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David Stephensen 1,2 , Wendy Drechsler 1 , Oona Scott 1

Altered muscle strength and architecture influences motor performance in boys with severe haemophilia and ankle joint haemarthrosis. David Stephensen 1,2 , Wendy Drechsler 1 , Oona Scott 1. 1 Human Motor Performance Laboratory, School of Health, Sport & Bioscience University of East London

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David Stephensen 1,2 , Wendy Drechsler 1 , Oona Scott 1

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  1. Altered muscle strength and architecture influences motor performance in boys with severe haemophilia and ankle joint haemarthrosis David Stephensen1,2, Wendy Drechsler1, Oona Scott1 1Human Motor Performance Laboratory, School of Health, Sport & Bioscience University of East London 2Kent Haemophilia Centre, Kent & Canterbury Hospital

  2. Haemophilia • Deficiency of factor 8 (haemophilia A) or factor 9 (Haemophilia B / Christmas disease) • X linked recessive • Presents before 1 year of age

  3. Haemophilia • Deficiency of factor 8 (haemophilia A) or factor 9 (Haemophilia B / Christmas disease) • X linked recessive • Presents before 1 year of age • Recurrent frequent spontaneous bleeding into muscles and joints • Results in chronic disabling arthropathy

  4. Background Annual bleed frequency of 1-2 bleeds / yr (Feldmen et al. 2006; Manco-Johnson et al. 2007; Gringeri et al. 2011) Ankle joint is the most common site of bleeding (Stephensen et al. 2009) Muscles are smaller and weaker than their unaffected peers (Stephensen et al., 2012) Alterations in balance and gait when compared to unaffected peers (Bladen et al. 2007; Stephensen et al. 2009; De Souza et al., 2012)

  5. Aim of the study Relationship of lateral gastrocnemius muscle architecture to: Ankle plantar flexor muscle strength Knee and ankle function

  6. Participants Haemophilic boys were receiving prophylactic treatment and had a history of only ankle joint bleeding

  7. Methodology Muscle architecture Anatomical cross sectional area (ACSA) Thickness (MT) and width (MW) Muscle fascicle length (FL) and pennation angle (PA) Isokinetic muscle strength Three-dimensional joint angles and moments

  8. Three-dimensional joint angles and moments

  9. Three-dimensional joint angles and moments Terminal Double Support Initial Double Support Single Support Swing

  10. Results *p<0.05; **p<0.01; ***p<0.005

  11. Results *p<0.05; **p<0.01; ***p<0.005

  12. Results *p<0.05; **p<0.01; ***p<0.005

  13. 25 800 700 20 ) 2 600 Muscle thickness (mm) 15 ACSA (mm 500 10 400 300 5 0 20 40 60 0 20 40 60 Muscle strength (Nm) Muscle strength (Nm) Muscle strength is related to muscle size TD: r = 0.43 TD: r = 0.35 H: r = 0.06 H: r = 0.53

  14. 1.5 1.0 Knee flexion moment (Nm/kg) 0.5 0.0 0.00 0.05 0.10 2 Specific muscle torque (Nm/mm ) H: r = -0.61 (p < 0.05) TD: r = -0.32

  15. H: r = -0.58 (p < 0.05) H: r = -0.59 (p < 0.05) 1.5 TD: r = 0.24 TD: r = -0.45 15 1.0 14 Knee flexion moment (Nm/kg) 13 0.5 Maximum GRF (N/kg) 12 0.0 11 30 40 50 60 70 80 Fascicle length (mm) 10 0 20 40 60 Muscle strength (Nm)

  16. 0.3 30 ) 0.2 0 20 0.1 Ankle dorsiflexion moment (Nm/kg) Ankle plantarflexion ( 10 0.0 -0.1 0 5 10 15 20 25 0.00 0.05 0.10 2 Muscle thickness (mm) Specific muscle torque (Nm/mm ) H: r = -0.46 (p < 0.05) H: r = 0.52 (p < 0.05) TD: r = 0.13 TD: r = -0.13

  17. Clinical significance Importance of evaluating muscle function and strength Ankle plantar flexors are weaker and smaller Muscle strength and architecture strongly influence gait adaptations Impacts ankle and knee joint function during weight-bearing phases of walking

  18. Acknowledgements (NIHR) National Institute for Health Research NHS d.stephensen@uel.ac.uk

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