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Completed

NCT Number: NCT03579173

Determinants of Early Cystic Fibrosis Lung Disease

The overall objective of this study is to determine the impact early nutritional and respiratory indices have on early CF lung disease. This knowledge will guide clinical management of infants with CF, who are now primarily diagnosed through newborn screening.

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Key information

Conditions

Cystic Fibrosis in Children

Age range

1 month–5 year

Sex eligibility

All sexes

Study type

Observational

Primary location

Riley Hospital for Children

Indianapolis, Indiana, 46202, United States

About this study

The overall objective of this study is to determine the impact early nutritional and respiratory indices have on early CF lung disease. This knowledge will guide clinical management of infants with CF, who are now primarily diagnosed through newborn screening. We hypothesize that respiratory and nutritional indices during the first year of life are critical determinants of lung function in infants and preschoolers with CF.

Aim #1 - To examine the relationship between nutritional status (weight-for-age (WFA) and weight-for-length (WFL)) at 6 months of age and lung function at 1-2 years of age in infants with CF.

Hypothesis: Infants with CF with poor nutritional status at 6 months of age will have worse lung function, as assessed through the raised volume rapid thoracoabdominal compression technique and plethysmography, compared to those with better nutritional indices, defined as improved weight-for-age and weight-for-length.

Aim #2 - To examine the relationship between nutritional status (WFA and WFL) in infants with CF at 12 months of age and the lung clearance index (LCI) at 3-5 years of age.

Hypothesis: Infants with CF with poor nutritional indices at 12 months of age will have a higher (worse) LCI at 3-5 years of age compared to those with better nutritional status.

Aim #3 - To delineate the relationship between passive tidal breathing lung function testing in infants with CF at 4-8 weeks of age and subsequent lung function at 6-12 months of age.

Hypothesis: Abnormalities in passive tidal breathing lung function testing will be associated with abnormal infant pulmonary function testing obtained via the raised volume rapid thoracoabdominal compression technique and plethysmography at 6-12 months of age.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • Informed consent by a parent or legal guardian
  • A confirmed diagnosis of CF by newborn screening and either a documented sweat chloride of at least 60 mEq/L by quantitative pilocarpine iontophoresis or a genotype with two CF-causing mutations
  • One month to five years of age.

Exclusion criteria

  • Chronic lung disease not due to CF
  • Contraindications to sedation, including structural abnormalities of the upper airway or chest wall and severe gastroesophageal reflux
  • Gestational age < 36 weeks

Treatment and study plan

Infant PFT

Diagnostic Test

Assessed through the raised volume rapid thoracoabdominal compression technique and plethysmography

Lung Clearance Index

Diagnostic Test

The Lung Clearance Index is derived from the Multiple Breath Washout test. It is the cumulative exhaled volume (the volume of gas needed to wash out resident gas mixture out of the lungs) divided by the FRC. The FRC is the amount of air left in the lungs after normal exhalation.

Passive tidal breathing

Diagnostic Test

During quiet sleep, tidal breathing flow-volume curves are obtained. The tPEF/tE is calculated by taking the time it takes to reach peak expiratory flow divided by the total peak expiratory time.

Primary outcomes

  1. Lung function

    Time frame: 24 months

    Forced expiratory volume in 0.5 seconds

Secondary outcomes

  1. Lung function - FRC

    Time frame: 24 months

    Functional Residual Capacity

  2. Lung function - MBW

    Time frame: 24 months

    Multiple breath washout result

Sponsors and collaborators

Lead sponsor

Indiana University

Other

Registry information

Important dates

Study start
2018
Primary completion
2019
Study completion
2019
First posted
Jul 6, 2018
Registry last updated
Jun 22, 2022

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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