Related Experiment Video

Updated: Aug 13, 2026

Isolation of Lung Retinoid-Containing Cells by Cell Sorting
07:47

Isolation of Lung Retinoid-Containing Cells by Cell Sorting

Published on: April 11, 2025

Vitamin A and E deficiency and lung disease in infants with cystic fibrosis

J E Bines1, H D Truby, D S Armstrong

  • 1Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Victoria, Australia.

Journal of Paediatrics and Child Health
|January 10, 2006
PubMed

Insights

Vitamin A and E deficiency is common in cystic fibrosis (CF). This study found no link between low vitamin levels in infants and early lung disease or inflammation in CF patients.

Area of Science:

  • Pediatric Pulmonology
  • Nutritional Immunology
  • Cystic Fibrosis Research

Background:

  • Vitamin A and E deficiencies are prevalent in cystic fibrosis (CF) patients.
  • These vitamins possess immunomodulatory functions, potentially impacting disease progression.
  • Early detection of nutritional status is crucial for managing CF complications.

Purpose of the Study:

  • To investigate the association between decreased serum vitamin A and E levels in infants with CF and the development of early lung disease.
  • To determine if low vitamin levels correlate with lower airway inflammation in young CF patients.
  • To assess the impact of vitamin supplementation on these parameters over the first year of life.

Main Methods:

  • A post-hoc analysis of prospectively collected data from 39 infants diagnosed with CF via newborn screening.
  • Assessment included CF genotype, nutritional status, pancreatic function, and serum vitamin A (retinol) and E (alpha-tocopherol) levels at diagnosis.
  • Pulmonary status was evaluated using clinical assessments, chest radiography, and analysis of inflammatory markers and bacterial counts in bronchial lavage fluid, with repeat assessments at 12 months.

Main Results:

  • At diagnosis, 51% of CF infants had low serum retinol and 24% had low alpha-tocopherol.
  • Serum retinol levels correlated with dietary energy intake at diagnosis.
  • Despite normalization of vitamin levels after supplementation, respiratory symptoms, radiographic scores, and inflammatory indices in bronchial lavage fluid worsened over the first year. No association was found between initial vitamin levels and subsequent airway inflammation.

Conclusions:

  • This study found no evidence in a CF birth cohort to suggest that vitamin A or E deficiency contributes to the development of lung disease or airway inflammation during infancy.
  • Further research may be needed to explore other factors influencing early lung disease in CF.
  • Vitamin supplementation normalized levels but did not prevent disease progression in this cohort.
Abstract

More Related Videos

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

Real-Time, Semi-Automated Fluorescent Measurement of the Airway Surface Liquid pH of Primary Human Airway Epithelial Cells10:18

Real-Time, Semi-Automated Fluorescent Measurement of the Airway Surface Liquid pH of Primary Human Airway Epithelial Cells

Published on: June 13, 2019

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...
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.
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...
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 I: Introduction01:23

Chronic Obstructive Pulmonary Disease I: Introduction

Chronic obstructive pulmonary disease is a common, preventable, and treatable respiratory disorder characterized by persistent symptoms and progressive airflow limitation. This limitation results from a combination of small-airway disease (obstructive bronchiolitis) and parenchymal destruction (emphysema), both driven by chronic inflammation from exposure to harmful particles or gases.The disease includes two main pathological entities: emphysema, marked by destruction of alveolar walls and...