Related Experiment Video
Updated: Nov 26, 2025

Design and Development of a Model to Study the Effect of Supplemental Oxygen on the Cystic Fibrosis Airway Microbiome
Published on: August 3, 2021
Growth, Body Composition, and Lung Function in Prepubertal Children with Cystic Fibrosis Diagnosed by Newborn
Elizabeth Owen1, Jane E Williams2, Gwyneth Davies3
1Dietetics Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Insights
Newborn screening for cystic fibrosis (CF) identifies children with altered body composition (BC) and lower fat-free mass index (FFMI), particularly in girls. FFMI, not BMI, predicts lung function in these children.
Area of Science:
- Pediatric Pulmonology
- Body Composition Analysis
- Cystic Fibrosis Research
Background:
- Children with cystic fibrosis (CF) face risks of altered body composition (BC).
- Newborn screening (NBS) for CF may improve BC outcomes.
- This study examines BC in prepubertal children diagnosed via NBS.
Purpose of the Study:
- To investigate body composition (BC) in prepubertal children diagnosed with CF through newborn screening (NBS).
- To explore the relationship between BC and lung function (FEV1).
- To identify predictors of fat-free mass (FFM) and lung function.
Main Methods:
- Dual-energy x-ray absorptiometry (DXA) scans were used to assess whole-body (WB) and appendicular BC in 37 children with CF.
- Anthropometry and spirometry were performed, with results adjusted for height (FMI, FFMI).
- Linear regression analyzed predictors of forced expiratory volume in 1 second (FEV1).
Main Results:
- Children with CF diagnosed by NBS showed significantly lower weight and BC (WB FMI, WB FFMI) despite normal height, BMI, and FEV1.
- Gender differences were observed, with girls exhibiting lower weight, BMI, and BC.
- Whole-body fat-free mass index (WB FFMI) was the sole significant predictor of FEV1 (P = .016).
Conclusions:
- Gender-specific differences in growth and BC are evident in prepubertal children with CF diagnosed via NBS.
- Body composition assessment is recommended for these children, especially girls.
- Interventions targeting FFM optimization are suggested for improved outcomes.
Background:
Children with cystic fibrosis (CF) are at risk of altered body composition (BC). Newborn screening (NBS) may lead to improved BC outcomes. We investigated BC and its relationship with lung function in prepubertal children diagnosed with CF by NBS. Secondary aims explored predictors of fat-free mass (FFM) and lung function.
Methods:
Thirty-seven screened (non-meconium ileus) children with CF (20 boys) born 2007-2012 had a dual-energy x-ray absorptiometry scan at 5-8 years to determine whole-body (WB) and appendicular BC. Anthropometry was performed and routine spirometry recorded. Results were converted to z-scores, height-adjusted (fat mass index [FMI] and FFM index [FFMI]) and compared with population mean values. Predictors of forced expiratory volume in 1 second (FEV1 ) were assessed using linear regression.
Results:
Height, body mass index (BMI), and FEV1 were within normal limits, however, weight and BC were significantly low compared with reference data (weight, P = .03; WB FMI, P = .001; WB FFMI, P = .009). Gender differences were detected, with lower appendicular BC in boys and lower weight, BMI, and BC in girls. The association between FEV1 and WB FFMI (r = 0.38; P = .02) was stronger than with BMI (r = 0.29; P = .08). WB FFMI was the only significant predictor of FEV1 in a multivariable model (95% CI, 0.11-0.99; P = .016).
Conclusion:
In this NBS CF population, gender differences in growth and BC were apparent despite preserved lung function. These results support BC assessment in prepubertal children, particularly girls, with an opportunity to direct interventions to optimize FFM.
More Related Videos
05:56Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
Published on: August 29, 2025
08:00Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Related Concept Videos
Cystic Fibrosis: Pathogenesis
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,...
Cystic Fibrosis: Management
Sinus disease and chronic...
Pulmonary Function Tests
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...
Lung Capacity
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History