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Muscle Strength, Power, and Torque Deficits in Children With Type SS Sickle Cell Disease

Kelly A Dougherty1, Chiara Bertolaso2, Joan I Schall3

  • 1School of Health Sciences, Stockton University, Galloway, NJ.

Journal of Pediatric Hematology/Oncology
|April 6, 2018
PubMed

Insights

Children with sickle cell disease (SCD-SS) show reduced muscle strength and power compared to healthy peers. These deficits persist even after adjusting for body composition, suggesting other contributing factors in pediatric SCD-SS.

Area of Science:

  • Pediatric Physical Performance
  • Hematology
  • Sickle Cell Disease Research

Background:

  • Sickle cell disease (SCD-SS) is a genetic blood disorder affecting red blood cells.
  • Children with SCD-SS often exhibit poorer growth and body composition compared to healthy peers.
  • Understanding physical performance deficits is crucial for managing SCD-SS in children.

Purpose of the Study:

  • To compare maximal handgrip strength, peak power, and isometric maximal voluntary contraction (MVC) torque in African-American children with and without SCD-SS.
  • To analyze these performance metrics after adjusting for body size and composition.
  • To identify potential factors contributing to attenuated anaerobic performance in pediatric SCD-SS.

Main Methods:

  • Cross-sectional study comparing African-American children aged 5-17 with SCD-SS (n=21) and healthy controls (n=23).
  • Measurements included dominant hand maximal handgrip strength, peak power, and plantar flexion isometric MVC torque.
  • Statistical analyses adjusted for body size (height, weight, BMI) and composition (lean mass, fat mass, leg length).

Main Results:

  • Children with SCD-SS had significantly lower Z scores for height, weight, BMI, and various body composition metrics.
  • Unadjusted handgrip strength, peak power, and MVC torques were significantly lower in children with SCD-SS.
  • Performance decrements remained significant after adjusting for body size and composition, indicating other contributing factors.

Conclusions:

  • Children with SCD-SS exhibit significant deficits in muscle strength and anaerobic performance.
  • These deficits are not solely explained by differences in body size or composition.
  • Additional underlying factors likely contribute to the attenuated physical performance observed in pediatric SCD-SS.

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