Related Experiment Video

Updated: Sep 28, 2025

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections
06:57

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections

Published on: May 8, 2017

9.3K

Factors in childhood associated with lung function decline to adolescence in cystic fibrosis

Nelufa Begum1, Catherine A Byrnes2, Joyce Cheney3

  • 1Children's Health and Environment Program, Child Health Research Centre, The University of Queensland, 62 Graham St, South Brisbane, QLD 4101, Australia.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|March 28, 2022
PubMed

Insights

Early childhood hospitalizations and neutrophil elastase activity in children with cystic fibrosis (CF) predict lung function decline. Interventions targeting these factors in early life may improve long-term respiratory health in CF patients.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Genetics and Disease

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
  • Despite advances in care, progressive lung function decline remains a significant challenge for individuals with CF, particularly during adolescence and early adulthood.
  • Understanding early-life predictors of lung function decline is crucial for developing targeted interventions.

Purpose of the Study:

  • To identify factors present at age 5 years that predict lung function decline from childhood to adolescence in children with CF.
  • To analyze lung function trajectories in a longitudinal cohort of Australasian children with CF.

Main Methods:

  • A longitudinal study followed 119 Australasian children with CF from 1999 to 2017.
  • Lung function was assessed from childhood (median age 5.0 years) to early adolescence (median age 12.5 years).
  • Multivariable analyses examined predictors of lung function decline, including hospitalization history and bronchoalveolar lavage neutrophil elastase activity.

Main Results:

  • Lung function progressively declined in children with CF, with significant annual decreases in forced vital capacity (FVC) Z-score, forced expiratory volume in 1-second (FEV1) Z-score, FEV1/FVC ratio, and forced expiratory flow between 25% and 75% of FVC (FEF25-75) Z-score.
  • Childhood hospitalization for respiratory exacerbations and higher bronchoalveolar lavage neutrophil elastase activity at age 5 predicted greater lung function decline in adolescence.
  • No factors examined predicted a decline in FVC Z-score.

Conclusions:

  • Preventing and treating respiratory exacerbations and managing neutrophilic inflammation in the early years of life are potential strategies to mitigate lung function decline in children with CF.
  • These early-life factors represent critical targets for future research and therapeutic interventions in pediatric CF care.
Abstract

Keywords:
ACFBAL studyAspergillusCF-FAB studyHospitalisationNeutrophilic inflammationRespiratory exacerbations

More Related Videos

Design and Development of a Model to Study the Effect of Supplemental Oxygen on the Cystic Fibrosis Airway Microbiome07:38

Design and Development of a Model to Study the Effect of Supplemental Oxygen on the Cystic Fibrosis Airway Microbiome

Published on: August 3, 2021

2.5K
Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis05:56

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

109

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,...
382
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...
231
Chronic Obstructive Pulmonary Disease-I: Introduction01:20

Chronic Obstructive Pulmonary Disease-I: Introduction

Chronic Obstructive Pulmonary Disease (COPD) is a long-lasting respiratory condition requiring continuous attention and care. It is a progressive lung disease that leads to breathing challenges due to airflow obstruction. It manifests as persistent respiratory symptoms and restricted airflow resulting from abnormalities in the airways and alveoli, usually due to long-term exposure to harmful particles or gases. COPD mainly consists of two primary conditions: emphysema and chronic bronchitis.
3.0K
Chronic Obstructive Pulmonary Disease-II: Pathophysiology01:20

Chronic Obstructive Pulmonary Disease-II: Pathophysiology

Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
3.2K
Factors Affecting Pulmonary Ventilation01:19

Factors Affecting Pulmonary Ventilation

Besides the pressure difference between the external environment and the lungs, the airflow rate and ease of pulmonary ventilation are also influenced by three other factors: surface tension of the fluid in the alveoli, compliance of the lungs, and airway resistance.
Alveolar Surface Tension
The alveolar fluid lines the luminal surface of the alveoli and exerts a force called surface tension. This force is caused by the polar water molecules in the liquid being more strongly attracted to each...
1.7K
COPD: Pathogenesis and Clinical Features01:20

COPD: Pathogenesis and Clinical Features

Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
649