Respiratory system parameters in children with low severity cystic fibrosis: is there early involvement in relation

Related Experiment Video

Updated: Jul 8, 2025

Design and Development of a Model to Study the Effect of Supplemental Oxygen on the Cystic Fibrosis Airway Microbiome
07:38

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

Published on: August 3, 2021

2.4K

Gabriela Castilhos Ducati1, Juliana Cardoso1, Elaine Paulin Ferrazeane1

  • 1Universidade do Estado de Santa Catarina, Florianópolis, SC, Brasil.

Revista Paulista De Pediatria : Orgao Oficial Da Sociedade De Pediatria De Sao Paulo
|December 13, 2023
PubMed

Insights

Children with cystic fibrosis exhibit obstructive ventilatory disorders and compromised peripheral airways compared to healthy peers. These findings highlight early pulmonary function changes in cystic fibrosis patients.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Genetics

Background:

  • Cystic fibrosis (CF) is a genetic disorder impacting multiple organs, primarily the lungs.
  • Early detection of pulmonary dysfunction is crucial for managing CF progression.
  • Understanding respiratory mechanics in pediatric CF is essential for targeted interventions.

Purpose of the Study:

  • To compare pulmonary function and respiratory mechanics in children with and without cystic fibrosis.
  • To identify specific patterns of lung impairment in pediatric CF patients.

Main Methods:

  • Cross-sectional analytical study involving 110 children (6-13 years), 55 with cystic fibrosis (CFG) and 55 healthy (HSG).
  • Patients were age and sex-matched.
  • Pulmonary function assessed using spirometry and impulse oscillometry; CFG medical records reviewed for disease severity (Schwachman-Doershuk Score - SDS), genotype, and colonization.

Main Results:

  • Significant differences (p<0.05) in most pulmonary function and respiratory mechanics parameters were observed between CFG and HSG groups.
  • In CFG, 58.2% had excellent SDS, 49.1% carried the ΔF508 genotype, and 67.3% had pathogen colonization.
  • Obstructive ventilatory patterns were evident in the CFG group.

Conclusions:

  • Children with cystic fibrosis demonstrate obstructive ventilatory disorders.
  • Peripheral airway compromise is a key finding in pediatric CF.
  • These results underscore the early onset of pulmonary function and mechanics alterations in cystic fibrosis.
Abstract

More Related Videos

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections06:57

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

9.2K
Noninvasive Sampling of Mucosal Lining Fluid for the Quantification of In Vivo Upper Airway Immune-mediator Levels05:31

Noninvasive Sampling of Mucosal Lining Fluid for the Quantification of In Vivo Upper Airway Immune-mediator Levels

Published on: August 7, 2017

10.4K

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,...
252
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...
170
Physical Assessment of the Respiratory Tract II: Inspection01:27

Physical Assessment of the Respiratory Tract II: Inspection

Physical assessment of the respiratory tract through inspection is a crucial step in understanding the patient's respiratory health. It provides insights into the functioning of the respiratory system, the musculoskeletal structure, and even the patient's nutritional status. This comprehensive approach involves observing several vital aspects: chest configuration, breathing patterns, respiratory rates, skin color, and use of accessory muscles.
Chest Configuration
The chest configuration...
300
Assessment of Respiration01:23

Assessment of Respiration

The respiratory system's basic structures and primary functions lay the foundation for nurses' comprehensive respiratory assessments. This assessment includes subjective and objective data to gauge the patient's respiratory health.
Subjective Assessment: Nurses interview the patient to gather information directly during the subjective assessment. It includes questions about the individual's medical history, medications, and symptoms, focusing on past respiratory conditions like...
1.1K
Respiratory System Abnormal Finding II: Palpation and Auscultation01:31

Respiratory System Abnormal Finding II: Palpation and Auscultation

In assessing respiratory abnormalities, palpation and auscultation are critical tools for detecting and interpreting various pathophysiological changes. These techniques provide insight into underlying disorders by evaluating tactile sensations and sounds produced by the respiratory system.
Palpation Findings
During a respiratory assessment, palpation can reveal several vital abnormalities:
477
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies01:27

Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies

Assessing and diagnosing Chronic Obstructive Pulmonary Disease (COPD) involves a detailed approach that includes a comprehensive review of medical history, physical examination, and a variety of diagnostic tests. This thorough evaluation is essential to ensure an accurate diagnosis and guide effective management strategies.
Medical History
2.5K