Giulia Santini

Meyer Children's Hospital

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Giovanni Taccetti

Meyer Children's Hospital

Chiara Castellani

Meyer Children's Hospital

Cristina Fevola

Meyer Children's Hospital

Vito Terlizzi

Meyer Children's Hospital

Eleonora Masi

Meyer Children's Hospital

Béatrice Ferrari

Meyer Children's Hospital

Diletta Innocenti

Meyer Children's Hospital

Anna Silvia Neri

Meyer Children's Hospital

Paolo Bonomi

National Institute of Statistics

Silvia Bresci

Azienda Ospedaliero-Universitaria Careggi

Cooperative Institutions

Meyer Children's Hospital

University of Florence

Meyer Children's Hospital

Royal Manchester Children's Hospital

Nicolae Testemițanu State University of Medicine and Pharmacy

Temple Street Children's University Hospital

Azienda Ospedaliero-Universitaria Careggi

Istituti di Ricovero e Cura a Carattere Scientifico

Indiana University – Purdue University Indianapolis

Cardiff University

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Lung clearance index in children with cystic fibrosis previously diagnosed with CRMS/CFSPID: A monocentric prospective experience

Pediatric Pulmonology (2023)

Vito Terlizzi Cristina Fevola Béatrice Ferrari Chiara Castellani Giulia Santini

Abstract Introduction No data are available on the values and role of lung clearance index (LCI) in cystic fibrosis (CF) Screen Positive Inconclusive Diagnosis (CFSPID) progressed to CF diagnosis (CFSPID > CF). This study aimed to assess the value of the LCI in correctly predicting the progression of CFSPID to CF. Methods This is a prospective study carried out at the CF Regional Center of Florence, Italy from September 1, 2019. We compared LCI values in children with CF diagnosed for positive newborn screening (NBS), CFSPID or CFSPID > CF for pathological sweat chloride (SC). The Exhalyzer‐D (EcoMedics AG, Duernten, Switzerland, software version 3.3.1) was used to conduct the LCI tests, every 6 months on stable children. Results Forty‐two cooperating children were enrolled (mean age at LCI tests: 5.4 years, range: 2.7−8.7): 26 (62%) had CF, 8 (19%) were CFSPID > CF for positive SC, while 8 (19%) kept the CFSPID label at last LCI test. The mean LCI value for patients with CF (7.39; 5.98−10.24) was statistically higher compared to both the mean LCI in the CFSPID > CF (6.62; 5.69−7.58) and in CFSPID (6.56; 5.64−7.21). Conclusions Most of asymptomatic CFSPID or progressed to CF have normal LCI. Further data on the longitudinal course of LCI during follow up of CFSPID and on larger cohorts is needed.

10.1002/ppul.26442

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Respiratory infections of upper and lower airways in patients with cystic fibrosis.

Cristina Fevola Silvia Campana Daniela Dolce N. Ravenni Chiara Bianchimani

Respiratory infections represent the leading cause of lung damage in CF patients. A strong correlation has been shown between the flora colonizing the upper (UAW) and lower airways (LAW). We analyzed UAW and LAW to evaluate the role of bacterial infections of paranasal sinuses in the development of subsequent lung infections. Paired samples of UAW and LAW were screened performing microbiological analyses. UAW specimens were collected by nasal lavage. LAW specimens were collected as throat swab or sputum. Between December 2020 and March 2022, 109 adult patients in total were enrolled, including 83 (76.1%) not chronically colonized by Pseudomonas aeruginosa (PA) (Group A: 62.7% male; median age 29y; range 18-58), and 26 (23.9%) transplanted subjects (Group B: 61.5% male; median age 39y; range 19-60). Overall, 187 paired samples of UAW and LAW were screened, 141 for Group A, and 46 for Group B. The most prevalent bacterium in group A was Staphylococcus aureus (SA) in both UAW (41.1%) and LAW (69.5%) cultures (p<.05), followed by Enterobacteriaceae (UAW 17.7%, LAW 14.2%), Achromobacter xylosoxidans (UAW 3.5%, LAW 7.8%), methicillin-resistant SA (UAW 4.3%, LAW 3.5%), and PA (UAW 1.4%, LAW 6.4%; p<.05). Compared to group A, group B exhibited a higher prevalence of PA (UAW 36.1%, LAW 52.8%), a lower presence of SA (UAW 41.7%, LAW 44.4%) with lower bacterial diversity. The two groups of patients showed different airways microbiota. Upper airways could play a role as reservoir in the initial phases of lung disease and progression towards chronicity. In transplant patients the high PA prevalence in UAW could infect the allografts, conditioning the organ rejection. This work was supported by the Tuscany Region

Group A

Group B

10.1183/23120541.acf-2023.6

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