دورية أكاديمية

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

التفاصيل البيبلوغرافية
العنوان: Comparison of the characteristics at diagnosis and treatment of children with heterozygous familial hypercholesterolaemia (FH) from eight European countries
المؤلفون: Ramaswami, U, Futema, M, Bogsrud, MP, Holven, KB, Roeters van Lennep, J, Wiegman, A, Descamps, OS, Vrablik, M, Freiberger, T, Dieplinger, H, Greber-Platzer, S, Hanauer-Mader, G, Bourbon, M, Drogari, E, Humphries, SE
المصدر: Atherosclerosis , 292 pp. 178-187. (2020)
بيانات النشر: Elsevier BV
سنة النشر: 2020
المجموعة: University College London: UCL Discovery
مصطلحات موضوعية: Heterozygous familial hypercholesterolaemia, Paediatric FH, LDL-C concentrations, Statin treatment
الوصف: 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.
نوع الوثيقة: article in journal/newspaper
وصف الملف: text
اللغة: English
العلاقة: https://discovery.ucl.ac.uk/id/eprint/10086523/1/Humphries_Comparison%20of%20the%20characteristics%20at%20diagnosis%20and%20treatment%20of%20children%20with%20heterozygous%20familial%20hypercholesterolaemia%20%28FH%29%20from%20eight%20European%20countries_VoR.pdfTest; https://discovery.ucl.ac.uk/id/eprint/10086523Test/
الإتاحة: https://discovery.ucl.ac.uk/id/eprint/10086523/1/Humphries_Comparison%20of%20the%20characteristics%20at%20diagnosis%20and%20treatment%20of%20children%20with%20heterozygous%20familial%20hypercholesterolaemia%20%28FH%29%20from%20eight%20European%20countries_VoR.pdfTest
https://discovery.ucl.ac.uk/id/eprint/10086523Test/
حقوق: open
رقم الانضمام: edsbas.E6325265
قاعدة البيانات: BASE