This JCL Roundtable discussion probes the knowledge of 3 experts in pediatric lipidology, an emerging discipline both in the United States and internationally. In the 1990s, only 3 US institutions could be said to have dedicated pediatric lipid clinics; that number has grown to 25 today. The Pediatric Atherosclerosis Prevention and Lipidology Group of the National Lipid Association has regular teleconferences to support advocacy and convey best practices. Guidelines for pediatric lipidology initially focused on low-density lipoprotein cholesterol in 1992 as part of the National Cholesterol Education Program. Today the most comprehensive coverage comes from the 2011 National Heart Lung and Blood Institute Pediatric Guidelines. Universal screening was recommended for children between ages 9 and 11 years and teenagers/young adults between 17 to 21 years, a position echoed as "may be recommended" by the 2018 AHA/ACC/Multisociety Cholesterol Guidelines. While pediatric lipidologists continue to treat uncommon genetic disorders, they increasingly confront an issue of epidemic proportions-dyslipidemia as the initial presentation of metabolic dysregulation associated with obesity. Consequences of such altered metabolism extend to atherosclerosis, diabetes, liver disease, and other serious problems in adult life. Pediatric lipid science and practice differ from adult experience in several ways, including importance of family and birth history as well as genetics/epigenetics, lack of general pediatricians' familiarity with lipid drugs, value of family counseling, need for biomarkers of early metabolic dysregulation, and anticipation of endpoints in adult life not fully defined by randomized clinical trials in children.
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http://dx.doi.org/10.1016/j.jacl.2019.10.010 | DOI Listing |
Eur Heart J
January 2025
Department of Primary Care and Public Health, Imperial Centre for Cardiovascular Disease Prevention, School of Public Health, Imperial College London, White City Campus, 90 Wood Lane, London W12 0BZ, UK.
Background And Aims: Overweight and obesity are modifiable risk factors for atherosclerotic cardiovascular disease (ASCVD) in the general population, but their prevalence in individuals with heterozygous familial hypercholesterolaemia (HeFH) and whether they confer additional risk of ASCVD independent of LDL cholesterol (LDL-C) remains unclear.
Methods: Cross-sectional analysis was conducted in 35 540 patients with HeFH across 50 countries, in the EAS FH Studies Collaboration registry. Prevalence of World Health Organization-defined body mass index categories was investigated in adults (n = 29 265) and children/adolescents (n = 6275); and their association with prevalent ASCVD.
Eur J Prev Cardiol
November 2024
Clinical Lipidology and Rare Lipid Disorders Unit, Community Genomic Medicine Centre and ECOGENE-21, Department of Medicine, Université de Montréal, Chicoutimi, Quebec, Canada.
Aim: Children with heterozygous familial hypercholesterolaemia (HeFH) show greater carotid intima-media thickness (cIMT). Evolocumab, a proprotein convertase subtilisin/kexin type 9 inhibitor monoclonal antibody, substantially reduced low-density lipoprotein cholesterol (LDL-C) and modestly reduced lipoprotein(a) in children with HeFH. We investigated evolocumab's effect on cIMT progression.
View Article and Find Full Text PDFAtherosclerosis
December 2024
Paris Cité University and Sorbonne Paris Nord University, INSERM UMRS 1148, Laboratory for Vascular Translational Science (LVTS), F-75018, Paris, France. Electronic address:
Eur Heart J
July 2024
Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
Background And Aims: Homozygous familial hypercholesterolaemia (HoFH) is a rare genetic disorder characterized by severely elevated LDL cholesterol (LDL-C) and premature atherosclerotic cardiovascular disease. In the pivotal Phase 3 HoFH trial (NCT03399786), evinacumab significantly decreased LDL-C in patients with HoFH. This study assesses the long-term safety and efficacy of evinacumab in adult and adolescent patients with HoFH.
View Article and Find Full Text PDFLancet Child Adolesc Health
July 2024
Department of Paediatrics, Amsterdam Gastroenterology Endocrinology Metabolism, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands.
Background: Homozygous familial hypercholesterolaemia (HoFH) is a rare genetic disease characterised by extremely high plasma LDL cholesterol from birth, causing atherosclerotic cardiovascular disease at a young age. Lipoprotein apheresis in combination with lipid-lowering drugs effectively reduce LDL cholesterol, but long-term health outcomes of such treatment are unknown. We aimed to investigate the long-term cardiovascular outcomes associated with lipoprotein apheresis initiated in childhood or adolescence.
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