Lung clearance index short-term variability in cystic fibrosis: a pre-post pulmonary exacerbation study

Matteo De Marchis; Enza Montemitro; Alessandra Boni; Alessandra Federici; Daniele Di Giovanni; Luca Cristiani; Renato Cutrera; Alessandro G Fiocchi

doi:10.1186/s13052-023-01574-w

Lung clearance index short-term variability in cystic fibrosis: a pre-post pulmonary exacerbation study

Ital J Pediatr. 2024 Jan 17;50(1):6. doi: 10.1186/s13052-023-01574-w.

Authors

Matteo De Marchis^{1

2}, Enza Montemitro³, Alessandra Boni³, Alessandra Federici³, Daniele Di Giovanni^{4

5}, Luca Cristiani³, Renato Cutrera³, Alessandro G Fiocchi⁶

Affiliations

¹ Pneumology and Cystic Fibrosis Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy. matteo.demarchis@opbg.net.
² University of Magna Graecia of Catanzaro, Calabria, Italy. matteo.demarchis@opbg.net.
³ Pneumology and Cystic Fibrosis Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
⁴ Industrial Engineering Department, University of Rome Tor Vergata, Rome, Italy.
⁵ Unicamillus, Saint Camillus International University of Health Sciences, Rome, Italy.
⁶ Allergy Division, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.

Abstract

Background: Multiple Breath washout (MBW) represents an important tool to detect early a possible pulmonary exacerbation especially in Cystic Fibrosis (CF) disease. Lung clearance index (LCI) is the most commonly reported multiple breath washout (MBW) index and in the last years was used as management measure for evaluation. Our aim was to analyze clinical utility of LCI index variability in pulmonary exacerbation in CF after intravenous (IV) antibiotic therapy.

Methods: A single-center study was conducted at CF Unit of Bambino Gesù Children's Hospital among hospitalized > 3 years patients for pulmonary exacerbations and treated with antibiotic IV treatment for 14 days. MBW and spirometry were evaluated within 72 h of admission to hospital and at the end of hospitalization. Descriptive analysis was conducted and correlations between quantitative variables were investigated.

Results: Fifty-seven patients (M22/F35) with an average age 18.56 (± 8.54) years were enrolled. LCI2.5 was significantly reduced at the end of antibiotic treatment in both pediatric and adult populations with an average reduction of -6,99%; 37/57 patients denoted an improvement, 20/57 are stable or worsened in LCI2.5 values and 4/57 (7.02%) had a significant deterioration (> 15%) at end of treatment. On the contrary a significative elevation of FEV1 and FVC were found, respectively of + 7,30% and of + 5,46%. A positive good correlection among LCI 2.5 and Scond (rho = + 0,615, p = 0.000) and LCI 2.5 and Sacin (rho = + 0,649, p = 0.000) and a negative strong correlation between FEV1 and LCI 2.5 were found in post treatment period. A similar modification of LCI 2.5 and FEV1 was noticed in both adult and pediatric population.

Conclusions: LCI may have a role in the routine clinical care of both adult and pediatric CF patients as a good tool to assess response to IV antibiotic end-therapy in the same way as FEV1.

Keywords: Cystic fibrosis; Intravenous antibiotic therapy; Lung clearance index; Multiple breath washout; Spirometry pulmonary exacerbation.

MeSH terms

Adolescent
Adult
Anti-Bacterial Agents / therapeutic use
Child
Cystic Fibrosis* / diagnosis
Cystic Fibrosis* / drug therapy
Forced Expiratory Volume / physiology
Humans
Lung
Respiratory Function Tests

Substances

Anti-Bacterial Agents

Grants and funding

Ministero della Salute/Ministero della Salute