Literature DB >> 22284919

Follow-up of children diagnosed with familial hypercholesterolemia in a national genetic screening program.

Hans J Avis1, D Meeike Kusters, Maud N Vissers, Roeland Huijgen, Thomas H Janssen, Albert Wiegman, Iris Kindt, John J P Kastelein, Frits A Wijburg, Barbara A Hutten.   

Abstract

OBJECTIVE: To assess the follow-up of children diagnosed as having familial hypercholesterolemia (FH) in the nationwide DNA-based cascade screening program (the Netherlands). STUDY
DESIGN: Questionnaires covering topics such as demographics, family history, physician consultation, and treatment were sent to parents of patients with FH (age 0-18 years), 18 months after diagnosis.
RESULTS: We retrieved 207 questionnaires of patients aged 10.9 ± 4.2 years (mean ± SD) at diagnosis; 48% were boys, and the mean low-density lipoprotein cholesterol (LDL-C) level at diagnosis was 167 ± 51 mg/dL. Of these patients, 164 (79%) consulted a physician: a general practitioner (35%), lipid-clinic specialist (27%), pediatrician (21%), internist (11%), or another physician (6%). LDL-C level at diagnosis and a positive family history for cardiovascular disease were independent predictors for physician consultation. Of the patients who visited a physician, 62% reported to have received lifestyle advice, and 43 (26%) were prescribed statin treatment. Independent predictors for medication use were age, LDL-C level, and educational level of parents.
CONCLUSION: The follow-up of children with FH after diagnosis established through cascade screening is inadequate. Better education of patients, parents, and physicians, with a structured follow-up after screening, should improve control of LDL-C levels and hence cardiovascular risk in children with FH.
Copyright © 2012 Mosby, Inc. All rights reserved.

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Year:  2012        PMID: 22284919     DOI: 10.1016/j.jpeds.2011.12.037

Source DB:  PubMed          Journal:  J Pediatr        ISSN: 0022-3476            Impact factor:   4.406


  10 in total

1.  Patterns and predictors of medication adherence to lipid-lowering therapy in children aged 8 to 20 years.

Authors:  Nina R Joyce; Gregory A Wellenius; Charles B Eaton; Amal N Trivedi; Justin P Zachariah
Journal:  J Clin Lipidol       Date:  2016-03-10       Impact factor: 4.766

2.  Patterns of Lipid Lowering Therapy among Children Ages 8-20 Years.

Authors:  Nina Joyce; Gregory A Wellenius; David D Dore; Jane W Newburger; Justin P Zachariah
Journal:  J Pediatr       Date:  2015-05-04       Impact factor: 4.406

3.  Patient Perspectives Regarding Genetic Testing for Familial Hypercholesterolemia.

Authors:  Miles Marchand; Victoria Chen; Mark Trinder; Lubomira Cermakova; Liam R Brunham
Journal:  CJC Open       Date:  2021-04-08

Review 4.  Management of hypercholesterolemia in children.

Authors:  Marjet J A M Braamskamp; Barbara A Hutten; Albert Wiegman; John J P Kastelein
Journal:  Paediatr Drugs       Date:  2014-04       Impact factor: 3.930

5.  Expert and Advocacy Group Consensus Findings on the Horizon of Public Health Genetic Testing.

Authors:  Stephen M Modell; Karen Greendale; Toby Citrin; Sharon L R Kardia
Journal:  Healthcare (Basel)       Date:  2016-01-27

6.  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.

Authors:  Fiona J Kinnear; Rachel Perry; Aidan Searle; Julian P Hamilton-Shield; Fiona E Lithander
Journal:  Syst Rev       Date:  2018-08-16

7.  The development of a theory informed behaviour change intervention to improve adherence to dietary and physical activity treatment guidelines in individuals with familial hypercholesterolaemia (FH).

Authors:  F J Kinnear; E Wainwright; J E Bourne; F E Lithander; J Hamilton-Shield; A Searle
Journal:  BMC Health Serv Res       Date:  2020-01-08       Impact factor: 2.655

8.  Reducing cardiovascular disease risk among families with familial hypercholesterolaemia by improving diet and physical activity: a randomised controlled feasibility trial.

Authors:  Fiona Jane Kinnear; Fiona E Lithander; Aidan Searle; Graham Bayly; Christina Wei; David J Stensel; Alice E Thackray; Linda Hunt; Julian P H Shield
Journal:  BMJ Open       Date:  2020-12-28       Impact factor: 2.692

Review 9.  Should We Be Screening for Ischaemic Heart Disease Earlier in Childhood?

Authors:  Pier Paolo Bassareo; Stephen T O'Brien; Esme Dunne; Sophie Duignan; Eliana Martino; Francesco Martino; Colin J Mcmahon
Journal:  Children (Basel)       Date:  2022-06-30

10.  Enablers and barriers to treatment adherence in heterozygous familial hypercholesterolaemia: a qualitative evidence synthesis.

Authors:  Fiona J Kinnear; Elaine Wainwright; Rachel Perry; Fiona E Lithander; Graham Bayly; Alyson Huntley; Jennifer Cox; Julian Ph Shield; Aidan Searle
Journal:  BMJ Open       Date:  2019-07-31       Impact factor: 2.692

  10 in total

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