13,217 results match your criteria Hyperlipoproteinemia


Prevalence and Impact of Apolipoprotein E7 on LDL Cholesterol Among Patients With Familial Hypercholesterolemia.

Front Cardiovasc Med 2021 13;8:625852. Epub 2021 Apr 13.

Department of Cardiovascular Medicine, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan.

It has been suggested that a rare mutant apolipoprotein E7, APOE7 (p.Glu262Lys, p.Glu263Lys), has been identified to be associated with hyperlipoproteinemia in the general population. Read More

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Effect of lipoprotein on coronary stents expansion and its risk factors.

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2021 Mar;46(3):249-256

Department of Cardiology, Third Xiangya Hospital, Central South University, Changsha 410013, China.

Objectives: To analyze the effect of hyperlipoproteinemia (α) on immediate expansion after coronary stent implantation guided by intravascular ultrasound (IVUS).

Methods: A total of 160 patients (175 lesions) with coronary heart disease diagnosed by coronary artery angiography, who were performed percutaneous intervention guided by IVUS in the Department of Cardiology, Third Xiangya Hospital, Central South University, were enrolled retrospectively.According to the concentration of lipoproteina, the patients were divided into 2 groups: a hyperlipoproteinemia (α) group and a control group. Read More

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Role of PCSK9 Inhibitors in Patients with Familial Hypercholesterolemia.

Endocrinol Metab (Seoul) 2021 Apr 19;36(2):279-295. Epub 2021 Apr 19.

Faculty of Medicine, Macau University of Science and Technology, Macau, China.

Patients with familial hypercholesterolemia (FH) are at high or very high risk for cardiovascular disease. Those with heterozygous FH (HeFH) often do not reach low-density lipoprotein cholesterol (LDL-C) targets with statin and ezetimibe therapy, and those with homozygous FH (HoFH) usually require additional lipid-modifying therapies. Drugs that inhibit proprotein convertase subtilisin/kexin type 9 (PCSK9) offer a novel approach to reduce LDL-C. Read More

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Association of Vitamin D Receptor Gene Polymorphisms With the Evolution of MODY Diabetes: Study in Tunisian Patients.

Biol Res Nurs 2021 Apr 8:10998004211004770. Epub 2021 Apr 8.

Laboratory of Clinical Biology, Research Unit UR17SP01: Molecular Biology Applied to the Study of Hyperlipoproteinemias, Diabetes and Hormones, National Institute of Nutrition and Food Technology, Tunis, Tunisia.

Vitamin D (VD) cannot be considered as a true vitamin, but rather as a hormone, which exerts its action via a vitamin D receptor (VDR). Many genes have been shown to be involved in the evolution of diabetes in various populations, such as the gene. The aim of our study was to investigate if , , , polymorphisms of gene have an impact on MODY diabetes and its clinical aspects in a Tunisian population. Read More

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Efficacy and safety of volanesorsen in patients with multifactorial chylomicronaemia (COMPASS): a multicentre, double-blind, randomised, placebo-controlled, phase 3 trial.

Lancet Diabetes Endocrinol 2021 05 30;9(5):264-275. Epub 2021 Mar 30.

Department of Medicine, University California San Diego, La Jolla, CA, USA. Electronic address:

Background: Volanesorsen is an antisense oligonucleotide that targets hepatic apolipoprotein C-III synthesis and reduces plasma triglyceride concentration. The aim of this study was to explore the safety and efficacy of volanesorsen in patients with multifactorial chylomicronaemia syndrome.

Methods: The COMPASS trial was a randomised, placebo-controlled, double-blind, phase 3 study done at 38 international clinical sites in Canada, France, Germany, the Netherlands, UK, and USA. Read More

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[TREATMENT OF HOMOZYGOTES OF FAMILIAL HYPERCHOLESTEROLEMIA: RECOMMENDATIONS OF THE ISRAELI SOCIETY OF ATHEROSCLEROSIS].

Harefuah 2021 Mar;160(3):170-174

Center for Prevention and Treatment of Atherosclerosis, Hadassah Hebrew University Medical Center, Jerusalem, Israel.

Introduction: Familial hypercholesterolemia (FH) is an autosomal dominant genetic disorder caused by mutations affecting the function of the LDL receptor. In the Israeli population, the carrier heterozygote state is quite common, with the prevalence of 1:250, and the estimated prevalence of homozygote (hoFH) patients is 1:500,000. The life span of untreated hoFH patients is significantly shortened due to premature atherosclerosis and cardiovascular mortality. Read More

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Bempedoic Acid (Nexletol) for the Treatment of Hyperlipidemia and Familial Hypercholesterolemia.

Am Fam Physician 2021 03;103(6):377-378

University of South Florida/Morton Plant Mease Family Medicine Residency, Clearwater, FL, USA.

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A novel GPIHBP1 mutation related to familial chylomicronemia syndrome: A series of cases.

Atherosclerosis 2021 04 23;322:31-38. Epub 2021 Feb 23.

Laboratório de Biologia Molecular e Genômica, Departamento de Biologia Celular e Genética, Centro de Biociências, Universidade Federal Do Rio Grande Do Norte, Natal, RN, Brazil.

Background And Aims: GPIHBP1 is an accessory protein of lipoprotein lipase (LPL) essential for its functioning. Mutations in the GPIHBP1 gene cause a deficit in the action of LPL, leading to severe hypertriglyceridemia and increased risk for acute pancreatitis.

Methods: We describe twelve patients (nine women) with a novel homozygous mutation in intron 2 of the GPIHBP1 gene. Read More

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Familial Hypercholesterolemia: A Narrative Review on Diagnosis and Management Strategies for Children and Adolescents.

Vasc Health Risk Manag 2021 17;17:59-67. Epub 2021 Feb 17.

Department of Cardiology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan.

Familial hypercholesterolemia (FH) is a relatively common inherited disorder caused by deleterious mutation(s) in the low-density lipoprotein (LDL) receptor or its associated genes. Given its nature as a heritable disease, any useful screening scheme, including universal, and cascade screening, allows for the early identification of patients with FH. Another important aspect to note is that early diagnosis associated with appropriate treatment can promote better prognosis. Read More

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Establishing low-density lipoprotein apheresis tolerability in patients with prior anaphylactoid reactions to lipoprotein apheresis using magnesium sulfate.

J Clin Apher 2021 Feb 18. Epub 2021 Feb 18.

Eliaz Therapeutics, Santa Rosa, California, USA.

Background: Lipoprotein apheresis (LA) tolerability is a key factor for the utilization of this therapy. Common reactions to LA are hypotension and nausea. Serious reactions include severe hypotension and anaphylactoid reactions (0. Read More

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February 2021

Evinacumab for Homozygous Familial Hypercholesterolemia.

Authors:
Hua-Guo Xu

N Engl J Med 2021 02;384(6):e17

First Affiliated Hospital of Nanjing Medical University, Nanjing, China

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February 2021

Evinacumab for Homozygous Familial Hypercholesterolemia.

N Engl J Med 2021 02;384(6):e17

Second Xiangya Hospital of Central South University, Changsha, China

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February 2021

Evinacumab for Homozygous Familial Hypercholesterolemia.

Authors:
Mehmet Agirbasli

N Engl J Med 2021 02;384(6):e17

Istanbul Medeniyet University Medical School, Istanbul, Turkey

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February 2021

Serious flaws in targeting LDL-C reduction in the management of cardiovascular disease in familial hypercholesterolemia.

Expert Rev Clin Pharmacol 2021 03 22;14(3):405-406. Epub 2021 Feb 22.

Department of Psychology and Department of Molecular Pharmacology and Physiology, Center for Preclinical and Clinical Research on PTSD, University of South Florida, Tampa, FL, USA.

Recently, Polychronopoulos and Tziomalos reviewed research on the use of inclisiran and bempedoic acid in the management of cardiovascular disease (CVD) risk in people with familial hypercholesterolemia (FH). Their treatment recommendations were based on the general premise that high LDL-cholesterol (LDL-C) is inherently atherogenic, and that low levels of LDL-C need to be achieved to reduce CVD risk in FH individuals. However, their perspective on LDL-C is flawed at two levels of analysis: 1) They ignored the extensive literature demonstrating that CVD is not caused by high LDL-C; and 2) they failed to consider CVD treatment strategies that take into account the extensive literature that has shown that coagulation factors are more closely related to coronary events in FH than is LDL-C. Read More

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Impact of Diet on Plasma Lipids in Individuals with Heterozygous Familial Hypercholesterolemia: A Systematic Review of Randomized Controlled Nutritional Studies.

Nutrients 2021 Jan 15;13(1). Epub 2021 Jan 15.

Centre Nutrition, Santé et Société (NUTRISS), Institut Sur la Nutrition et les Aliments Fonctionnels (INAF), Université Laval, Québec, QC G1V 0A6, Canada.

Background: Conclusive data on the effectiveness of dietary interventions in heterozygous familial hypercholesterolemia (HeFH) management are unavailable. Whether this is due to a true lack of effects or biases in intervention designs remains unsettled. We systematically assessed the impact on LDL-C of published dietary randomized controlled trials (RCTs) conducted among individuals with HeFH in relation to their design and risk of bias. Read More

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January 2021

Dietary intake and lipid levels in Norwegian and Spanish children with familial hypercholesterolemia.

Nutr Metab Cardiovasc Dis 2021 04 3;31(4):1299-1307. Epub 2021 Feb 3.

Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway; Norwegian National Advisory Unit on Familial Hypercholesterolemia, Oslo University Hospital, Aker Hospital, Norway.

Background And Aims: Both the Nordic and Mediterranean diets claim to have a beneficial effect on lipid metabolism and cardiovascular prevention. The objective of this study was to compare diets consumed by children with FH at the time of diagnosis in Norway and Spain and to study their relationship with the lipid profile.

Methods And Results: In this cross-sectional study, we appraised the dietary intake in children (4-18 years old) with (n = 114) and without FH (n = 145) from Norway and Spain. Read More

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Analysis of steatosis biomarkers and inflammatory profile after adding on PCSK9 inhibitor treatment in familial hypercholesterolemia subjects with nonalcoholic fatty liver disease: A single lipid center real-world experience.

Nutr Metab Cardiovasc Dis 2021 03 13;31(3):869-879. Epub 2020 Nov 13.

Department of Clinical and Experimental Medicine, University of Catania, Italy.

Background And Aims: Nonalcoholic fatty liver disease (NAFLD) may be crucial in subjects with familial hypercholesterolemia (FH). We aimed to evaluate the effect of the inhibitors of proprotein convertase subtilisin/kexin type 9 (PCSK9-i) on steatosis biomarkers such as triglyceride-glucose index (TyG) and hepatic steatosis index (HSI) and analyse the role of TG/HDL in this population before and after adding-on PCSK9-i.

Methods And Results: In this observational study, we evaluated 26 genetically confirmed FH patients with NAFLD and an LDL-C off-target despite high-intensity statins plus ezetimibe. Read More

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Familial hypercholesterolaemia and COVID-19: A two-hit scenario for endothelial dysfunction amenable to treatment.

Atherosclerosis 2021 03 24;320:53-60. Epub 2021 Jan 24.

Wihuri Research Institute, Helsinki, Finland.

Patients with familial hypercholesterolemia (FH) are likely at increased risk for COVID-19 complications in the acute phase of the infection, and for a long time thereafter. Because in FH patients the level of low density lipoprotein cholesterol (LDL-C) is elevated from birth and it correlates with the degree of systemic endothelial dysfunction, both heterozygous FH (HeFH) patients and, in particular, homozygous FH (HoFH) patients have a dysfunctional endothelium prone to further damage by the direct viral attack and the hyper-inflammatory reaction typical of severe COVID-19. Evidence to date shows the benefit of statin use in patients with COVID-19. Read More

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Expert position statements: comparison of recommendations for the care of adults and youth with elevated lipoprotein(a).

Curr Opin Endocrinol Diabetes Obes 2021 04;28(2):159-173

The Department of Internal Medicine, University of Kansas Medical Center, Kansas City, Kansas, USA.

Purpose Of Review: Summarize recent recommendations on clinical management of adults and youth with elevated lipoprotein(a) [Lp(a)] who are at-risk of or affected by cardiovascular disease (CVD).

Recent Findings: There is ample evidence to support elevated Lp(a) levels, present in approximately 20% of the general population, as a causal, independent risk factor for CVD and its role as a significant risk enhancer. Several guidelines and position statements have been published to assist in the identification, treatment and follow-up of adults with elevated levels of Lp(a). Read More

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Lipoprotein(a), Immunity, and Inflammation in Polyvascular Atherosclerotic Disease.

J Cardiovasc Dev Dis 2021 Jan 27;8(2). Epub 2021 Jan 27.

Institute of Experimental Cardiology, National Medical Research Center of Cardiology, Ministry of Health of the Russian Federation, 121552 Moscow, Russia.

Background And Aims: lipoprotein(a) (Lp(a)) is a genetically determined risk factor for coronary artery disease and its complications, although data on the association with other vascular beds and the severity of atherosclerosis is limited. The aim of this study was to evaluate the association of atherosclerosis of various vascular beds with Lp(a), as well as its autoantibodies and generalized inflammatory markers.

Material And Methods: this study included 1288 adult patients with clinical and imaging examination of three vascular beds (coronary, carotid, and lower limb arteries). Read More

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January 2021

Lipoprotein(a) in familial hypercholesterolemia: Tips from family history.

Rev Port Cardiol 2021 03 24;40(3):225-227. Epub 2021 Jan 24.

Lipoapheresis Unit and Reference Center for Inherited Dyslipidemias, Fondazione Toscana Gabriele Monasterio, Pisa, Italy.

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Systemic diseases and the cornea.

Exp Eye Res 2021 03 21;204:108455. Epub 2021 Jan 21.

Eye Program, Board of Governors Regenerative Medicine Institute, Department of Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA, USA; Departments of Molecular and Medical Pharmacology, Medicine, and Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA. Electronic address:

There is a number of systemic diseases affecting the cornea. These include endocrine disorders (diabetes, Graves' disease, Addison's disease, hyperparathyroidism), infections with viruses (SARS-CoV-2, herpes simplex, varicella zoster, HTLV-1, Epstein-Barr virus) and bacteria (tuberculosis, syphilis and Pseudomonas aeruginosa), autoimmune and inflammatory diseases (rheumatoid arthritis, Sjögren's syndrome, lupus erythematosus, gout, atopic and vernal keratoconjunctivitis, multiple sclerosis, granulomatosis with polyangiitis, sarcoidosis, Cogan's syndrome, immunobullous diseases), corneal deposit disorders (Wilson's disease, cystinosis, Fabry disease, Meretoja's syndrome, mucopolysaccharidosis, hyperlipoproteinemia), and genetic disorders (aniridia, Ehlers-Danlos syndromes, Marfan syndrome). Corneal manifestations often provide an insight to underlying systemic diseases and can act as the first indicator of an undiagnosed systemic condition. Read More

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THE PREVALENCE OF PROBABLE FAMILIAL CHYLOMICRONEMIA SYNDROME IN A SOUTHERN CALIFORNIA POPULATION.

Endocr Pract 2021 Jan 18;27(1):71-76. Epub 2020 Nov 18.

Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, California. Electronic address:

Objective: To estimate the prevalence of probable familial chylomicronemia syndrome (FCS) in a major Southern California Academic Center as well as to provide a systematic review of past FCS studies and management recommendations.

Methods: Electronic medical records were queried based on single fasting plasma triglyceride (TG) levels of ≥880 mg/dL and at least 1 episode of acute pancreatitis. After the exclusion of secondary causes (diabetes, alcohol misuse, gallbladder disease, chronic kidney disease, uncontrolled hypothyroidism, estrogen, and drug use) and responses to lipid-lowering treatment, probable patients with FCS were identified. Read More

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January 2021

Supravalvular Aortic Stenosis and the Risk of Premature Death Among Patients With Homozygous Familial Hypercholesterolemia.

Am J Cardiol 2021 04 14;145:58-63. Epub 2021 Jan 14.

Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China. Electronic address:

Patients with homozygous familial hypercholesterolemia (HoFH) have a high risk for premature death. Supravalvular aortic stenosis (SVAS) is a common and the feature lesion of the aortic root in HoFH. The relation between SVAS and the risk of premature death in patients with HoFH has not been fully investigated. Read More

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Risk Factors in Patients with Non-ST Segment Elevation Myocardial Infarction.

Mater Sociomed 2020 Sep;32(3):224-226

Clinic for Invasive Cardiology, University Clinical Center Tuzla.

Introduction: The most common cause of myocardial ischemia is atherosclerotic epicardial coronary artery disease, present in 90% of patients. Risk factors positively correlate with the onset, development and subsequent complications of atherosclerotic disease.

Aim: Determine the percentage frequency of classic risk factors for coronary disease in patients with non-ST segment elevation myocardial infarction (NSTEMI), with regard to gender. Read More

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September 2020

Cardiovascular events in patients with familial hypercholesterolemia and hyperlipoproteinaemia (a): Indications for lipoprotein apheresis in Poland.

J Clin Apher 2021 Jan 2. Epub 2021 Jan 2.

Department of Cardiology I, Medical University of Gdansk, Gdansk, Poland.

Background: Lipoprotein apheresis (LA) is a safe method of reducing atherogenic lipoproteins and improving cardiovascular (CV) outcomes. We aimed to assess the reductions in low-density lipoprotein cholesterol (LDL-C) and lipoprotein (a) [Lp(a)] levels in patients undergoing regular LA therapy and to evaluate its influence on the incidence rate of adverse cardiac and vascular events (ACVE) and major adverse cardiac events (MACE).

Methods: A longitudinal study in Poland evaluated the prospective and retrospective observational data of 23 patients with hyperlipoproteinaemia (a) [hyper-Lp(a)] and familial hypercholesterolemia (FH), undergoing 1014 LA sessions between 2013 and 2020. Read More

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January 2021