Table 1 Baseline characteristics of patients in the UK CF Registry on December 31st 2012. (^§Continuous variables: median (inter-quartile range)).

Gender (% male)

2,027 (55.0)

192 (50.2)

0.075

Pancreatic

Age (years)^§

27.6 (12)

29.2 (14)

<0.001

 Cirrhosis

86 (2.3)

24 (6.3)

<0.001

Height (cm)^§

168.0 (14)

166.0 (15)

<0.001

 Liver Disease

578 (15.7)

81 (21.2)

0.007

Weight (kg)^§

63.1 (17)

54.8 (15)

<0.001

 Pancreatitis

57 (1.5)

3 (0.8)

0.368

BMI (kg/m²)^§

22.3 (4)

20.1 (4)

<0.001

 Liver Enzymes

521 (14.1)

98 (25.7)

<0.001

CFTR genotype

 Gall Bladder

20 (0.5)

3 (0.8)

0.472

 Homozygous

1,784 (48.4)

208 (54.4)

<0.001

 GI Bleed (variceal)

3 (0.1)

3 (0.8)

0.013

 Heterozygous

1,240 (33.7)

92 (24.0)

<0.001

Gastrointestinal

 ΔF508

3,189 (86.6)

325 (85.0)

0.388

 GERD

747 (20.3)

100 (26.2)

0.008

 G551D

224 (6.0)

15 (3.9)

0.108

 GI Bleed (no variceal)

4 (0.1)

1 (0.3)

0.390

 Class I

169 (4.6)

23 (6.0)

0.205

 Intestinal Obstruction

303 (8.2)

33 (8.6)

0.770

 Class II

3,207 (87.1)

326 (85.3)

0.338

Musculoskeletal

 Class III

3,281 (89.1)

330 (86.3)

0.123

 Arthropathy

338 (9.2)

52 (13.6)

0.008

 Class IV

184 (5.0)

4 (1.0)

<0.001

 Bone Fracture

39 (1.1)

6 (1.6)

0.310

 Class V

130 (3.5)

8 (2.0)

0.179

 Osteopenia

710 (19.3)

126 (33.0)

<0.001

 Class VI

3,189 (86.6)

325 (85.0)

0.388

Other

Spirometry ^§

 Cancer

8 (0.2)

5 (1.3)

0.005

 FEV₁ (L)

2.34 (1.4)

0.99 (0.6)

<0.001

 Diabetes

906 (24.6)

199 (52.1)

<0.001

 FEV₁%

67.8 (35)

29.6 (19)

<0.001

 CFRD

1,096 (29.8)

223 (58.4)

<0.001

 Best FEV₁ (L)

2.57 (1.4)

1.2 (0.7)

<0.001

 Pulmonary Abscess

2 (0.1)

0 (0.0)

1.000

 Best FEV₁%

75.2 (31)

35.2 (18)

<0.001

 Chronic Pseudomonas

2,005 (54.5)

281 (73.6)

<0.001

 FEV₁% (2011)

70.2 (34)

36.2 (20)

<0.001

 Osteoporosis

293 (8.0)

91 (23.8)

<0.001

 FEV₁% (2010)

70.7 (33)

37.5 (23)

<0.001

 AICU

5 (0.1)

1 (0.3)

0.447

 FEV₁% (2009)

72.2 (32)

41.2 (27)

<0.001

 Kidney Stones

45 (1.2)

17 (4.5)

<0.001

 FEV₁% (2008)

73.7 (31)

45.2 (27)

<0.001

 Cough Fracture

1 (0.0)

3 (0.8)

0.003

Lung Infections

 Hypertension

121 (3.3)

23 (6.0)

0.012

 B. Cepacia

176 (4.8)

35 (9.2)

0.001

 Atypical Mycobacteria

127 (3.4)

17 (4.5)

0.308

 P. Aeruginosa

2,190 (59.5)

295 (77.2)

<0.001

 Hearing Loss

82 (2.2)

26 (6.8)

<0.001

 MRSA

154 (4.2)

17 (4.5)

0.789

 Depression

257 (7.0)

59 (15.4)

<0.001

 Aspergillus

478 (13.0)

70 (18.3)

0.006

Inhaled Antibiotics

2,194 (59.6)

280 (73.3)

<0.001

 NTM

186 (5.1)

20 (5.2)

0.902

Muco-active Therapies

 H. Influenza

191 (5.2)

10 (2.6)

0.025

 DNase

2,057 (55.9)

297 (77.7)

<0.001

 E. Coli

17 (0.5)

2 (0.5)

0.698

 Hypertonic Saline

859 (23.3)

109 (28.5)

0.027

 K. Pneumoniae

10 (0.3)

3 (0.8)

0.116

Promixin

765 (20.8)

71 (18.6)

0.352

 Gram-negative

14 (0.4)

4 (1.0)

0.082

Tobramycin

110 (3.0)

28 (7.3)

<0.001

 ALCA

97 (2.6)

25 (6.5)

<0.001

iBuprofen

8 (0.2)

2 (0.5)

0.241

 Staph. Aureus

1,175 (31.9)

64 (16.8)

<0.001

Oral Corticosteroids

347 (9.4)

122 (31.9)

<0.001

 Xanthomonas

165 (4.5)

23 (6.0)

0.199

IV Antibiotics

1493 (40.5)

292 (76.4)

<0.001

 B. Multivorans

86 (2.3)

16 (4.2)

0.037

IV Antibiotic Courses ^§

 B. Cenocepacia

51 (1.4)

13 (3.4)

0.007

 Days at Home

0 (14)

14 (49)

<0.001

 Pandoravirus

8 (0.2)

2 (0.5)

0.241

 Days at Hospital

0 (13)

27.5 (56)

<0.001

Comorbidities

Non-IV Hospitalization

312 (8.5)

62 (16.2)

<0.001

Respiratory

Non-invasive Ventilation

161 (4.4)

82 (21.5)

<0.001

 ABPA

432 (11.7)

71 (18.6)

<0.001

Oxygen Therapy

279 (7.6)

205 (53.7)

<0.001

 Nasal Polyps

123 (3.3)

4 (1.0)

0.012

 Continuous

13 (0.4)

75 (19.6)

<0.001

 Asthma

578 (15.7)

58 (15.2)

0.825

 Nocturnal

42 (1.1)

48 (12.6)

<0.001

 Sinus Disease

486 (13.2)

41 (10.7)

0.200

 Exacerbation

100 (2.7)

46 (12.0)

<0.001

 Hemoptysis

48 (1.3)

11 (2.9)

0.022

 Pro re nata

37 (1.0)

29 (7.6)

<0.001