Clinical Trial: Dynamic Gait Analysis in Children With Mucopolysaccharidosis Type IVa

Study Status: Withdrawn
Recruit Status: Withdrawn
Study Type: Observational

Official Title:

Brief Summary:

Mucopolysaccharidosis Type IVa (MPS IVa, Morquio Disease) is a rare inherited lysosomal storage disorder caused by deficiency of the enzyme galactose-6-sulfatase.

Children with this disease accumulate a chemical called keratan sulphate, which stops their skeletons developing properly. They are very short in stature and many of their joints are unstable. Children with MPS IVa walk in a different way to other people due to a combination of lax ligaments and skeletal problems such as knock-knees.

Human walking involves the coordinated movements of all four limbs. As we walk, the arms swing oppositely to the legs. This movement pattern is very different in children with MPS IVa. This change seems to involve the whole musculoskeletal system and depends on the severity of the disease.

Recent studies in children with MPS IVa describing walking pattern have concentrated solely on the lower or upper limb respectively, and have not looked at the interaction of the upper and lower limbs during walking.

To our knowledge, the mechanics of walking in children with MPS IVa has not been investigated using a dynamic gait analysis tool (using cameras, sensors and electrodes to track the movements of different parts of the body during walking) and we aim to characterise this in a small number of children with MPS IVa and also examine the effects of splinting the wrist upon the walking pattern to see if this simple intervention makes it easier or more difficult for children with MPS IVa to walk.


Detailed Summary:
Sponsor: Birmingham Women's and Children's NHS Foundation Trust

Current Primary Outcome: Evidence of abnormal walking pattern and surface EMG activity as assessed by Dynamic Gait Analysis [ Time Frame: Within 6 months of recruitment ]

Assessment of head, trunk and joint positions during walking using a 12 camera Vicon motion analysis system. Surface EMG analysis using a 16 channel wireless surface electromyographic (sEMG) system. Assessment of lower limb joint moments and powers using Kistler 9281 and AMTI OPT 400600 force plates.


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Change in gait pattern over one year [ Time Frame: 12 months after first analysis ]
    Comparison of two gait analysis studies taken 12 months apart
  • Effect on gait pattern of using wrist splints [ Time Frame: Within 6 months of recruitment ]
    Assessment of repeat gait analysis measurements done at first visit whilst wearing wrist splints
  • Effect on gait pattern of lower limb surgery [ Time Frame: Within 3 and 6 months of any lower limb surgery ]
    Further gait analysis studies will be done if a child undergoes any lower limb orthopaedic surgery during the study period and compared with pre-surgery analyses


Original Secondary Outcome: Same as current

Information By: Birmingham Women's and Children's NHS Foundation Trust

Dates:
Date Received: May 28, 2014
Date Started: July 2016
Date Completion:
Last Updated: July 27, 2016
Last Verified: July 2016