Physical fitness in children with Marfan and Loeys-Dietz syndrome: associations between cardiovascular parameters... 2024 Warnink-Kavelaars et al

Full title: Physical fitness in children with Marfan and Loeys-Dietz syndrome: associations between cardiovascular parameters, systemic manifestations, fatigue, and pain

Abstract

Children with Marfan (MFS) and Loeys-Dietz syndrome (LDS) report limitations in physical activities, sports, school, leisure, and work participation in daily life. This observational, cross-sectional, multicenter study explores associations between physical fitness and cardiovascular parameters, systemic manifestations, fatigue, and pain in children with MFS and LDS. Forty-two participants, aged 6–18 years (mean (SD) 11.5(3.7)), diagnosed with MFS (n = 36) or LDS (n = 6), were enrolled. Physical fitness was evaluated using the Fitkids Treadmill Test’s time to exhaustion (TTE) outcome measure. Cardiovascular parameters (e.g., echocardiographic parameters, aortic surgery, cardiovascular medication) and systemic manifestations (systemic score of the revised Ghent criteria) were collected. Pain was obtained by visual analog scale. Fatigue was evaluated by PROMIS® Fatigue-10a-Pediatric-v2.0-short-form and PROMIS® Fatigue-10a-Parent-Proxy-v2.0-short-form. Multivariate linear regression analyses explored associations between physical fitness (dependent variable) and independent variables that emerged from the univariate linear regression analyses (criterion p < .05).

The total group (MFS and LDS) and the MFS subgroup scored below norms on physical fitness TTE Z-score (mean (SD) −3.1 (2.9); −3.0 (3.0), respectively). Univariate analyses showed associations between TTE Z-score aortic surgery, fatigue, and pain (criterion p < .05). Multivariate analyses showed an association between physical fitness and pediatric self-reported fatigue that explained 48%; 49%, respectively, of TTE Z-score variance (F (1,18) = 18.6, p ≤ .001, r2 = .48; F (1,15) = 16,3, p = .01, r2 = .49, respectively).

Conclusions: Physical fitness is low in children with MFS or LDS and associated with self-reported fatigue. Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue, ultimately enhancing the physical activity and sports, school, leisure, and work participation of children with MFS and LDS.

What is Known:

• Marfan and Loeys-Dietz syndrome are heritable connective tissue disorders and share cardiovascular and systemic manifestations.

• Children with Marfan and Loeys-Dietz syndrome report increased levels of disability, fatigue and pain, as well as reduced levels of physical activity, overall health and health-related quality of life.

What is New:

• Physical fitness is low in children with Marfan and Loeys-Dietz syndrome and associated with self-reported fatigue.

• Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue, ultimately enhancing the physical activity and sports, school, leisure, and work participation of children with Marfan and Loeys-Dietz syndrome.

Open access, https://link.springer.com/article/10.1007/s00431-024-05456-z

 

I did a quick Google of mitochondrial dysfunction and Marfan.

This comes up:
https://www.nature.com/articles/s41467-021-22933-3
So, pretty common serious heart issues in Marfan.

Perhaps a large dollop of overconfidence going on here:
"Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue...."

'Cos exercise is the cure all / solver of all fatigue issues and seemingly oblivious to alternate explanation and schools of thought

 

They absolutely don't. This is what's truly wild about this brain-dead ideology, there is nothing that ever suggests any of this, it's simply their opinion, which they don't reason into and is blatantly post hoc fallacy. Their reasoning about finding the perfect balance of words and activities that will solve this are no different than sorcery being about the combination of the right incantations and ingredients.

Fatigue be gone!
Banish the spirits of low physical activity!
Wish upon a star and you will be as healthy as the others!