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: | Kirsten B. Holven, Mafalda Bourbon, Tomáš Freiberger, Olivier S. Descamps, Susanne Greber-Platzer, Albert Wiegman, Jeanine E. Roeters van Lennep, Michal Vrablík, Gabriele Hanauer-Mader, Marta Futema, Euridiki Drogari, Uma Ramaswami, Hans Dieplinger, Martin Prøven Bogsrud, Steve E. Humphries |
| المساهمون: | Cardiology, Internal Medicine, Paediatric Metabolic Diseases, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, ACS - Heart failure & arrhythmias, ACS - Diabetes & metabolism, ACS - Atherosclerosis & ischemic syndromes |
| المصدر: | Atherosclerosis, 292, 178-187. Elsevier Ireland Ltd Atherosclerosis |
| بيانات النشر: | Elsevier BV, 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Male, 0301 basic medicine, Heterozygote, Pediatrics, medicine.medical_specialty, Statin, Statin treatment, medicine.drug_class, Heterozygous familial hypercholesterolaemia, Paediatric FH, Age at diagnosis, Low density lipoprotein cholesterol, Heterozygous Familial Hypercholesterolaemia, 030204 cardiovascular system & hematology, Article, Doenças Cardio e Cérebro-vasculares, Hyperlipoproteinemia Type II, LDL-C concentrations, 03 medical and health sciences, 0302 clinical medicine, Ezetimibe, Humans, Medicine, Child, Retrospective Studies, business.industry, Cholesterol, LDL, Statin Treatment, Treatment characteristics, 3. Good health, Europe, 030104 developmental biology, Lifestyle advice, Child, Preschool, Female, lipids (amino acids, peptides, and proteins), Statin therapy, Hydroxymethylglutaryl-CoA Reductase Inhibitors, Cardiology and Cardiovascular Medicine, business, LDL-C Concentrations, medicine.drug |
| 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. Graphical abstract Image 1 Highlights • The age of HeFH diagnosis varies significantly between 8 European countries. • The proportion of HeFH children being treated varies across 8 European countries. • A quarter of FH children on statins have LDL-C above the target (>3.5 mmol/L). • Many FH children are not getting the full benefit of early diagnosis and treatment. |
| وصف الملف: | application/pdf |
| تدمد: | 0021-9150 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::61124bbc44f5e8d6ec46d690f10c2cb7Test https://doi.org/10.1016/j.atherosclerosis.2019.11.012Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....61124bbc44f5e8d6ec46d690f10c2cb7 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 00219150 |
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