmd.com/read/38087844/abnormal-carnitine-metabolism-in-hemodialysis-patients-on-different-anticoagulants
#18
JOURNAL ARTICLE
Kie Sekiguchi, Takaya Abe, Ei Shiomi, Daiki Ikarashi, Tomohiko Matsuura, Shigekatsu Maekawa, Renpei Kato, Mitsugu Kanehira, Ryo Takata, Jun Sugimura, Takashi Sekiguchi, Wataru Obara
INTRODUCTION: We aimed to determine whether unfractionated heparin (UH) and low molecular weight heparin (LH) contribute to aberrant carnitine metabolism in patients receiving hemodialysis. METHODS: The rate of increase in serum free fatty acids (FFAs) and the ratio of acylcarnitine to free carnitine (AC/FC) from before to after hemodialysis were determined in patients receiving UH and LH. Additionally, the effect of switching patients to UH from LH was examined...
December 12, 2023: Therapeutic Apheresis and Dialysis
https://read.qxmd.com/read/38016879/widespread-xanthomas-regression-by-personalized-lipid-lowering-therapy-in-heterozygous-familial-hypercholesterolemia
#19
JOURNAL ARTICLE
Francesco Sbrana, Beatrice Dal Pino, Federico Bigazzi, Tiziana Sampietro
"The lower, the better" is the recommended approach in the management of high LDL cholesterol. Unfortunately, this does not always achieve as in the case of a 69-year-old woman referred to our Institute for her lipid profile (LDL cholesterol 412mg/dl), bilateral xanthelasma and cutaneous xanthomas. With a maximized and personalized lipid-lowering therapies (rosuvastatin, ezetimibe, PCSK9i and lipoprotein apheresis), after only six months, the patient showed an impressive regression in her cutaneous xanthomas...
November 27, 2023: Clínica e Investigación en Arteriosclerosis
https://read.qxmd.com/read/38014132/clinical-practice-recommendations-on-lipoprotein-apheresis-for-children-with-homozygous-familial-hypercholesterolemia-an-expert-consensus-statement-from-erknet-and-espn
#20
M Doortje Reijman, D Meeike Kusters, Jaap W Groothoff, Klaus Arbeiter, Eldad J Dann, Lotte M de Boer, Sarah D de Ferranti, Antonio Gallo, Susanne Greber-Platzer, Jacob Hartz, Lisa C Hudgins, Daiana Ibarretxe, Meral Kayikcioglu, Reinhard Klingel, Genovefa D Kolovou, Jun Oh, R Nils Planken, Claudia Stefanutti, Christina Taylan, Albert Wiegman, Claus Peter Schmitt
Homozygous familial hypercholesterolaemia is a life-threatening genetic condition, which causes extremely elevated LDL-C levels and atherosclerotic cardiovascular disease very early in life. It is vital to start effective lipid- lowering treatment from diagnosis onwards. Even with dietary and current multimodal pharmaceutical lipid- lowering therapies, LDL-C treatment goals cannot be achieved in many children. Lipoprotein apheresis is an extracorporeal lipid-lowering treatment, which is well established since three decades, lowering serum LDL-C levels by more than 70% per session...
November 15, 2023: medRxiv
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