Viral Pathogenesis of Early Cystic Fibrosis Lung Disease

Completed

• Conditions: Cystic Fibrosis

• Registration Number: NCT01973192
• Lead Sponsor: Indiana University School of Medicine

Brief Summary

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.

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.

Study Timeline

• Study Start: 2013-05
• Study First Submitted: 2013-09-17
• Study First Submitted QC: 2013-10-25
• Study First Posted: 2013-10-31
• Primary Completion: 2016-12-01
• Study Completion: 2016-12-01
• Status Verified: 2017-04
• Last Update Submitted: 2017-04-03
• Last Update Posted: 2017-04-04

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 65

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.

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Viral infection	12 months	To determine the effect(s) of viral infections on the evolution of endobronchial bacterial infection and inflammation in CF infants.

Secondary Outcome Measures

Name	Time	Method
Forced Expiratory Volume	12 months	To assess development of early lung disease as defined through physiological measures of forced expiratory flows, lung volumes, and ventilation inhomogeneity in CF infants.
Pulmonary exacerbation rate	12 Months	To identify the impact of respiratory viruses on the onset, frequency, and duration of respiratory symptoms in CF infants diagnosed through newborn screening.

Trial Locations

Locations (4)

St. Louis Children's Hospital; 🇺🇸 St. Louis, Missouri, United States

Riley Hospital for Children at Indiana University Health; 🇺🇸 Indianapolis, Indiana, United States

The Royal Children's Hospital; 🇦🇺 Melbourne, Victoria, Australia

Telethon Kids Institute; 🇦🇺 West Perth, Australia