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Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel

Heterozygous familial hypercholesterolaemia (FH) is among the most common genetic metabolic lipid disorders characterised by elevated low-density lipoprotein cholesterol (LDL-C) levels from birth and a significantly higher risk of developing premature atherosclerotic cardiovascular disease. The majo...

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Autores principales: Bjelakovic, Bojko, Stefanutti, Claudia, Reiner, Željko, Watts, Gerald F., Moriarty, Patrick, Marais, David, Widhalm, Kurt, Cohen, Hofit, Harada-Shiba, Mariko, Banach, Maciej
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8585021/
https://www.ncbi.nlm.nih.gov/pubmed/34768450
http://dx.doi.org/10.3390/jcm10214930
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author Bjelakovic, Bojko
Stefanutti, Claudia
Reiner, Željko
Watts, Gerald F.
Moriarty, Patrick
Marais, David
Widhalm, Kurt
Cohen, Hofit
Harada-Shiba, Mariko
Banach, Maciej
author_facet Bjelakovic, Bojko
Stefanutti, Claudia
Reiner, Željko
Watts, Gerald F.
Moriarty, Patrick
Marais, David
Widhalm, Kurt
Cohen, Hofit
Harada-Shiba, Mariko
Banach, Maciej
author_sort Bjelakovic, Bojko
collection PubMed
description Heterozygous familial hypercholesterolaemia (FH) is among the most common genetic metabolic lipid disorders characterised by elevated low-density lipoprotein cholesterol (LDL-C) levels from birth and a significantly higher risk of developing premature atherosclerotic cardiovascular disease. The majority of the current pediatric guidelines for clinical management of children and adolescents with FH does not consider the impact of genetic variations as well as characteristics of vascular phenotype as assessed by recently developed non-invasive imaging techniques. We propose a combined integrated approach of cardiovascular (CV) risk assessment and clinical management of children with FH incorporating current risk assessment profile (LDL-C levels, traditional CV risk factors and familial history) with genetic and non-invasive vascular phenotyping. Based on the existing data on vascular phenotype status, this panel recommends that all children with FH and cIMT ≥0.5 mm should receive lipid lowering therapy irrespective of the presence of CV risk factors, family history and/or LDL-C levels Those children with FH and cIMT ≥0.4 mm should be carefully monitored to initiate lipid lowering management in the most suitable time. Likewise, all genetically confirmed children with FH and LDL-C levels ≥4.1 mmol/L (160 mg/dL), should be treated with lifestyle changes and LLT irrespective of the cIMT, presence of additional RF or family history of CHD.
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spelling pubmed-85850212021-11-12 Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel Bjelakovic, Bojko Stefanutti, Claudia Reiner, Željko Watts, Gerald F. Moriarty, Patrick Marais, David Widhalm, Kurt Cohen, Hofit Harada-Shiba, Mariko Banach, Maciej J Clin Med Review Heterozygous familial hypercholesterolaemia (FH) is among the most common genetic metabolic lipid disorders characterised by elevated low-density lipoprotein cholesterol (LDL-C) levels from birth and a significantly higher risk of developing premature atherosclerotic cardiovascular disease. The majority of the current pediatric guidelines for clinical management of children and adolescents with FH does not consider the impact of genetic variations as well as characteristics of vascular phenotype as assessed by recently developed non-invasive imaging techniques. We propose a combined integrated approach of cardiovascular (CV) risk assessment and clinical management of children with FH incorporating current risk assessment profile (LDL-C levels, traditional CV risk factors and familial history) with genetic and non-invasive vascular phenotyping. Based on the existing data on vascular phenotype status, this panel recommends that all children with FH and cIMT ≥0.5 mm should receive lipid lowering therapy irrespective of the presence of CV risk factors, family history and/or LDL-C levels Those children with FH and cIMT ≥0.4 mm should be carefully monitored to initiate lipid lowering management in the most suitable time. Likewise, all genetically confirmed children with FH and LDL-C levels ≥4.1 mmol/L (160 mg/dL), should be treated with lifestyle changes and LLT irrespective of the cIMT, presence of additional RF or family history of CHD. MDPI 2021-10-25 /pmc/articles/PMC8585021/ /pubmed/34768450 http://dx.doi.org/10.3390/jcm10214930 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Bjelakovic, Bojko
Stefanutti, Claudia
Reiner, Željko
Watts, Gerald F.
Moriarty, Patrick
Marais, David
Widhalm, Kurt
Cohen, Hofit
Harada-Shiba, Mariko
Banach, Maciej
Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel
title Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel
title_full Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel
title_fullStr Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel
title_full_unstemmed Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel
title_short Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel
title_sort risk assessment and clinical management of children and adolescents with heterozygous familial hypercholesterolaemia. a position paper of the associations of preventive pediatrics of serbia, mighty medic and international lipid expert panel
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8585021/
https://www.ncbi.nlm.nih.gov/pubmed/34768450
http://dx.doi.org/10.3390/jcm10214930
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