Viral Pathogenesis of Early Cystic Fibrosis Lung Disease (Early CF)

This study is currently recruiting participants. (see Contacts and Locations)
Verified November 2015 by Indiana University
National Institutes of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Information provided by (Responsible Party):
Stephanie D. Davis, Indiana University Identifier:
First received: September 17, 2013
Last updated: November 13, 2015
Last verified: November 2015
The purpose of this study is to test the hypothesis that early viral infections alter the bacterial flora and inflammatory profile in the airway and accelerate progression of pulmonary disease in infants with cystic fibrosis.

Cystic Fibrosis

Study Type: Observational
Study Design: Observational Model: Cohort
Time Perspective: Prospective
Official Title: Viral Pathogenesis of Early Cystic Fibrosis Lung Disease

Resource links provided by NLM:

Further study details as provided by Indiana University:

Primary Outcome Measures:
  • Viral infection [ Time Frame: 12 months ] [ Designated as safety issue: No ]
    To determine the effect(s) of viral infections on the evolution of endobronchial bacterial infection and inflammation in CF infants.

Secondary Outcome Measures:
  • Pulmonary exacerbation rate [ Time Frame: 12 Months ] [ Designated as safety issue: No ]
    To identify the impact of respiratory viruses on the onset, frequency, and duration of respiratory symptoms in CF infants diagnosed through newborn screening.

  • Forced Expiratory Volume [ Time Frame: 12 months ] [ Designated as safety issue: No ]
    To assess development of early lung disease as defined through physiological measures of forced expiratory flows, lung volumes, and ventilation inhomogeneity in CF infants.

Other Outcome Measures:
  • Bronchiectasis [ Time Frame: 12 Months ] [ Designated as safety issue: No ]
    To evaluate the association of early viruses on the development of early lung disease in CF infants as defined through comprehensive structural and airway modeling techniques.

Biospecimen Retention:   Samples With DNA
Blood Urea, Bronchopulmonary Lavage samples, Nasal swabs and Oral swabs, Stool samples

Estimated Enrollment: 90
Study Start Date: May 2013
Estimated Study Completion Date: May 2016
Estimated Primary Completion Date: May 2016 (Final data collection date for primary outcome measure)
Detailed Description:
The proposed study is a unique international collaboration between three large CF research centers. This proposal will determine the impact of early respiratory viral infections on bacterial flora and inflammatory profiles in the CF airway as well as the impact of these pathogens on clinical, physiologic and structural markers of disease.The proposed study is designed to follow infants diagnosed with CF through newborn screening to determine the effect of viral infections on the lower airway microbiome, clinical symptoms, pulmonary function and structural changes during the first year of life. The proposed study will measure lower airway inflammation and infection using BAL, oral swabs, and nasal swabs; outcomes will be assessed through infant lung function testing, computerized tomography scans of the chest, and pulmonary exacerbation rate.

Ages Eligible for Study:   2 Months to 4 Months
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Infants, less than 4 months of age who have been diagnosed with Cystic Fibrosis

Inclusion Criteria:

  1. Diagnosis of CF by newborn screening, at least one clinical feature of CF, and documented sweat chloride greater than 60 mEq/L by quantitative pilocarpine iontophoresis or compatible genotype with two identifiable mutant CFTR alleles.
  2. Less than 4 months of age at Screening Visit
  3. Ability to comply with study visits and study procedures as judged by site investigator.

Exclusion Criteria:

  1. Intercurrent respiratory illness, defined as increase in cough, wheezing, or respiratory rate with onset 14 days before iPFT-bronchoscopy visit.
  2. Measured hemoglobin oxygen saturation less than 95% during the iPFT-bronchoscopy visit.
  3. History of adverse reaction to sedation.
  4. Clinically significant upper airway obstruction as determined by the site investigator.
  5. Severe gastroesophageal reflux, defined as persistent frequent emesis despite therapy.
  6. Major organ dysfunction, not including pancreatic dysfunction.
  7. Physical findings that would compromise the safety of the subject or the quality of the study data as determined by site investigator.
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its identifier: NCT01973192

Contact: Miriam Davis, RN, BSN

United States, Indiana
Riley Hospital for Children at Indiana University Health Recruiting
Indianapolis, Indiana, United States, 46202
Contact: Miriam Davis, RN, BSN   
Principal Investigator: Stephanie D Davis, MD         
United States, Missouri
St. Louis Children's Hospital Recruiting
St. Louis, Missouri, United States, 63110
Contact: Jane Quante    314-454-2353   
Principal Investigator: Thomas Ferkol, MD         
Australia, Victoria
The Royal Children's Hospital Recruiting
Melbourne, Victoria, Australia
Contact: Billy Skoric    +011-61-3-9345-5843   
Principal Investigator: Sarath Ranganathan, MD         
Telethon Kids Institute Recruiting
West Perth, Australia, 6872
Contact: Rachel Foong   
Sponsors and Collaborators
Indiana University School of Medicine
National Institutes of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Principal Investigator: Stephanie D. Davis, MD Indiana University School of Medicine
  More Information

Responsible Party: Stephanie D. Davis, MD, Section Director of Pediatric Pulmonology, Allergy and Sleep Medicine, Indiana University Identifier: NCT01973192     History of Changes
Other Study ID Numbers: 1R01HL116211-01 
Study First Received: September 17, 2013
Last Updated: November 13, 2015
Health Authority: United States: Institutional Review Board

Additional relevant MeSH terms:
Cystic Fibrosis
Lung Diseases
Pulmonary Fibrosis
Digestive System Diseases
Genetic Diseases, Inborn
Infant, Newborn, Diseases
Pancreatic Diseases
Pathologic Processes
Respiratory Tract Diseases processed this record on May 26, 2016