Study of cardiovAscular Contrasted Phenotypes in Patients With FamIliaI hypercholesteRolemia
Homozygous Familial HypercholesterolemiaThe main objective of SAFIR is to identify the atherosclerotic genetic factors in these patients, which will identify new therapeutic targets for the treatment of CV and Familial Hypercholesterolemia diseases. In addition, SAFIR will allow the identification of new CV protection biomarkers, which will be useful tools for the development of a personalized medicine for the management of dyslipidemias.
Safety and Efficacy of the DALI LDL-adsorber and MONET Lipoprotein Filter
HypercholesterolemiaFamilial1 moreLow Density Lipoprotein (LDL)-apheresis refers to a procedure in which blood taken from a patient's vein is cleaned from pathogenic substances, e.g. cholesterol, outside the body and then given back to the patient. In the DALI (Direct Adsorption of Lipoproteins)-system whole blood is pumped over an adsorber containing beads that selectively bind LDL-cholesterol. The MONET (Membrane filtration Optimized Novel Extracorporeal Treatment)-system works with plasma which is cleaned by filtration. This study comprises the recording of safety and efficacy data from patients treated either with the DALI or MONET-system over a period of 2 years.
Long Term Post Marketing Specified Drug Use Result Survey for Evolocumab in Japan
HypercholesterolemiaFamilial HypercholesterolemiaThe purpose of this post-marketing survey is to obtain real-world information on the safety and effectiveness of evolocumab in Japan.
A 12-week Post-marketing, Observational Study to Confirm the Safety and Efficacy of Zetia Alone...
HypercholesterolemiaFamilial Hypercholesterolemia1 moreThis study is a non-interventional (observational) study in Japan to confirm the safety and efficacy of Zetia when administered alone or in combination with other lipid-lowering drugs in daily medical practice throughout a 12-week period. It is being conducted as a post-approval commitment, in accordance with the Ministry of Health, Labour and Welfare's guideline on Good Post-marketing Study Practice. Post-marketing surveys are not considered applicable clinical trials and thus the results of this survey will not be posted at its conclusion. The results will be submitted to public health officials as required by applicable national and international laws.
Hyperapo B and Coronary Heart Disease
Cardiovascular DiseasesCoronary Disease5 moreTo determine the role of apolipoprotein B and apolipoprotein A1 in the etiology of coronary artery disease.
French Observatory of Familial Hypercholesterolemia in Cardiology
Familial HypercholesterolemiaThe Family Hypercholesterolemia remains poorly diagnosed disease with an outlet sometimes suboptimal care. However, the Family Hypercholesterolemia exposes patients concerned at increased cardiovascular risk. The frequency of familial hypercholesterolemia in cardiologic is little studied and remains unknown, and there is little data on the profile of patients, diagnostic methods and management. Main objectives: Establish a monitoring patients with hypercholesterolemia Family cardiology in France Characterize the Family hypercholesterolemia in cardiology, including assessing the frequency of the most severe forms, which are at higher cardiovascular risk.
Evaluation of Chylomicrons Metabolism in Sub-Clinical Atherosclerosis in Patients Whit Heterozigous...
Heterozigous Familial HypercholesterolemiaStudy Title: Evaluation of chylomicrons metabolism in sub-clinical atherosclerosis in patients whit Heterozigous Familial Hypercholesterolemia (FH) treated with statin plus ezetimibe. Background: Coronary artery calcification (CAC) is a marker of sub-clinical coronary atherosclerosis which correlates with higher risk of clinical events. It was already demonstrated that CAC is more prevalent in patients with FH compared with normal individuals. A number of studies demonstrated that plasmatic removal of chylomicrons is defective in patients with atherosclerosis. Despite the fact that is still controversial whether this impairment occurs in patients with HF when compared to normal controls, the kinetics of chylomicrons has not been studied in HF patients with and without atherosclerosis and more important, it is not clear if those changes may be observed in the sub-clinical disease, as reported for CAC in asymptomatic individuals. Previous studies have demonstrated the inverse correlation among LDL-C levels and the removal of remnants chylomicrons using artificial chylomicrons technique. It is also well known that high doses of more potent statins are more effective to remove chylomicrons from the plasma due to better expression of LDL-C receptors through plasma LDL-C reduction. It was not evaluated yet if the association of ezetimibe and statin, enhancing LDL-C receptors expression in the liver would enhance the efficacy of the monotherapy with statins to remove artificial chylomicrons in patients with HF. Study design: Open, randomized, single-blinded study in which twenty six outpatients from the Lipids Clinical Unit at the Heart Institute (INCOR), University of São Paulo, previously diagnosed with FH according to US MED PED criteria, without history of CD and a CAC evaluation by MSCT (Multiple Sensors Computed Tomography) in the previous year will be compared to 26 control individuals matched by age and sex collected from the database of the Lipids Metabolism Laboratory. Patients will be randomized to receive simvastatin 40 mg as monotherapy or in combination with ezetimibe 10 mg and will undergoing three kinetics studies to demonstrate the effects of simvastatin 40 mg on the kinetics of the chylomicrons along with other laboratorial dosages ( lipid fractions, hepatic enzymes and CK). The primary endpoint of this study is to evaluate if there is any correlation among the reduction of the plasma clearance of chylomicrons by the artificial chylomicrons technique and the presence of sub-clinical atherosclerosis; the secondary endpoint is to evaluate if ezetimibe/simvastatin enhances the effects of simvastatin alone in the removal of chylomicrons in patients with HF.
Biomarker for Homozygous Familial Hypercholesterolemia (BioHoFH)
Lipoprotein Lipase DeficiencyInborn Error of Lipid Metabolism1 moreDevelopment of a new MS-based biomarker for the early and sensitive diagnosis of Homozygous familial Hypercholesterolemia from blood
Identifying and Genotyping Homozygous Familial Hypercholesterolemia (HoFH) Patients
Homozygous Familial Hypercholesterolemia (HoFH)This study is designed to help identify patients with HoFH due to mutations in the LDLR as confirmed by genotyping.
Equitable Implementation of Cascade Screening for Familial Hypercholesterolemia
Familial HypercholesterolemiaDesign, refine, and pilot the two implementation approaches using behavioral economics and then seek further feedback prior to the proposed clinical trial, consistent with these recommendations. Aim 1. Co-design both implementation strategies using behavioral economics in partnership with the Family Heart Foundation and key partners from diverse backgrounds. Aim 2. Pilot strategies with 20 patients with high cholesterol and/or with familial hypercholesterolemia (FH) to ascertain feasibility, acceptability, appropriateness.