Lung clearance index in children with non-cystic fibrosis, non-primary ciliary dyskinesia bronchiectasis and in a healthy population

Hines, Christopher (2024). Lung clearance index in children with non-cystic fibrosis, non-primary ciliary dyskinesia bronchiectasis and in a healthy population. University of Birmingham. M.D.

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Abstract

Introduction:
Non-CF, non-PCD bronchiectasis in children is a chronic, suppurative lung disease diagnosed by high resolution computed tomography (HRCT) imaging of the lungs. Spirometry can be abnormal in bronchiectasis but has been shown to be insensitive to early disease in other related conditions such as cystic fibrosis. Lung clearance index (LCI) could have a role in assessment of the disease.
Lung clearance index is calculated from multiple breath washout tests. There are limited data on the normative values expected using some devices, in a range of ages, and between ethnic groups.
The aim was to establish normative values in children aged between 6 years and 12 years, to investigate differences between ethnic groups, and to establish the relationship between lung clearance index and other measures of disease in children with bronchiectasis.
Methods:
Healthy children were recruited from a range of settings and reviewed to ensure no previous lung disease. Children with bronchiectasis diagnosed on HRCT were recruited from the outpatient service of Birmingham Children's Hospital.
All participants performed lung function tests including LCI and spirometry. Basic demographic data was collected.

Results:
72 healthy children and 13 children with bronchiectasis were recruited and performed lung function. All children with disease were able to complete testing, 70.8% of healthy children were able to perform lung clearance index.
The mean LCI of healthy children was 7.19 (0.6 SD), children with bronchiectasis had significantly higher LCI (9.51, 2.2 SD, p<0.0001). There was no difference between healthy children from White-British and Indian Subcontinent ethnicity (7.04 vs. 7.20, p=0.33). LCI in children with bronchiectasis correlated with HRCT scores, FEV1, FVC and MMEF. LCI was also more sensitive to disease than these spirometry metrics.
Conclusion:
LCI is feasible in children (both inexperienced and experienced in previous lung function testing). We have demonstrated a normative value for our cohort and have shown no difference between ethnic groups. LCI was more sensitive to disease and correlated well with other markers of disease highlighting a potential role for LCI in the monitoring of non-CF, non-PCD bronchiectasis and as a potential therapeutic outcome measure.

Type of Work:

Thesis (Higher Doctorates > M.D.)

Award Type:

Higher Doctorates > M.D.

Supervisor(s):