ImaginAT

• Research type

  Research Study

• Full title

  Advanced Lung Imaging and Function Testing in Ataxia-Telangiectasia

• IRAS ID

  224417

• Contact name

  Jayesh Bhatt

• Contact email

  Jayesh.Bhatt@nhs.net

• Sponsor organisation

  Nottingham University Hospitals NHS Trust

• Duration of Study in the UK

  3 years, 0 months, 1 days

• Research summary

  Ataxia-Telangiectasia (A-T) is a progressive inherited condition in which the immune system does not work, and swallowing and muscle coordination fails due to brain disease. Survival has not improved since the 1950s – approximately half of patients die before their 20th birthday, mainly from respiratory failure.

  New lung treatments (such as drugs, physiotherapy or dietary interventions) are urgently required to improve survival. However, currently we do not know which tests are reliable to prove if a treatment has been effective.
  The usual measurements of lung health, such as the blowing tests in clinic are not reliable partly due to poor muscle co-ordination. We need to avoid X-rays in A-T, as these use radiation, and increase the risk of cancer.

  We believe that new advanced techniques in scanning (using magnetic imaging and not x-rays) and measuring lung function could potentially fill this need. We propose a 3 year study using these tests to measure lung health over time for a group of children. This will tell us if these tests are reliable in children with AT, and if they are suitable measurements of lung health that could be used in future trials.

• REC name

  East Midlands - Nottingham 2 Research Ethics Committee

• REC reference

  17/EM/0149

• Date of REC Opinion

  8 May 2017

• REC opinion

  Favourable Opinion