Clinical parameters of CF patients recruited in this study.

Patient ID, a specific number was assigned to each patient according to the chronology of their recruitment; Mutation, CFTR mutation for each patient was provided by the group of Pr. Taieb Messaoud at Children’s Hospital in Tunis. Confirmed CF diagnosis was established based on clinical symptoms with two positive sweat chloride tests, sequencing of the CFTR gene and identification of the CF-causing mutation; BMI, body mass index for each patient, defined as the body weight in kilograms divided by the square of height in meters; P. aeruginosa, colonization, chronicity of P. aeruginosa carriage (chronic, intermittent or undetectable); Mucoid P. aeruginosa, presence or absence of mucoid form of P. aeruginosa (Yes, presence; No, absence); ABPA, presence (Yes) or absence (No) of allergic bronchopulmonary aspergillosis; Bronchiectasis, absence (No) or presence of bronchiectasis in one, two (1 or 2 lobes) or more (≥ 2 lobes) lobes; Burkholderia cepacia, detection (Yes) or undetectable (No) of Burkholderia cepacia infection; Respiratory exacerbation, number of times patient was admitted in ICU for severe respiratory exacerbation; Cirrhosis, absence (No) or presence (Yes) of hepatic cirrhosis; Severity score, a severity score was calculated based on arbitrary scoring scheme provided in bottom table. (XLSX)