Table 1.

Clinical and electrophysiological data of the two boys bearing p.Phe508del and the p.[Arg74;Val201Met;Asp1270Asn] complex allele at the date of intestinal current measurements (ICM) assessment compared to non‐cystic fibrosis (CF) subjects

	Subject 1	Subject 2	Non‐CF (n = 68; median [inner quartiles, range])a
Current age (months)	108	82
Age at the date of ICM (months)	36	63
Age at diagnosis (months)	25	29
Cause of diagnosis	respiratory	familiarity
Pancreatic statusb	PS	PS
FEV1 (% predicted)	109	113
Lung clearance index	8.0	7.8
Sweat chloride (mmol/L)	42	45
ICM (µA/cm2): response to
IBMX/forskolin	37 ± 18	33 ± 25	31 (20–54; 10–104)
Carbachol	14 ± 2	81 ± 46	77 (43–144; 15–250)
Histamine	13 ± 4	49 ± 31	72 (37–125; 14–250)

^aOwn in‐house data due to the lack of published multicenter reference data for Standard Operating Procedure 2.7.

^bPancreatic status = exocrine pancreatic sufficient.