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Familial hypercholesterolaemia in children and adolescents: gaining decades of life by optimizing detection and treatment

Wiegman, Albert ; Gidding, Samuel S ; Watts, Gerald F ; Chapman, M John ; Ginsberg, Henry N ; Cuchel, Marina ; Ose, Leiv ; Averna, Maurizio ; Boileau, Catherine ; Borén, Jan ; Bruckert, Eric ; Catapano, Alberico L ; Defesche, Joep C ; Descamps, Olivier S ; Hegele, Robert A ; Hovingh, G Kees ; Humphries, Steve E ; Kovanen, Petri T ; Kuivenhoven, Jan Albert ; Masana, Luis ; Nordestgaard, Børge G ; Pajukanta, Päivi ; Parhofer, Klaus G ; Raal, Frederick J ; Ray, Kausik K ; Santos, Raul D ; Stalenhoef, Anton F H ; Steinhagen-Thiessen, Elisabeth ; Stroes, Erik S ; Taskinen, Marja-Riitta ; Tybjærg-Hansen, Anne ; Wiklund, Olov

European heart journal, 2015-09, Vol.36 (36), p.2425-2437 [Periódico revisado por pares]

England: Oxford University Press

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Título:
Familial hypercholesterolaemia in children and adolescents: gaining decades of life by optimizing detection and treatment
Autor: Wiegman, Albert ; Gidding, Samuel S ; Watts, Gerald F ; Chapman, M John ; Ginsberg, Henry N ; Cuchel, Marina ; Ose, Leiv ; Averna, Maurizio ; Boileau, Catherine ; Borén, Jan ; Bruckert, Eric ; Catapano, Alberico L ; Defesche, Joep C ; Descamps, Olivier S ; Hegele, Robert A ; Hovingh, G Kees ; Humphries, Steve E ; Kovanen, Petri T ; Kuivenhoven, Jan Albert ; Masana, Luis ; Nordestgaard, Børge G ; Pajukanta, Päivi ; Parhofer, Klaus G ; Raal, Frederick J ; Ray, Kausik K ; Santos, Raul D ; Stalenhoef, Anton F H ; Steinhagen-Thiessen, Elisabeth ; Stroes, Erik S ; Taskinen, Marja-Riitta ; Tybjærg-Hansen, Anne ; Wiklund, Olov
Assuntos: Adolescent ; Adult ; Atherosclerosis - diagnosis ; Atherosclerosis - drug therapy ; Cardiac and Cardiovascular Systems ; Carotid Intima-Media Thickness ; Child ; Clinical Laboratory Techniques - methods ; Cost of Illness ; Counseling ; Diet ; Dietary Supplements ; Early Diagnosis ; Economics, Medical ; Evidence-Based Medicine ; Female ; Genetic Testing ; Heterozygote ; Homozygote ; Humans ; Hyperlipoproteinemia Type II - diagnosis ; Hyperlipoproteinemia Type II - drug therapy ; Hyperlipoproteinemia Type II - genetics ; Kardiologi ; Life Expectancy ; Medication Adherence ; Middle Aged ; Pregnancy ; Pregnancy Complications - etiology ; Reviews ; Risk Factors ; Young Adult
É parte de: European heart journal, 2015-09, Vol.36 (36), p.2425-2437
Notas: ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-3
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ObjectType-Review-1
ObjectType-Article-1
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For list of Panel members, see Appendix.
Equal first authors.
Descrição: Familial hypercholesterolaemia (FH) is a common genetic cause of premature coronary heart disease (CHD). Globally, one baby is born with FH every minute. If diagnosed and treated early in childhood, individuals with FH can have normal life expectancy. This consensus paper aims to improve awareness of the need for early detection and management of FH children. Familial hypercholesterolaemia is diagnosed either on phenotypic criteria, i.e. an elevated low-density lipoprotein cholesterol (LDL-C) level plus a family history of elevated LDL-C, premature coronary artery disease and/or genetic diagnosis, or positive genetic testing. Childhood is the optimal period for discrimination between FH and non-FH using LDL-C screening. An LDL-C ≥5 mmol/L (190 mg/dL), or an LDL-C ≥4 mmol/L (160 mg/dL) with family history of premature CHD and/or high baseline cholesterol in one parent, make the phenotypic diagnosis. If a parent has a genetic defect, the LDL-C cut-off for the child is ≥3.5 mmol/L (130 mg/dL). We recommend cascade screening of families using a combined phenotypic and genotypic strategy. In children, testing is recommended from age 5 years, or earlier if homozygous FH is suspected. A healthy lifestyle and statin treatment (from age 8 to 10 years) are the cornerstones of management of heterozygous FH. Target LDL-C is <3.5 mmol/L (130 mg/dL) if >10 years, or ideally 50% reduction from baseline if 8-10 years, especially with very high LDL-C, elevated lipoprotein(a), a family history of premature CHD or other cardiovascular risk factors, balanced against the long-term risk of treatment side effects. Identifying FH early and optimally lowering LDL-C over the lifespan reduces cumulative LDL-C burden and offers health and socioeconomic benefits. To drive policy change for timely detection and management, we call for further studies in the young. Increased awareness, early identification, and optimal treatment from childhood are critical to adding decades of healthy life for children and adolescents with FH.
Editor: England: Oxford University Press
Idioma: Inglês

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Familial hypercholesterolaemia in children and adolescents: gaining decades of life by optimizing detection and treatment

England: Oxford University Press

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