ArticleArchives of disease in childhood2017
The UK Paediatric Familial Hypercholesterolaemia Register: preliminary data.
Article in Archives of disease in childhood, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
18 citing papers in PubMed, 1 synthesis or guideline pooled it, 52 citations in OpenAlex.
- Pooled it
- Screening for Familial Hypercholesterolemia in Childhood: An Overview of Current Practices Around the World.Children (Basel, Switzerland) · 2025Review
- Management of children with heterozygous familial hypercholesterolaemia worldwide: a meta-analysis.European heart journal open · 2025Article
- Should Familial Hypercholesterolaemia Be Included in the UK Newborn Whole Genome Sequencing Programme?Current atherosclerosis reports · 2023Review
- Efficacy and Safety of Statin Treatment in Children with Familial Hypercholesterolemia: Outcomes of 20 Years of Experience.Journal of clinical medicine · 2023Article
- Population-based screening in children for early diagnosis and treatment of familial hypercholesterolemia: design of the VRONI study.European journal of public health · 2022Article
- Review
- Population-based screening in children for early diagnosis and treatment of familial hypercholesterolemia: design of the VRONI study.Medizinische Genetik : Mitteilungsblatt des Berufsverbandes Medizinische Genetik e.V · 2022Article
- The Role of Registers in Increasing Knowledge and Improving Management of Children and Adolescents Affected by Familial Hypercholesterolemia: the LIPIGEN Pediatric Group.Frontiers in genetics · 2022Article
- Use of nuclear magnetic resonance spectroscopy in diagnosis of inborn errors of metabolism.Emerging topics in life sciences · 2021Review
- Familial hypercholesterolaemia: evolving knowledge for designing adaptive models of care.Nature reviews. Cardiology · 2020Review
- Comparison of the characteristics at diagnosis and treatment of children with heterozygous familial hypercholesterolaemia (FH) from eight European countries.Atherosclerosis · 2020Article
- How do the experiences and beliefs of adults and children with heterozygous familial hypercholesterolaemia influence their adherence to treatment? A systematic review of qualitative evidence protocol.Systematic reviews · 2018Article
- Design of the Familial Hypercholesterolaemia Australasia Network Registry: Creating Opportunities for Greater International Collaboration.Journal of atherosclerosis and thrombosis · 2017Article
- Knowns and unknowns in the care of pediatric familial hypercholesterolemia.Journal of lipid research · 2017Review
- Statin-Associated Myopathy in a Pediatric Preventive Cardiology Practice.The Journal of pediatrics · 2017Article
- Translational Research for Improving the Care of Familial Hypercholesterolemia: The "Ten Countries Study" and Beyond.Journal of atherosclerosis and thrombosis · 2016Review
- The UK Paediatric Familial Hypercholesterolaemia Register: Statin-related safety and 1-year growth data.Journal of clinical lipidologyArticle
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundThe National Institute for Health and Care Excellence 2008 guidelines on the treatment and management of familial hypercholesterolaemia (FH) recommend that children with FH should be considered for statin treatment by the age of 10 years. The Paediatric FH Register was established in 2012 to collect baseline and long-term follow-up data on all children with FH in the UK.
methodsPaediatricians and adult lipidologists have been invited to enter baseline data on any child with a clinical diagnosis of FH using an electronic capture record.
resultsBaseline data is on 232 children (50% boys, 80% Caucasian), with an untreated mean (SD) total cholesterol of 7.61 (1.48) mmol/L and low-density lipoprotein cholesterol (LDL-C) of 5.67 (1.46) mmol/L. Overall 111/232 (47.8%) of the children were on statins. Children over the age of 10 years at the most recent follow-up were twice as likely to be on statin treatment than those under 10 years (57.6% (102/177) vs 23.1% (9/39), p=0.00009). In both age groups, those subsequently on statin treatment had significantly higher diagnostic total and LDL-C (overall 6.01 (1.46) mmol/L vs 5.31 (1.37) mmol/L, p=0.00007), and had stronger evidence of a family history of early coronary heart disease (CHD) in parent or first-degree relative (overall 28.4% vs 19.0%, p=0.09). In statin-treated children LDL-C level was reduced by 35% (2.07 (1.38) mmol/L) compared with a reduction of 5.5% (0.29 (0.87) mmol/L), p=0.0001 in those not treated. None of those on statin had measured plasma levels of creatine kinase, alanine aminotransferase and AST indicative of statin toxicity (ie, >2.5 times the upper limit of the normal range).
conclusionsThe data indicates that treatment decisions in children with FH are appropriately based on a stronger family history of CHD and higher LDL-C.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.