Table 2.

Clinical and metabolic characteristics of the subjects before starting Elexacaftor-Ivacaftor-Tezacaftor and after 12–18 months of treatment. Data are expressed as mean ± SD or median [IQR], unless otherwise specified. Abbreviations: BMI body mass index, FEV1 Forced Expiratory volume in the 1st second, FVC forced vital capacity. NGT normal glucose tolerance, INDET indeterminate glucose tolerance, IGT impaired glucose tolerance, CFRD cystic fibrosis related diabetes, DC derivative control, PC proportional control, ISR insulin secretion rate at 4, 5.5, 8, 11 and 15 mmol/L of plasma glucose, OGIS Oral Glucose Insulin Sensitivity.

Variables	Baseline	After 12−18 months of Exacaftor/Ivacaftor/Tezacaftor	p
Gender (M/F) n (%)	4 (80)/1 (20)	−	−
Age (years)	22.0 ± 7.4	23.14 ± 7.50	0.028
Pubertal status			1.00
Pre−pubertal n (%)	0 (0)	0 (0)
Pubertal n (%)	1 (20)	1 (20)
Post−pubertal n (%)	4 (80)	4 (80)
Height (Z−score)	−0.32 ± 1.5	−0.29 ± 1.47	0.48
Weight (Z−score)	−0.67 ± 0.71	−0.43 ± 0.53	0.18
BMI (kg × m−2)	19.92 ± 3.0	21.10 ± 2.49	0.07
BMI z−score	−0.90 ± 0.72	−0.44 ± 0.85	0.13
FEV1 (L)	1.67 ± 0.56	2.11 ± 0.57	0.023
FEV1% of predicted	39.0 ± 11.14	49.00 ± 11.27	0.041
FVC (L)	3.57 ± 0.98	4.17 ± 0.95	0.014
FVC% of predicted	72.67 ± 18.18	80.67 ± 10.07	0.287
Sweat chloride (mmol/L)	98.67 ± 15.04	30.33 ± 11.72	0.002
Glucose tolerance categories:			0.07
NGT n (%)	2 (40.0)	2 (40.0)
INDET n (%)	1 (20)	0 (0)
IGT n (%)	1 (20)	2 (40)
CFRD n (%)	1 (20)	1 (20)
DC ((pmol·m−2·BSA) /(mmol·L −1 ·min−1))	400.7 ± 169.7	807.8 ± 341.8	0.35
PC (pmol/min/m2 BSA)
ISR4	61.7 [45.2−76.4]	66.8 [56.3−89.3]	0.35
ISR5.5	111.4 [79.3−167.9]	105.3 [81.6−145.1]	0.89
ISR8	258.3 [221.2−475.4]	259.5 [220.3−522.9]	0.69
ISR11	523.1 [382.9−844.5]	484.0 [387.1−976.3]	0.50
ISR15	882.6 [595.0−1336.5]	804.9 [598.7−1580.9]	0.69
PCadj ((pmol/L)·(mmol/L))	81.6 ± 28.4	88.2 ± 25.3	0.89
Insulin Clearance (L/min)	0.95 [0.83−1.14]	0.92 [0.77−1.15]	0.50
OGIS (µmol·min−1·m−2 BSA)	449.2 ± 44.4	396.2 ± 25.4	0.14