Viral Pathogenesis of Early Cystic Fibrosis Lung Disease

Cystic Fibrosis Clinical Trial

— Early CF  

Official title:

Viral Pathogenesis of Early Cystic Fibrosis Lung Disease

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01973192
• Other study ID #: 1R01HL116211-01
• Secondary ID: 1R01HL116211-01
• Status: Completed
• Phase: N/A
• First received: September 17, 2013
• Last updated: April 3, 2017
• Start date: May 2013
• Est. completion date: December 1, 2016

Study information

• Verified date: April 2017
• Source: Indiana University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 65
• Est. completion date: December 1, 2016
• Est. primary completion date: December 1, 2016
• Accepts healthy volunteers: No
• Gender: All
• Age group: 2 Months to 4 Months

Eligibility

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.

Related Conditions & MeSH terms

• Cystic Fibrosis
• Fibrosis
• Lung Diseases
• Pulmonary Fibrosis

Locations

Country	Name	City	State
Australia	The Royal Children's Hospital	Melbourne	Victoria
Australia	Telethon Kids Institute	West Perth
United States	Riley Hospital for Children at Indiana University Health	Indianapolis	Indiana
United States	St. Louis Children's Hospital	St. Louis	Missouri

Sponsors (3)

Lead Sponsor	Collaborator
Indiana University School of Medicine	National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH)

Countries where clinical trial is conducted

United States,  Australia, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Other	Bronchiectasis	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.	12 Months
Primary	Viral infection	To determine the effect(s) of viral infections on the evolution of endobronchial bacterial infection and inflammation in CF infants.	12 months
Secondary	Pulmonary exacerbation rate	To identify the impact of respiratory viruses on the onset, frequency, and duration of respiratory symptoms in CF infants diagnosed through newborn screening.	12 Months
Secondary	Forced Expiratory Volume	To assess development of early lung disease as defined through physiological measures of forced expiratory flows, lung volumes, and ventilation inhomogeneity in CF infants.	12 months