Clinical trial NCT03279965: MRI in Cystic Fibrosis and Primary Ciliary Dyskinesia
Official title: A Pilot Study to Assess the Use of MRI in the Assessment of Patients With Cystic Fibrosis and Primary Ciliary Dyskinesia
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Cystic Fibrosis
Primary Ciliary Dyskinesia
Keywords: Lung MRI cystic fibrosis primary ciliary dyskinesia
Lead sponsor: Royal Brompton & Harefield NHS Foundation Trust
Sponsor class: OTHER
Collaborators: name: Bioxydyn Ltd, Manchester
name: University Hospital, Basel, Switzerland
First posted: 2017-09-12
Enrollment: 50
Sex: ALL
Minimum age (years): 6
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: CASE_ONLY
Time perspective: PROSPECTIVE
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.

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.

Detailed description: In this small single site pilot / feasibility study we aim to recruit approximately 50 patients over the age of 6 years with known cystic fibrosis (CF) or primary ciliary dyskinesia (PCD).

Patients will be recruited from routine clinic appointments or at admission for inpatient investigation and/or treatment at the point of referral for computed tomography (CT) examination. If they consent to participation they will undergo MRI examination in addition to their standard clinical CT (within 7 days of the CT scan - on the same day if practicable).

The magnetic resonance imaging (MRI) scans will be anonymised and reported by radiologists, blinded to the CT findings. The reports and scores (Eichinger and Brody2) will then be compared those of the standard CT to assess concordance between the modalities and inter and intra observer variability.

Potential novel biomarkers (pulmonary ventilation and perfusion (non-contrast) and sinus mucus and liver tissue characteristics) will be compared with established markers (including lung clearance index) and known CF mutation / PCD type.

Patients or their carers will also be asked to fill in a short questionnaire comparing the differential patient acceptability of CT and MRI examinations. The cost (time, resources etc) of each examination will be calculated and compared.

Eligibility criteria: Inclusion Criteria:

* Known CF or PCD Referred for CT chest

Exclusion Criteria:

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

Primary outcomes:
measure: Structural and quantitative MRI vs CT in cystic fibrosis and primary ciliary dyskinesia assessment
timeFrame: 18 months
Secondary outcomes:
measure: Ventilation MRI vs Lung clearance index (LCI)
timeFrame: 18 months
measure: Sinus disease and exacerbations 1
timeFrame: 18 months
measure: Sinus disease and exacerbations 2
timeFrame: 18 months
measure: Sinus disease and exacerbations 3
timeFrame: 18 months
measure: Liver disease 1
timeFrame: 18 months
measure: Liver disease 2
timeFrame: 18 months
measure: Patient acceptability
timeFrame: 18 months
measure: Clinical implementation
timeFrame: 18 months
Other outcomes:
Locations:
city: London
country: United Kingdom
facility: Royal Brompton Hospital
Study officials:
affiliation: Royal Brompton Hospital, London
name: Anand Devaraj
role: PRINCIPAL_INVESTIGATOR
References: