Related Experiment Video

Updated: Aug 18, 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

Developmental paradigm for early features of cystic fibrosis

Janet E Larson1, J Craig Cohen

  • 1Joint Laboratory of Molecular Therapeutics, USA. jlarson@ochsner.org

Pediatric Pulmonology
|April 15, 2005
PubMed

Insights

Cystic fibrosis (CF) involves abnormal lung development before birth, not just infection. The cystic fibrosis transmembrane regulator (CFTR) plays a key role in fetal lung development, explaining early disease paradoxes.

Area of Science:

  • Pulmonary Medicine
  • Developmental Biology
  • Genetics

Background:

  • Cystic fibrosis (CF) is a progressive genetic disorder.
  • The lungs are typically considered normal at birth in CF patients.
  • Recurrent infections are thought to cause lung injury in CF.

Purpose of the Study:

  • To review the role of the cystic fibrosis transmembrane regulator (CFTR) in lung development.
  • To examine early functional and structural lung abnormalities in CF.
  • To explain disease paradoxes through disrupted fetal development.

Main Methods:

  • Literature review focusing on CFTR expression and function during fetal lung development.
  • Analysis of existing evidence on early CF lung changes.
  • Synthesis of data to explain disease manifestations.

Main Results:

  • The cystic fibrosis transmembrane regulator (CFTR) is highly expressed in fetal lung tissues.
  • Evidence suggests the CF lung is abnormal prior to clinical infection.
  • Disrupted CFTR function impacts regulatory cascades during lung development.

Conclusions:

  • Early alterations in fetal lung development due to CFTR dysfunction contribute to CF pathology.
  • Understanding these developmental changes can explain clinical paradoxes in CF.
  • Targeting developmental pathways may offer new therapeutic strategies.

More Related Videos

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study08:00

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

Rectal Organoid Morphology Analysis (ROMA): A Diagnostic Assay in Cystic Fibrosis07:56

Rectal Organoid Morphology Analysis (ROMA): A Diagnostic Assay in Cystic Fibrosis

Published on: June 10, 2022

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...
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features01:24

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
Chronic Obstructive Pulmonary Disease II: Emphysema01:23

Chronic Obstructive Pulmonary Disease II: Emphysema

Emphysema, a major phenotype of chronic obstructive pulmonary disease (COPD), is characterized by irreversible destruction of alveolar walls and permanent enlargement of distal airspaces. Unlike chronic bronchitis, which primarily affects the airways, emphysema predominantly involves the lung parenchyma, where structural damage leads to airflow limitation.PathophysiologyIt most commonly results from prolonged exposure to cigarette smoke and other toxic gases, particularly cigarette smoke.