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Muscle Function in Osteogenesis Imperfecta Type IV.

Louis-Nicolas Veilleux1,2, Vasiliki B Darsaklis3, Kathleen Montpetit3

  • 1Motion Analysis Center, Shriners Hospital for Children-Canada, 1003 Decarie Boulevard, Montreal, QC, H4A 0A9, Canada. lnveilleux@shriners.mcgill.ca.

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Summary

Children and adolescents with osteogenesis imperfecta (OI) type IV show normal upper-limb muscle force but significant lower-limb muscle deficits. These findings highlight potential contributions of leg weakness to functional impairments in OI type IV.

Keywords:
Lower-limb muscle functionMaximum isometric grip forceMechanographyMuscle powerOsteogenesis imperfectUpper-limb muscle force

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Area of Science:

  • Orthopedics
  • Pediatric medicine
  • Rehabilitation science

Background:

  • Previous studies suggest muscle force deficits in children and adolescents with osteogenesis imperfecta (OI) type IV.
  • Objective quantification of muscle function in this population is lacking.

Purpose of the Study:

  • To assess and compare upper and lower extremity muscle function in patients with OI type IV.
  • To compare muscle function between OI type IV, OI type I, and healthy controls.

Main Methods:

  • Study conducted in a pediatric orthopedic hospital outpatient department.
  • 27 individuals with OI type IV, 27 with OI type I, and 27 controls (ages 7-21) participated.
  • Muscle function assessed via hand dynamometry (upper limb) and mechanography (lower limb) including hopping, jumping, and chair-rise tests.

Main Results:

  • Patients with OI type IV exhibited normal upper-limb grip force compared to reference data.
  • OI type IV patients showed ~30% lower peak force and ~50% lower peak power in lower limbs compared to controls (P<0.05).
  • Lower-limb peak power was ~50% lower in OI type IV compared to OI type I patients (P<0.05).

Conclusions:

  • Individuals with OI type IV have normal upper-limb muscle force.
  • Significant lower-limb muscle function deficits are present in OI type IV patients.
  • Lower-limb muscle weakness may contribute to functional deficits in osteogenesis imperfecta type IV.