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Prospective Surveillance of Lung Development During Childhood, Adolescence and Adulthood in Healthy and Patients With Cystic Fibrosis (Prospective)

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ClinicalTrials.gov Identifier: NCT04395820
Recruitment Status : Active, not recruiting
First Posted : May 20, 2020
Last Update Posted : November 4, 2020
Sponsor:
Information provided by (Responsible Party):
Kathryn Ramsey, University Hospital Inselspital, Berne

Brief Summary:

Cystic fibrosis (CF) is the most common lethal inherited disease in Caucasian populations. To improve survival, it is essential to understand the development, progression and treatment of CF lung disease throughout early childhood.

Therefore the overall objective is to prospectively assess the clinical utility of novel and non-invasive measuring methods, namely Multiple Breath Washout and functional lung MRI in the longitudinal clinical surveillance of patients with CF and compare the results to those of healthy controls.


Condition or disease Intervention/treatment
Cystic Fibrosis Healthy Diagnostic Test: Lung function test Diagnostic Test: Imaging

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Study Type : Observational
Estimated Enrollment : 250 participants
Observational Model: Cohort
Time Perspective: Prospective
Official Title: Prospective Surveillance of Lung Development During Childhood, Adolescence and Adulthood in Healthy and Patients With Cystic Fibrosis
Actual Study Start Date : July 1, 2020
Estimated Primary Completion Date : December 1, 2050
Estimated Study Completion Date : December 1, 2100

Resource links provided by the National Library of Medicine

MedlinePlus related topics: Cystic Fibrosis

Group/Cohort Intervention/treatment
Cystic Fibrosis Diagnostic Test: Lung function test
MBW

Diagnostic Test: Imaging
MP-MRI

Healthy Diagnostic Test: Lung function test
MBW

Diagnostic Test: Imaging
MP-MRI




Primary Outcome Measures :
  1. Multiple Breath Washout [ Time Frame: Every third month up to 50 years. Healthy controls only during 1 year. ]
    Longitudinal assessment of lung volume and ventilation inhomogeneity

  2. Functional MP-MRI [ Time Frame: Every twelfth month up to 50 years. Healthy controls only during 1 year (2 time points). ]
    Longitudinal assessment of percentage of the lung volume with impaired fractional ventilation and relative perfusion


Secondary Outcome Measures :
  1. Morphological MRI [ Time Frame: Every twelfth month up to 50 years. Healthy controls only during 1 year (2 time points) ]
    Longitudinal assessment of the presence and extent of bronchiectasis, mucous plugging and air trapping by Eichinger MRI scoring.

  2. Spirometry: FEV1 [ Time Frame: Every third month up to 50 years. Healthy controls only during 1 year. ]
    Longitudinal assessment of forced expired volume in 1 second.

  3. Spirometry: FVC [ Time Frame: Every third month up to 50 years. Healthy controls only during 1 year. ]
    Longitudinal assessment of forced vital capacity.

  4. Spirometry: FEF [ Time Frame: Every third month up to 50 years. Healthy controls only during 1 year. ]
    Longitudinal assessment of forced expiratory flows.

  5. Body plethysmography: sRAW [ Time Frame: Every third month up to 50 years. Healthy controls only during 1 year. ]
    Longitudinal assessment of specific airway resistance.

  6. Body plethysmography: FRC [ Time Frame: Every third month up to 50 years. Healthy controls only during 1 year. ]
    Longitudinal assessment of functional residual capacity.

  7. Body plethysmography: TLC [ Time Frame: Every third month up to 50 years. Healthy controls only during 1 year. ]
    Longitudinal assessment of total lung capacity.

  8. Respiratory symptoms [ Time Frame: Every third month up to 50 years. Only CF patients. ]
    Longitudinal assessment of clinical respiratory symptoms.

  9. Exacerbations [ Time Frame: Every third month up to 50 years. Only CF patients. ]
    Longitudinal assessment of clinical status.

  10. CF-related quality of life [ Time Frame: Every third month up to 50 years. Only CF patients. ]
    Longitudinal assessment of standardised age-specific CF-related quality of life questions.The scale goes from 0-100, higher score means better outcome.

  11. Microbiology: presence of respiratory pathogens [ Time Frame: Every third month up to 50 years. Only CF patients. ]
    Longitudinal assessment of presence of respiratory pathogens from oropharyngeal swabs and sputum samples.

  12. Microbiology: abundance of respiratory pathogens [ Time Frame: Every third month up to 50 years. Only CF patients. ]
    Longitudinal assessment of abundance of respiratory pathogens from oropharyngeal swabs and sputum samples.


Biospecimen Retention:   Samples With DNA
Oropharyngeal swabs


Information from the National Library of Medicine

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Ages Eligible for Study:   3 Years to 18 Years   (Child, Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population
Children and adults with CF and healthy volunteers from the local community in Bern and surrounding areas.
Criteria

Inclusion Criteria:

Individuals with CF:

  • Diagnosis of CF
  • Signed written informed consent
  • ≥3 - 18 years of age, depending on the cooperation and if lung function measurements are possible

Healthy volunteers:

  • Signed written informed consent
  • Informed consent of participant and if under 18 years, legal representative respectively
  • Children and adults with no history of chronic lung disease or acute respiratory infection in the four weeks prior to the study visit
  • ≥3 - 18 years of age, depending on the cooperation and if lung function measurements are possible

Exclusion Criteria:

The presence of any one of the following exclusion criteria will lead to exclusion of the participant, for example:

  • Women who are pregnant or breast feeding.
  • Intention to become pregnant during the course of the study
  • Lack of safe contraception, defined as: Female participants of childbearing potential, not using and not willing to continue using a medically reliable method of contraception for the entire study duration, such as oral, injectable, or implantable contraceptives, or intrauterine contraceptive devices, or who are not using any other method considered sufficiently reliable by the investigator in individual cases.
  • Please note that female participants who are surgically sterilised/hysterectomised or post-menopausal for longer than 2 years are not considered as being of child bearing potential.
  • Other clinically significant concomitant disease states (e.g. renal failure, hepatic dysfunction, cardiovascular disease, etc.)
  • Known or suspected non-compliance, drug or alcohol abuse
  • Continuous glucose monitor
  • Inability to follow the procedures of the study, e.g. due to language problems, psychological disorders, etc. of the participant
  • Metal in body, e.g. pacemaker
  • Participation in another study with investigational drug within the 30 days preceding and during the present study
  • Subjects which are respiratory insufficient to attend on the lung function measurements (oxygen demand)
  • Subjects who are unable to perform the MRI without sedation
  • Participants which were born preterm (<36. week of pregnancy)
  • Current smokers

In addition for individuals with CF:

  • Known diseases other than related to CF

In addition for healthy individuals:

  • Current upper respiratory infection (cough, cold, fever) will lead to postponement of the visit to 4 weeks after the end of symptoms

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT04395820


Locations
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Switzerland
University Children's Hospital Bern
Bern, Switzerland, 3010
Sponsors and Collaborators
University Hospital Inselspital, Berne
Investigators
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Principal Investigator: Kathryn Ramsey, PhD University Children's Hospital Bern
Publications:
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Responsible Party: Kathryn Ramsey, Principal Investigator, University Hospital Inselspital, Berne
ClinicalTrials.gov Identifier: NCT04395820    
Other Study ID Numbers: 2019-01591
First Posted: May 20, 2020    Key Record Dates
Last Update Posted: November 4, 2020
Last Verified: November 2020

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Keywords provided by Kathryn Ramsey, University Hospital Inselspital, Berne:
Cystic Fibrosis
MBW
MRI
Airway growth
Airway development
Healthy
Additional relevant MeSH terms:
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Cystic Fibrosis
Fibrosis
Pathologic Processes
Pancreatic Diseases
Digestive System Diseases
Lung Diseases
Respiratory Tract Diseases
Genetic Diseases, Inborn
Infant, Newborn, Diseases