Association of body composition and lung function in children with cystic fibrosis

C C Pedreira1, R G D Robert, V Dalton

  • 1Department of Endocrinology and Diabetes, Royal Children's Hospital, Parkville, Victoria, Australia.

Pediatric Pulmonology
|January 26, 2005
PubMed

Insights

In children with cystic fibrosis (CF), body mass index (BMI) is strongly linked to lung function. Maintaining a healthy BMI is crucial for preserving pulmonary function in pediatric CF patients.

Area of Science:

  • Pediatric Pulmonology
  • Biomedical Science
  • Clinical Nutrition

Background:

  • Cystic Fibrosis (CF) survival has improved, with key goals including slowing lung function decline and ensuring normal growth.
  • Dual-energy X-ray absorptiometry (DEXA) is utilized to evaluate bone mineral density and body composition in pediatric CF patients.

Purpose of the Study:

  • To investigate the association between body composition and pulmonary function in children with Cystic Fibrosis.
  • To determine if body composition metrics correlate with lung function in pediatric CF patients.

Main Methods:

  • Fifty pediatric subjects with Cystic Fibrosis (CF) underwent Dual-energy X-ray absorptiometry (DEXA) for body composition analysis.
  • Lung function was assessed using spirometry, with Forced Expired Volume in 1 second percent predicted (FEV1% predicted) being a key metric.
  • Body Mass Index (BMI) was calculated as weight/height² (kg/m²).

Main Results:

  • A significant positive association was found between FEV1% predicted and BMI (R=0.59, P=0.0001).
  • Fat-free mass showed a statistically significant positive correlation with pulmonary function tests (R=0.30, P=0.03).
  • While fat mass trended positively with pulmonary function, the association did not reach statistical significance.

Conclusions:

  • In children with CF and mild lung disease, BMI demonstrated a stronger association with pulmonary function compared to fat and fat-free mass.
  • These findings highlight the importance of BMI as an indicator of pulmonary status in pediatric CF patients.
  • Further research may explore targeted nutritional interventions to optimize body composition and lung health in this population.

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: 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
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
Pulmonary Function Tests01:25

Pulmonary Function Tests

Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
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...