Related Experiment Video

Updated: Feb 9, 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

586

Gas exchanges in children with cystic fibrosis or primary ciliary dyskinesia: A retrospective study

Marilyn Fuger1, Camille Aupiais2, Guillaume Thouvenin3

  • 1AP-HP, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, Paris, France.

Respiratory Physiology & Neurobiology
|January 26, 2018
PubMed

Insights

Children with primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) have similar initial blood gas impairments. However, PCD patients show slower deterioration over time, highlighting the benefit of early diagnosis for managing pulmonary health.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Genetic Diseases

Background:

  • Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are chronic childhood respiratory diseases.
  • Both conditions can lead to bronchiectasis and pulmonary function impairment.
  • Understanding differences in disease progression, particularly gas exchange, is crucial for patient management.

Purpose of the Study:

  • To compare the progression of blood gas exchange in children with PCD versus CF.
  • To investigate if age-related changes in blood gases differ between these two conditions.
  • To assess the impact of early diagnosis on long-term respiratory outcomes.

Main Methods:

  • Retrospective study analyzing repeated measurements in children with PCD and CF.
  • Statistical comparisons included Wilcoxon-Mann-Whitney and Chi-squared tests.
  • A mixed-effects linear model, adjusted for age, was used to evaluate disease impact on blood gases (PaO2, PaCO2, PaO2-PaCO2 ratio).

Main Results:

  • Initial spirometry and blood gas levels were comparable between 42 PCD and 73 CF children.
  • Both groups showed a significant age-related decrease in PaO2 and PaO2-PaCO2 ratio (P < 0.01).
  • PaCO2 increased more significantly with age in CF patients (P = 0.02), though generally remained within normal limits.

Conclusions:

  • Gas exchange is initially similarly impaired in children with PCD and CF.
  • Blood gas exchange deteriorates more slowly over time in children with PCD compared to CF.
  • Early diagnosis in PCD may contribute to better long-term management of gas exchange abnormalities.

Keywords:
Childhood respiratory diseaseClinical aspects of cystic fibrosisPrimary ciliary dyskinesiaPulmonary gas exchange

More Related Videos

High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia05:32

High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia

Published on: January 19, 2022

5.2K
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

2.9K

Related Concept Videos

Gas Exchange and Transport01:20

Gas Exchange and Transport

Gas exchange, the intake of molecular oxygen (O2) from the environment and the outflow of carbon dioxide (CO2) into the environment, is necessary for cellular function. Gas exchange during respiration occurs largely via the movement of gas molecules along pressure gradients. Gas travels from areas of higher partial pressure to areas of lower partial pressure. In mammals, gas exchange occurs in the alveoli of the lungs, which are adjacent to capillaries and share a membrane with them.
77.1K
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,...
891
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...
547
Physical Principles Governing Gas Exchange01:16

Physical Principles Governing Gas Exchange

Gas behavior plays a vital role in understanding bodily processes such as external and internal respiration. External respiration involves the diffusion of oxygen into the blood and carbon dioxide out of it in the lungs. In contrast, internal respiration happens in body tissues, where these gases move in opposite directions.
Gas Laws Governing Respiration
The behavior of gases is guided by Dalton's Law of partial pressures and Henry's Law.
Dalton's Law asserts that the total...
3.9K
Social Exchange Theory02:06

Social Exchange Theory

We have discussed why we form relationships, what attracts us to others, and different types of love. But what determines whether we are satisfied with and stay in a relationship? One theory that provides an explanation is social exchange theory. According to social exchange theory, we act as naïve economists in keeping a tally of the ratio of costs and benefits of forming and maintaining a relationship with others (Rusbult & Van Lange, 2003).
40.8K
Mechanism of Ciliary Motion01:05

Mechanism of Ciliary Motion

The ciliary structures were first seen in 1647 by Antonie Leeuwenhoek while observing the protozoans. In lower organisms, these appendages are responsible for cell movement, while in higher organisms, these appendages help in the movement of the extracellular fluids within the body cavities.
The cilia are made up of microtubules in a 9+2 arrangement, with nine microtubule doublet ring bundles, surrounding a pair of central singlet microtubule bundles. The doublet microtubule bundles are...
5.2K