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. 2022 Jun 22:10:926248.
doi: 10.3389/fped.2022.926248. eCollection 2022.

Time Free From Hospitalization in Children and Adolescents With Cystic Fibrosis: Findings From FEV1, Lung Clearance Index and Peak Work Rate

Simone Gambazza  1 , Alessandra Mariani  1   2 , Anna Brivio  1   2 , Federica Carta  1   2 , Chiara Blardone  1   2 , Saba Lisiero  1   2 , Maria Russo  2 , Carla Colombo  1   3
Affiliations

Time Free From Hospitalization in Children and Adolescents With Cystic Fibrosis: Findings From FEV1, Lung Clearance Index and Peak Work Rate

Simone Gambazza et al. Front Pediatr. .

Abstract

Background: An exercise test combined with a multiple breath washout nitrogen test (MBWN2) may offer a comprehensive clinical evaluation of cystic fibrosis (CF) disease in children with normal spirometry. The purpose of the present study is to explore whether information derived from spirometry, MBWN2, and exercise tests can help the CF multidisciplinary team to characterize time free from hospitalization due to pulmonary exacerbation (PE) in a cohort of pediatric patients with CF.

Methods: This prospective observational study was carried out at the Lombardia Region Reference Center for Cystic Fibrosis in Milano, Italy. In 2015, we consecutively enrolled children and adolescents aged <18 years with spirometry, MBWN2, and Godfrey exercise test performed during an outpatient visit.

Results: Over a median follow-up time of 2.2 years (interquartile range [IQR], 2.01; 3.18), 28 patients aged between 13.0 and 17.4 years were included. When lung functions were outside the normal range, 50% of patients were hospitalized 4 months after the outpatient visit, and their response to exercise was abnormal (100%). Half of the individuals with normal forced expiratory volume in the first second (FEV1) and abnormal lung clearance index (LCI) experienced the first hospital admission 9 months after the clinic visit, and 84.2% presented an abnormal response to exercise. Conversely, 15.8% had abnormal exercise responses when lung functions were considered normal, with half of the adolescents hospitalized at 11 months.

Conclusion: Maintaining ventilation homogeneity, along with a normal ability to sustain intense work, may have a positive impact on the burden of CF disease, here conceived as time free from hospitalization due to PE.

Keywords: cystic fibrosis; exercise tolerance; hospitalization; lung clearance index (LCI); lung function; pulmonary exacerbation.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Upper panel shows the relationship between ppWpeak (Wpeak), z-score of FEV1 and LCI. The curved lines represent nonparametric regressions. The lower panel shows correlation coefficients (rho). FEV1, forced expiratory volume in 1 s; LCI, lung clearance index at 1/40th of starting concentration.
Figure 2
Figure 2
Time at which children and adolescents experienced hospitalization due to pulmonary exacerbation (PE), stratified by FEV1 z-score and LCI at 1/40th of starting concentration. Group A identifies individuals with FEV1 ≥ LLN and LCI < ULN; group B individuals with FEV1 ≥ LLN and LCI ≥ ULN, whereas group C individuals with FEV1 < LLN and LCI ≥ ULN.
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