Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Mar 17:11:1057070.
doi: 10.3389/fped.2023.1057070. eCollection 2023.

Physical activity and physical fitness in children with heritable connective tissue disorders

Lisanne de Koning  1   2 , Jessica Warnink-Kavelaars  2   3 , Marion van Rossum  4   5 , Selina Limmen  1 , Ruth Van der Looven  6 , Laura Muiño-Mosquera  7   8 , Annelies van der Hulst  9 , Jaap Oosterlaan  10   11 , Lies Rombaut  8 , Raoul Engelbert  1   2   3 ; Pediatric Heritable Connective Tissue Disorders Study Group
Affiliations

Physical activity and physical fitness in children with heritable connective tissue disorders

Lisanne de Koning et al. Front Pediatr. .

Abstract

Objectives: Health problems in patients with heritable connective tissue disorders (HCTD) are diverse and complex and might lead to lower physical activity (PA) and physical fitness (PF). This study aimed to investigate the PA and PF of children with heritable connective tissue disorders (HCTD).

Methods: PA was assessed using an accelerometer-based activity monitor (ActivPAL) and the mobility subscale of the Pediatric Evaluation of Disability Inventory Computer Adaptive Test (PEDI-CAT). PF was measured in terms of cardiovascular endurance using the Fitkids Treadmill Test (FTT); maximal hand grip strength, using hand grip dynamometry (HGD) as an indicator of muscle strength; and motor proficiency, using the Bruininks-Oseretsky Test of Motor Proficiency-2 (BOTMP-2).

Results: A total of 56 children, with a median age of 11.6 (interquartile range [IQR], 8.8-15.8) years, diagnosed with Marfan syndrome (MFS), n = 37, Loeys-Dietz syndrome (LDS), n = 6, and genetically confirmed Ehlers-Danlos (EDS) syndromes, n = 13 (including classical EDS n = 10, vascular EDS n = 1, dermatosparaxis EDS n = 1, arthrochalasia EDS n = 1), participated. Regarding PA, children with HCTD were active for 4.5 (IQR 3.5-5.2) hours/day, spent 9.2 (IQR 7.6-10.4) hours/day sedentary, slept 11.2 (IQR 9.5-11.5) hours/day, and performed 8,351.7 (IQR 6,456.9-1,0484.6) steps/day. They scored below average (mean (standard deviation [SD]) z-score -1.4 (1.6)) on the PEDI-CAT mobility subscale. Regarding PF, children with HCTD scored well below average on the FFT (mean (SD) z-score -3.3 (3.2)) and below average on the HGD (mean (SD) z-score -1.1 (1.2)) compared to normative data. Contradictory, the BOTMP-2 score was classified as average (mean (SD) z-score.02 (.98)). Moderate positive correlations were found between PA and PF (r(39) = .378, p < .001). Moderately sized negative correlations were found between pain intensity and fatigue and time spent actively (r(35) = .408, p < .001 and r(24) = .395 p < .001, respectively).

Conclusion: This study is the first to demonstrate reduced PA and PF in children with HCTD. PF was moderately positively correlated with PA and negatively correlated with pain intensity and fatigue. Reduced cardiovascular endurance, muscle strength, and deconditioning, combined with disorder-specific cardiovascular and musculoskeletal features, are hypothesized to be causal. Identifying the limitations in PA and PF provides a starting point for tailor-made interventions.

Keywords: Ehlers Danlos Syndromes; Heritable Connective Tissue Disorders; Loeys Dietz Syndrome; Marfan Syndrome; physical activity; physical fitness.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Similar articles

See all similar articles

Cited by

References

    1. Bar-Or O, Rowland TW. Pediatric exercise medicine: From physiologic principles to health care application. Champaign, IL: Human Kinetics; (2004).
    1. Thornton JS, Frémont P, Khan K, Poirier P, Fowles J, Wells GD, et al. Physical activity prescription : a critical opportunity to address a modi fi able risk factor for the prevention and management of chronic disease : a position statement by the Canadian academy of sport and exercise medicine. Br J Sports Med. (2016) 50(18):1109–14. 10.1136/bjsports-2016-096291 - DOI - PubMed
    1. Dimitri P, Joshi K, Jones N, Medicine M. Moving more: physical activity and Its positive effects on long term conditions in children and young people. Arch Dis Child. (2020) 105(11):1035–40. 10.1136/archdischild-2019-318017 - DOI - PubMed
    1. van Brussel M, van der Net J, Hulzebos E, Helders PJM, Takken T. The Utrecht approach to exercise in chronic childhood conditions: the decade in review. Pediatr Phys Ther. (2011) 23(1):2–14. 10.1097/PEP.0b013e318208cb22 - DOI - PubMed
    1. Bouchard C, Blair SN, Haskell WL. Physical activity and health. 2nd edi. Bouchard C, Blair SN, Haskell WL, editors. Champaign, IL: Human Kinetics; (2012). p. 1–426.