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:	Bojko Bjelakovic, Claudia Stefanutti, Željko Reiner, Gerald F. Watts, Patrick Moriarty, David Marais, Kurt Widhalm, Hofit Cohen, Mariko Harada-Shiba, Maciej Banach
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	familial hypercholesterolaemia children cardiovascular risk vascular phenotype Medicine R
Acceso en línea:	https://doaj.org/article/a550713b086740e59606f49fcc9b6b6f
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spelling	oai:doaj.org-article:a550713b086740e59606f49fcc9b6b6f2021-11-11T17:34:04ZRisk 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 Panel10.3390/jcm102149302077-0383https://doaj.org/article/a550713b086740e59606f49fcc9b6b6f2021-10-01T00:00:00Zhttps://www.mdpi.com/2077-0383/10/21/4930https://doaj.org/toc/2077-0383Heterozygous 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.Bojko BjelakovicClaudia StefanuttiŽeljko ReinerGerald F. WattsPatrick MoriartyDavid MaraisKurt WidhalmHofit CohenMariko Harada-ShibaMaciej BanachMDPI AGarticlefamilial hypercholesterolaemiachildrencardiovascular riskvascular phenotypeMedicineRENJournal of Clinical Medicine, Vol 10, Iss 4930, p 4930 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	familial hypercholesterolaemia children cardiovascular risk vascular phenotype Medicine R
spellingShingle	familial hypercholesterolaemia children cardiovascular risk vascular phenotype Medicine R Bojko Bjelakovic Claudia Stefanutti Željko Reiner Gerald F. Watts Patrick Moriarty David Marais Kurt Widhalm Hofit Cohen Mariko Harada-Shiba Maciej Banach 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
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.
format	article
author	Bojko Bjelakovic Claudia Stefanutti Željko Reiner Gerald F. Watts Patrick Moriarty David Marais Kurt Widhalm Hofit Cohen Mariko Harada-Shiba Maciej Banach
author_facet	Bojko Bjelakovic Claudia Stefanutti Željko Reiner Gerald F. Watts Patrick Moriarty David Marais Kurt Widhalm Hofit Cohen Mariko Harada-Shiba Maciej Banach
author_sort	Bojko Bjelakovic
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_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_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_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
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/a550713b086740e59606f49fcc9b6b6f
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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

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