The relationship between body growth and pulmonary function in children with cystic fibrosis

Introduction: Cystic fibrosis (CF) is a complex disease that includes both pulmonary and gastrointestinal challenges, resulting in decreased weight. Pulmonary symptoms of CF are extremely variable. Greater body mass at an early age is associated with improved pulmonary function, but it is unknown at what age weight becomes predictive of pulmonary disease severity. The purpose of this study was to investigate the relationship between birth weight and pulmonary function in CF. Methods: Birth weight and pulmonary data were obtained. Linear regressions were used to examine the relationship between these two variables. A one-tailed t-test was used to compare birth weights between CF patients and the national average. Results: Birth weight was significantly lower in babies with CF and correlated with pulmonary disease at ages 6 and 10 years but not with age at which Pseudomonas aeruginosa colonization was observed. Discussion: These data suggest that CF growth deficiency has prenatal origins. Early nutritional intervention for babies with CF and a low birth weight is warranted to maximize pulmonary potential.

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What Is Most Suitable for Children With Cystic Fibrosis—The Relationship Between Spirometry, Oscillometry, and Multiple Breath Nitrogen Washout

Frontiers in Pediatrics ◽

10.3389/fped.2021.692949 ◽

2022 ◽

Vol 9 ◽

Author(s):

Magdalena Postek ◽

Katarzyna Walicka-Serzysko ◽

Justyna Milczewska ◽

Dorota Sands

Keyword(s):

Cystic Fibrosis ◽

Pulmonary Function ◽

Study Group ◽

Z Score ◽

Nitrogen Washout ◽

Significant Difference ◽

Function Test ◽

Test Sets ◽

The Relationship ◽

Function Testing

IntroductionIn cystic fibrosis (CF), pathological lung changes begin early in life. The technological progress currently gives many diagnostic possibilities. However, pulmonary function testing in children remains problematic.ObjectivesOur study aimed to correlate the results of impulse oscillometry (IOS) with those of multiple breath nitrogen washout (MBNW) in our pediatric CF population. We also compared those parameters between the groups with and without spirometric features of obturation.MethodsWe collected 150 pulmonary function test sets, including spirometry, IOS, and MBNW in patients with CF aged 12.08 ± 3.85 years [6–18]. The study group was divided into two subgroups: IA (without obturation) and IB (with obturation). We also compared Sacin, Scond, and oscillometry parameters of 20 patients aged 14–18 years who reached the appropriate tidal volume (VT) during MBNW.ResultsStatistical analysis showed a negative correlation between lung clearance index (LCI) and spimoetric parameters. Comparison of subgroups IA (n = 102) and IB (n = 48) indicated a statistically significant difference in LCI (p < 0.001) and FEV1z-score (p < 0.001), FEV1% pred (p < 0.001), MEF25z-score (p < 0.001), MEF50 z-score (p < 0.001), MEF75 z-score (p < 0.001), R5% pred (p < 0.05), and R20% pred (p < 0.01). LCI higher than 7.91 was found in 75.33% of the study group, in subgroup IB—91.67%, and IA−67.6%.ConclusionsLCI derived from MBNW may be a better tool than IOS for assessing pulmonary function in patients with CF, particularly those who cannot perform spirometry.

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