Related Experiment Video

Updated: Jul 1, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

Lung function in infants with cystic fibrosis diagnosed by newborn screening

Barry M Linnane1, Graham L Hall, Gary Nolan

  • 1Department of Respiratory Medicine, Royal Children's Hospital Melbourne, Parkville, Australia.

American Journal of Respiratory and Critical Care Medicine
|September 13, 2008
PubMed

Insights

Lung function in infants with cystic fibrosis (CF) is normal at diagnosis but declines by 6 months of age. Early intervention within the first six months is crucial for preserving lung health in CF patients.

Area of Science:

  • Pediatric Pulmonology
  • Genetic Diseases
  • Respiratory Medicine

Background:

  • Progressive lung damage in cystic fibrosis (CF) begins in infancy.
  • Early detection and intervention are key for managing CF progression.
  • Newborn screening enables early diagnosis of CF.

Purpose of the Study:

  • To assess lung function in infants diagnosed with CF via newborn screening.
  • To investigate the relationship between lung function, pulmonary infection, and inflammation in infants with CF.

Main Methods:

  • Studied infants with CF (n=68) and healthy controls (n=49) aged 6 weeks to 30 months.
  • Measured lung function using forced vital capacity, FEV(0.5), and FEF(75) via the raised-volume rapid thoracoabdominal compression technique.
  • Collected bronchoalveolar lavage (BAL) samples from CF infants for infection and inflammation analysis.

Main Results:

  • Lung function (FEV(0.5) z score) was normal in infants with CF under 6 months compared to controls.
  • Lung function significantly decreased in CF infants older than 6 months.
  • Pulmonary infection and inflammation did not correlate with the observed lung function deficits.

Conclusions:

  • Infants with CF show normal lung function at diagnosis but diminished function by 6 months of age.
  • Therapeutic interventions in CF should ideally commence within the first 6 months of life.
  • Early intervention is critical for preserving lung function in infants with CF.
Abstract

More Related Videos

Protocol and Guidelines for Point-of-Care Lung Ultrasound in Diagnosing Neonatal Pulmonary Diseases Based on International Expert Consensus06:15

Protocol and Guidelines for Point-of-Care Lung Ultrasound in Diagnosing Neonatal Pulmonary Diseases Based on International Expert Consensus

Published on: March 6, 2019

Real-time X-ray Imaging of Lung Fluid Volumes in Neonatal Mouse Lung11:26

Real-time X-ray Imaging of Lung Fluid Volumes in Neonatal Mouse Lung

Published on: July 18, 2016

Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Pulmonary Function Tests01:25

Pulmonary Function Tests

Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
Breathing01:05

Breathing

The process of breathing, inhaling and exhaling, involves the coordinated movement of the chest wall, the lungs, and the muscles that move them. Two muscle groups with important roles in breathing are the diaphragm, located directly below the lungs, and the intercostal muscles, which lie between the ribs. When the diaphragm contracts, it moves downward, increasing the volume of the thoracic cavity and creating more room for the lungs to expand. When the intercostal muscles contract, the ribs...
Pulmonary Cycle: Exhalation01:17

Pulmonary Cycle: Exhalation

In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...