Table 4 Current age and clinical variables in the different patient groups

	CF-PI + CF-PS	CF-PI	CF-PS	CFTR dysfunction	CF unlikely	CF vs CFTR dysfunction	CF-PI vs CF-PS	CF-PS vs CFTR dysfunction	CF-PS vs CF unlikely	CFTR dysfunction vs CF unlikely
Subjects (n)	165	62	103	59	46	NS	NS	NS	NS	NS
Current age (years)†	19.7 (13.4–29.5)	19.5 (10.5–25.7)	19.8 (13.5–34.5)	19.3 (13.7–34.0)	16.6 (9.5–28.5)
Gender (M/F)	89/76	39/23	50/53	26/33	24/22	0.19	0.07	0.58	0.68	0.41
Age at time of diagnosis (years)†	3.0 (0.3–10.7)	0.2 (0.1–0.9)	7.4 (2.7–19.6)	11.3 (5.8–24.6)	13.9 (4.9–24.5)	<0.001	<0.001	0.03	0.01	0.60
Sweat chloride (mmol/l)†	96.0 (78.0–103.0)	102.0 (96.8–110.0)	85.0 (73.0–98.9)	45.7 (38.8–53.9)	40.3 (36.0–50.1)	<0.001	<0.001	<0.001	<0.001	0.05
Z-scores*
For weight	−0.08 (1.25)	−0.40 (1.04)	0.10 (1.33)	0.09 (0.98)	−0.53 (1.65)	0.64	0.05	0.99	0.14	0.16
For height	−0.09 (1.03)	−0.12 (0.97)	−0.07 (1.06)	0.13 (1.03)	−0.26 (1.32)	NS	NS	NS	NS	NS
For BMI	−0.16 (1.30)	−0.46 (1.16)	0.02 (1.36)	−0.08 (1.03)	−0.55 (1.80)	0.95	0.09	0.99	0.29	0.51
Most recent FEV₁% predicted†	78 (60–94)	73 (52–87)	83 (62–97)	89 (64–105)	91 (75–106)	0.02	0.02	0.15	0.03	0.66
Recurrent LRTI‡	83	100	71	71	44	0.06	<0.001	0.99	0.005	0.01
Sputa‡	66	86	54	48	42	0.02	0.001	0.46	0.25	0.60
P aeruginosa colonisation‡	39	53	30	17	5	0.002	0.004	0.06	<0.001	0.07
S aureus or P aeruginosa isolation‡	86	100	78	65	14	<0.001	<0.001	0.05	<0.001	<0.001
Clubbing‡	51	71	36	27	2	0.003	<0.001	0.26	<0.001	0.001
ABPA‡	15	21	11	10	2	0.38	0.08	0.89	0.11	0.14
Nasal polyposis‡	44	82	21	14	18	<0.001	<0.001	0.22	0.66	0.56
Pancreatitis‡	9	2	14	14	7	0.31	0.01	0.97	0.27	0.34
MI/DIOS‡	21	42	8	0	0	<0.001	<0.001	0.05	0.11	0.99
Liver disease/PTH/CL‡	15	19	13	2	0	0.007	0.24	0.04	0.04	0.99

Data are presented as *mean (SD), †median (interquartile range) or ‡percentage unless otherwise stated.
CF-PI, sweat chloride concentration >60 mmol/l and pancreatic insufficiency; CF-PS, sweat chloride concentration >60 mmol/l and pancreatic sufficiency; CFTR dysfunction, sweat chloride concentration 30–60 mmol/l and two mutations identified and/or abnormal nasal potential difference (NPD); CF unlikely, patients with an intermediate sweat chloride concentration but no detection of two CFTR mutations and/or normal NPD.
ABPA, allergic bronchopulmonary aspergillosis; CL, choledocolithiasis; DIOS, distal intestinal obstruction syndrome; FEV₁, forced expiratory volume in 1 s; LRTI, lower respiratory tract infection; MI, meconium ileus; PTH, portal hypertension; P aeruginosa, Pseudomonas aeruginosa; S aureus, Staphylococcus aureus.
p Values ⩽0.05 are shown in bold type. The H-values for age at diagnosis, sweat chloride value and for FEV₁% predicted were all significant at the level of 0.001. However, the F-value for height (ANOVA) and the H-value for current age (Kruskal-Wallis) were not significant.