Τίτλος:
Comparison of the characteristics at diagnosis and treatment of children with heterozygous familial hypercholesterolaemia (FH) from eight European countries
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Background and aims: For children with heterozygous familial hypercholesterolaemia (HeFH), European guidelines recommend consideration of statin therapy by age 8–10 years for those with a low density lipoprotein cholesterol (LDL-C) >3.5 mmol/l, and dietary and lifestyle advice. Here we compare the characteristics and lipid levels in HeFH children from Norway, UK, Netherlands, Belgium, Czech Republic, Austria, Portugal and Greece. Methods: Fully-anonymized data were analysed at the London centre. Differences in registration and on treatment characteristics were compared by standard statistical tests. Results: Data was obtained from 3064 children. The median age at diagnosis differed significantly between countries (range 3–11 years) reflecting differences in diagnostic strategies. Mean (SD) LDL-C at diagnosis was 5.70 (±1.4) mmol/l, with 88% having LDL-C>4.0 mmol/l. The proportion of children older than 10 years at follow-up who were receiving statins varied significantly (99% in Greece, 56% in UK), as did the proportion taking Ezetimibe (0% in UK, 78% in Greece). Overall, treatment reduced LDL-C by between 28 and 57%, however, in those >10 years, 23% of on-treatment children still had LDL-C>3.5 mmol/l and 66% of those not on a statin had LDL-C>3.5 mmol/l. Conclusions: The age of HeFH diagnosis in children varies significantly across 8 countries, as does the proportion of those >10 years being treated with statin and/or ezetimibe. Approximately a quarter of the treated children and almost three quarters of the untreated children older than 10 years still have LDL-C concentrations over 3.5 mmol/l. These data suggest that many children with FH are not receiving the full potential benefit of early identification and appropriate lipid-lowering treatment according to recommendations. © 2019 The Authors
Συγγραφείς:
Ramaswami, U.
Futema, M.
Bogsrud, M.P.
Holven, K.B.
Roeters van Lennep, J.
Wiegman, A.
Descamps, O.S.
Vrablik, M.
Freiberger, T.
Dieplinger, H.
Greber-Platzer, S.
Hanauer-Mader, G.
Bourbon, M.
Drogari, E.
Humphries, S.E.
Περιοδικό:
Atherosclerosis
Εκδότης:
Elsevier Ireland Ltd
Λέξεις-κλειδιά:
apolipoprotein B; ezetimibe; high density lipoprotein cholesterol; hydroxymethylglutaryl coenzyme A reductase inhibitor; lipid; low density lipoprotein cholesterol; low density lipoprotein receptor; proprotein convertase 9; triacylglycerol; low density lipoprotein cholesterol, adolescent; Article; Austria; Belgium; child; comparative study; Czech Republic; familial hypercholesterolemia; female; follow up; Greece; heterozygote; human; lipid level; major clinical study; male; medical record review; Netherlands; Norway; Portugal; prevalence; priority journal; retrospective study; total cholesterol level; triacylglycerol level; United Kingdom; blood; Europe; familial hypercholesterolemia; genetics; preschool child, Child; Child, Preschool; Cholesterol, LDL; Europe; Female; Heterozygote; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Hyperlipoproteinemia Type II; Male; Retrospective Studies
DOI:
10.1016/j.atherosclerosis.2019.11.012
