Title : Management of familial hypercholesterolemia in children and young adults: Consensus paper developed by a panel of lipidologists, cardiologists, paediatricians, nutritionists, gastroenterologists, general practitioners and a patient organization Language : English Author, co-author : Descamps, O. S. [Departement de Médecine Interne et Centre de Recherche Médicale de Jolimont, Hôpital de Jolimont, Haine Saint-Paul, Belgium] Tenoutasse, S. [Endocrinologie pédiatrique, Hôpital Universitaire Des Enfants, Bruxelles, Belgium] Stephenne, X. [Unité de gastroentérologie pédiatrique, Cliniques Universitaires Saint-Luc, Bruxelles, Belgium] Gies, I. [Department of Pediatrics, UZ Brussel, Vrije Universiteit Brussel, Belgium] Beauloye, V. [Endocrinologie pédiatrique, Cliniques universitaires Saint-Luc, Bruxelles, Belgium] Lebrethon, M.-C. [C.H.U de Liège-site N.D des Bruyères, Chênée, Belgium] De Beaufort, Carine [University of Luxembourg > Luxembourg Centre for Systems Biomedicine (LCSB) >] De Waele, K. [Department of Pediatry, Ghent University Hospital, Ghent, Belgium] Scheen, A. [Division of Diabetes, Nutrition and Metabolic Disorders, CHU Sart Tilman, University of Liege, Belgium] Rietzschel, E. [Department of Cardiovascular Disease, Ghent University Hospital, Ghent, Belgium] Mangano, A. [Belgian Patient Association for Familial Hypercholesterolemia, Belgium] Panier, J. P. [Belgian Patient Association for Familial Hypercholesterolemia, Belgium] Ducobu, J. [Université de Mons, Belgium] Langlois, M. [Department of Laboratory Medicine, AZ St-Jan Bruges, Belgium] Balligand, J.-L. [Département de Médecine Interne, Cliniques universitaires Saint-Luc, Bruxelles, Belgium] Legat, P. [Société Scientifique de Médecine Générale, PromoSanté and Médecine Générale asbl, Bruxelles, Belgium] Blaton, V. [Université de Mons, Belgium] Muls, E. [Department of Endocrinology-Nutrition, University Hospital Gasthuisberg, Leuven, Belgium] Van Gaal, L. [Department of Endocrinology, Diabetology and Clinical Pharmacology, Antwerp University Hospital, Belgium] Sokal, E. [Unité de gastroentérologie pédiatrique, Cliniques Universitaires Saint-Luc, Bruxelles, Belgium] Rooman, R. [Department of Pediatric Endocrinology-Diabetology, Antwerp University Hospital, Belgium] Carpentier, Y. [Laboratory of Experimental Surgery, Université Libre de Bruxelles, Belgium] De Backer, G. [Department of Cardiovascular Disease, Ghent University Hospital, Ghent, Belgium] Heller, F. R. [Departement de Médecine Interne et Centre de Recherche Médicale de Jolimont, Hôpital de Jolimont, Haine Saint-Paul, Belgium] Publication date : 2011 Journal title : Atherosclerosis Volume : 218 Issue/season : 2 Pages : 272-280 Peer reviewed : Yes (verified by ORBilu ) ISSN : 00219150 Keywords : [en] Apolipoprotein b ; Cardiovascular disease ; Children ; Cholesterol ; Diagnostic criteria ; Ezetimibe ; Familial hypercholesterolemia ; Fibrates ; LDL-receptor gene ; Plant sterols ; Stanols ; Statins ; Treatment consensus paper ; Adult ; Cardiology ; Child ; Consensus Development Conferences as Topic ; Decision Making ; Female ; Gastroenterology ; General Practice ; Guidelines as Topic ; Heterozygote ; Humans ; Hyperlipoproteinemia Type II ; Lipids ; Male ; Nutritional Sciences ; Pediatrics ; Young AdultAbstract : [en] Since heterozygous familial hypercholesterolemia (HeFH) is a disease that exposes the individual from birth onwards to severe hypercholesterolemia with the development of early cardiovascular disease, a clear consensus on the management of this disease in young patients is necessary. In Belgium, a panel of paediatricians, specialists in (adult) lipid management, general practitioners and representatives of the FH patient organization agreed on the following common recommendations.1.Screening for HeFH should be performed only in children older than 2 years when HeFH has been identified or is suspected (based on a genetic test or clinical criteria) in one parent.2.The diagnostic procedure includes, as a first step, the establishment of a clear diagnosis of HeFH in one of the parents. If this precondition is satisfied, a low-density-lipoprotein cholesterol (LDL-C) level above 3.5mmol/L (135mg/dL) in the suspected child is predictive for differentiating affected from non-affected children.3.A low saturated fat and low cholesterol diet should be started after 2 years, under the supervision of a dietician or nutritionist.4.The pharmacological treatment, using statins as first line drugs, should usually be started after 10 years if LDL-C levels remain above 5mmol/L (190mg/dL), or above 4mmol/L (160mg/dL) in the presence of a causative mutation, a family history of early cardiovascular disease or severe risk factors. The objective is to reduce LDL-C by at least 30% between 10 and 14 years and, thereafter, to reach LDL-C levels of less than 3.4mmol/L (130mg/dL).Conclusion: The aim of this consensus statement is to achieve more consistent management in the identification and treatment of children with HeFH in Belgium. © 2011 Elsevier Ireland Ltd.Permalink : http://hdl.handle.net/10993/27178 DOI : 10.1016/j.atherosclerosis.2011.06.016 
[University of Luxembourg > Luxembourg Centre for Systems Biomedicine (LCSB) >]
