. Author manuscript; available in PMC: 2017 Nov 6.

Published in final edited form as: Lancet Respir Med. 2016 Jun 10;4(8):636–645. doi: 10.1016/S2213-2600(16)30105-9

Table 1.

Baseline characteristics of the two cohorts.

	Derivation Cohort						Validation Cohort

	Total (n=74)	PA:A ≤ 1 (n=37)	PA:A >1 (n=37)	Point Estimate	95%CI	P-value	All (n=190)	PA:A≤1 (n=101)	PA:A>1 (n=89)	Point Estimate	95%CI	P-value

Age, years^†	28±8	29±10	26±6	−3	−6.9–0.9	0.06	29±9	30±11	26±8	−4	−7 – −2	<0.001

White race	72/74	37/37	35/37	−5%	−18 – 5	0.16	190/190	101/101	89/89	n/a	n/a	n/a
	(97%)	(100%)	(95%)				(100%)	(100%)	(100%)

Male sex	42/74	22/37	20/37	−5%	−27 – 16	0.64	97/190	54/101	43/89	−5%	−20 – 9	0.56
	(57%)	(59%)	(54%)				(51%)	(53%)	(48%)

BMI, kg/m²	22.0±2.	22.6±2.	21.5±2.3	−1.1	−2.3– 0.1	0.06	21.2±3.6	21.4±3.9	20.9±3.3	−0.5	−1.7– 0.5	0.27

Pseudomonas positive	31/74	17/37	14/37	−8%	−29 – 14	0.49	166/190	82/101	84/89	13%	4 – 22	0.008
	(42%)	(46%)	(38%)				(87%)	(81%)	(94%)

Functional CFTR genotype^a	3/74	2/37	1/37	−2%	−15 − 9	0.56	11/164	7/84 (8%)	4/80 (5%)	−3%	−12 – 4	0.37
	(4%)	(5%)	(3%)				(7%)

Sweat chloride, mmol/L	95.5±16.7	90.4±19.9	100.5±10.9	10.1	2.5–17.7	0.017	97.9±25.2	88.2±23.1	105.9±24.7^b	17.7	−0.6 – 36	0.057

FEV₁% predicted	57±14	58±15	56±12	−2	−8 –4	0.38	50±21	50±22	50±20	0	−6 – 6	0.86

FEV₁/FVC	0.64±0. 10	0.64±0. 11	0.65±0.10	0.01	−0.01– 0.03	0.67	0.62±0.24	0.63±0.31	0.61±0.13	−0.02	−0.09–0.05	0.95

PA, cm	2.55±0. 30	2.51±0. 34	2.59±0.25	0.08	0.01–0.15	0.032	2.57±0.32	2.48±0.28	2.67±0.33	0.19	0.10–0.28	<0.001

A, cm	2.57±0. 39	2.77±0. 38	2.36±0.28	−0.41	−0.57–−0.26	<0.001	2.59±0.34	2.72±0.32	2.45±0.30	−0.27	−0.36–−0.19	<0.001

PA:A ratio	1.01±0. 12	0.91±0. 07	1.10±0.08	0.19	0.16–0.23	<0.001	0.99±0.11	0.91±0.06	1.09±0.08	0.18	0.16–0.20	<0.001

Prior exacerbation	--	--	--				120/190	56/101	64/89	17%	3 – 30	0.02
							(63%)	(55%)	(72%)

Inhaled antibiotic use	53/74	26/37	27/37	3%	−17 – 23	0.80	--	--	--
	(72%)	(70%)	(73%)

Inhaled DNAse use	55/74	27/37	28/37	3%	−17 – 22	0.79	--	--	--
	(74%)	(73%)	(76%)

Data are mean±SD or n(%). Abbreviations: FEV₁ = forced expiratory volume in 1 second, FVC = forced vital capacity, PA = pulmonary artery diameter, A = ascending aortic diameter, prior exacerbation = at least one acute pulmonary exacerbation within the year prior to index CT scan, 95%CI = 95% confidence interval.

Functional CFTR genotype defined by the presence of one mutation with residual function.

Sweat chloride values are only available for a subset of subjects [n=29 total; n=13 PA:A≤1; n=16 PA:A>1] in the validation cohort. Data on exacerbation prior to index CT scan is unavailable for the derivation cohort.