J 2020

Comparison of the characteristics at diagnosis and treatment of children with heterozygous familial hypercholesterolaemia (FH) from eight European countries

RAMASWAMI, U., M. FUTEMA, M. P. BOGSRUD, K. B. HOLVEN, J. R. VAN LENNEP et. al.

Basic information

Original name

Comparison of the characteristics at diagnosis and treatment of children with heterozygous familial hypercholesterolaemia (FH) from eight European countries

Authors

RAMASWAMI, U. (826 United Kingdom of Great Britain and Northern Ireland), M. FUTEMA (826 United Kingdom of Great Britain and Northern Ireland), M. P. BOGSRUD (578 Norway), K. B. HOLVEN (578 Norway), J. R. VAN LENNEP (528 Netherlands), A. WIEGMAN (528 Netherlands), O. S. DESCAMPS (56 Belgium), M. VRABLIK (203 Czech Republic), Tomáš FREIBERGER (203 Czech Republic, belonging to the institution), H. DIEPLINGER (40 Austria), S. GREBER-PLATZER (40 Austria), G. HANAUER-MADER (40 Austria), M. BOURBON (620 Portugal), E. DROGARI (300 Greece) and S. E. HUMPHRIES (826 United Kingdom of Great Britain and Northern Ireland, guarantor)

Edition

Atherosclerosis, Clare, ELSEVIER SCI IRELAND LTD, 2020, 0021-9150

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30201 Cardiac and Cardiovascular systems

Country of publisher

Ireland

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

Impact factor

Impact factor: 5.162

RIV identification code

RIV/00216224:14110/20:00115978

Organization unit

Faculty of Medicine

DOI

UT WoS

000503400300024

Keywords in English

Heterozygous familial hypercholesterolaemia; Paediatric FH; LDL-C concentrations; Statin treatment

Tags

Tags

International impact, Reviewed
Změněno: 15/7/2020 08:10, Mgr. Tereza Miškechová

Abstract

V originále

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.