دورية أكاديمية
Comparison of the characteristics at diagnosis and treatment of children with heterozygous familial hypercholesterolaemia (FH) from eight European countries
| العنوان: | Comparison of the characteristics at diagnosis and treatment of children with heterozygous familial hypercholesterolaemia (FH) from eight European countries |
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| المؤلفون: | Ramaswami, U. (Uma), Futema, M. (Marta), Bogsrud, M.P. (Martin P.), Holven, K.B. (Kirsten B.), Roeters van Lennep, J.E. (Jeanine), Wiegman, A. (Albert), Descamps, O.S. (Olivier S.), Vrablik, M. (Michal), Freiberger, T. (Tomáš), Dieplinger, H. (Hans), Greber-Platzer, S. (Susanne), Hanauer-Mader, G. (Gabriele), Bourbon, M. (Mafalda), Drogari, E. (Euridiki), Humphries, S.E. (Steve) |
| المصدر: | Atherosclerosis vol. 292, pp. 178-187 |
| سنة النشر: | 2020 |
| المجموعة: | RePub - Publications from Erasmus University, Rotterdam |
| مصطلحات موضوعية: | Heterozygous familial hypercholesterolaemia, LDL-C concentrations, Paediatric FH, 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: | application/pdf |
| اللغة: | English |
| العلاقة: | http://repub.eur.nl/pub/122151Test; urn:hdl:1765/122151 |
| DOI: | 10.1016/j.atherosclerosis.2019.11.012 |
| الإتاحة: | https://doi.org/10.1016/j.atherosclerosis.2019.11.012Test http://repub.eur.nl/pub/122151Test |
| حقوق: | info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.83109E0A |
| قاعدة البيانات: | BASE |
| DOI: | 10.1016/j.atherosclerosis.2019.11.012 |
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