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

被引：6

作者：

de Koning, Lisanne ^{[1
,2
]}

Warnink-Kavelaars, Jessica ^{[2
,3
]}

van Rossum, Marion ^{[4
,5
]}

Limmen, Selina ^{[1
]}

Van der Looven, Ruth ^{[6
]}

Muino-Mosquera, Laura ^{[7
,8
]}

van der Hulst, Annelies ^{[9
]}

Oosterlaan, Jaap ^{[10
,11
,12
]}

Rombaut, Lies ^{[8
]}

Engelbert, Raoul ^{[1
,2
,3
]}

机构：

[1] Univ Appl Sci Amsterdam, Fac Hlth, Ctr Expertise Urban Vital, Amsterdam, Netherlands

[2] Univ Amsterdam, Dept Rehabil Med, Amsterdam UMC, Amsterdam, Netherlands

[3] Amsterdam Movement Sci, Rehabil & Dev, Amsterdam, Netherlands

[4] Emma Childrens Hosp, Acad Med Ctr, Dept Pediat, Amsterdam, Netherlands

[5] Reade, Amsterdam Rheumatol & Immunol Ctr, Amsterdam, Netherlands

[6] Ghent Univ Hosp, Dept Phys & Rehabil Med, Child Rehabil, Ghent, Belgium

[7] Ghent Univ Hosp, Dept Pediat, Div Pediat Cardiol, Ghent, Belgium

[8] Univ Ghent, Ghent Univ Hosp, Ctr Med Genet, Ghent, Belgium

[9] Univ Amsterdam, Dept Pediat Cardiol, Amsterdam UMC, Amsterdam, Netherlands

[10] Univ Amsterdam, Emma Childrens Hosp, Dept Pediat, Amsterdam UMC Follow Me Program, Amsterdam, Netherlands

[11] Univ Amsterdam, Emma Childrens Hosp, Emma Neurosci Grp, Amsterdam UMC, Amsterdam, Netherlands

[12] Amsterdam Reprod & Dev Res Inst, Amsterdam, Netherlands

来源：

FRONTIERS IN PEDIATRICS | 2023年 / 11卷

关键词：

Heritable Connective Tissue Disorders; Marfan Syndrome; Ehlers Danlos Syndromes; Loeys Dietz Syndrome; physical activity; physical fitness; JOINT HYPERMOBILITY SYNDROME; CHRONIC MUSCULOSKELETAL PAIN; EXERCISE CAPACITY; FEAR-AVOIDANCE; ADOLESCENTS; CHILDHOOD; VALIDITY; RELIABILITY; GUIDELINES; DISEASE;

D O I：

10.3389/fped.2023.1057070

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

ObjectivesHealth 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).MethodsPA 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).ResultsA 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).ConclusionThis 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.

引用

页数：9