MRI in Cystic Fibrosis and Primary Ciliary Dyskinesia

NCT03279965 | Unknown

• 1 other identifier: 2016LI002B
• Study Type: observational
• Target: 50
• Locations: 1 country
• Sites: 1

Brief Summary

This is a small pilot / feasibility study (Approximately 50 patients) to assess the possibility of clinical implementation of MRI assessment of patients with cystic fibrosis and primary ciliary dyskinesia. Patients will undergo their standard CT imaging and lung function investigations and additionally will undergo MRI examination. Reports from CT (the current gold standard) and MRI will be assessed for concordance and patient acceptability and examination implementation costs will also be assessed. Novel MRI-based potential markers of CF and PCD disease state will also be assessed.

Conditions

Cystic Fibrosis; Primary Ciliary Dyskinesia

Keywords

Lung MRI cystic fibrosis primary ciliary dyskinesia

Outcome Measures

Primary Outcomes (1)

• Structural and quantitative MRI vs CT in cystic fibrosis and primary ciliary dyskinesia assessment

  To determine whether MRI with ventilation imaging can produce sufficiently diagnostic images, in the setting of CF or PCD, to replace CT imaging follow up. This will be assessed via semi-quantitative visual scoring (CT-based Brody / CFCT score and MRI-based Eichinger score) with both scoring systems applied to both CT and MRI. Scoring of the "air-trapping" component of CFCT will be substituted with scoring of ventilation defects on MRI. Scores will come from 2 observers, blinded to each others opinion, with a 3rd observer acting to resolve discrepancies. Intermodality variation will be assessed via ICC and Bland-Altman

  18 months

Secondary Outcomes (8)

• Ventilation MRI vs Lung clearance index (LCI)

  18 months

• Sinus disease and exacerbations 1

  18 months

• Sinus disease and exacerbations 2

  18 months

• Sinus disease and exacerbations 3

  18 months

• Liver disease 1

  18 months

Study Arms (2)

Cystic fibrosis

Patients with known cystic fibrosis

Diagnostic Test: MRI

Primary ciliary dyskinesia

Patients with known primary ciliary dyskinesia

Diagnostic Test: MRI

Interventions

MRI DIAGNOSTIC_TEST

MRI of lungs, paranasal sinuses and liver in addition to established clinical examinations (lung CT, pulmonary function testing)

Also known as: Nuclear magnetic resonance (NMR)

Cystic fibrosis; Primary ciliary dyskinesia

Eligibility Criteria

• Age: 6 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with known cystic fibrosis or primary ciliary dyskinesia referred for CT chest as part of routine outpatient or inpatient investigations.

You may qualify if:

• Known CF or PCD Referred for CT chest

You may not qualify if:

• Contraindication to MRI (Pacemaker etc) Unable to stay still for MRI

Sponsors & Collaborators

• Royal Brompton & Harefield NHS Foundation Trust: lead
• Bioxydyn Ltd, Manchester: collaborator
• University Hospital, Basel, Switzerland: collaborator

Study Sites (1)

Royal Brompton Hospital

London, SW3 6NP, United Kingdom

RECRUITING

MeSH Terms

Conditions

Cystic Fibrosis; Ciliary Motility Disorders

Interventions

Magnetic Resonance Imaging

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases; Otorhinolaryngologic Diseases; Ciliopathies; Abnormalities, Multiple; Congenital Abnormalities

Intervention Hierarchy (Ancestors)

Tomography; Diagnostic Imaging; Diagnostic Techniques and Procedures; Diagnosis

Study Officials

• Anand Devaraj

  Royal Brompton Hospital, London

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Anand Devaraj

CONTACT

02073528121; A.Devaraj@rbht.nhs.uk

Thomas Semple, MBBS, BSc

CONTACT

02073528121; T.Semple@rbht.nhs.uk

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: June 19, 2017
• First Posted: September 12, 2017
• Study Start: May 5, 2017
• Primary Completion: May 5, 2018
• Study Completion: May 5, 2018
• Last Updated: September 12, 2017

Record last verified: 2017-09

Data Sharing

• IPD Sharing: Will not share

Locations

GB (1)