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Table 2 Clinical characteristics and treatment of CF carriers with recurrent airway infections

From: CFTR heterozygosity in severe asthma with recurrent airway infections: a retrospective review

#

Age (yrs)

Sex (M/F)

BMI (kg/m2)

CFTR mutation

Intron 8 alleles

FEV1 (L) (% pred)

Asthmaa (y/n)

Bronchiectasis (y/n)

Max sputum eos (%)

Sputum culture

Change in inf/yr with HTS

1

51

F

35.8

\(\Delta\)F508

7 T/9 T

N/Ab

N/A

N

6.5

SA/ST

− 1

2

75

M

25.0

\(\Delta\)F508

9 T/9 T

2.96 (94)

Y

Y

0

− 4

3

51

F

32.9

\(\Delta\)F508

7 T/9 T

2.4 (81)

N

N

34.5

 + 2

4

78

M

25.8

\(\Delta\)F508

7 T/9 T

1.82 (65)

N

N

5

HiB/NC/PA

− 4

5

47

M

33.8

\(\Delta\)F508

7 T/9 T

1.28 (50)

Y

N

5.8

HiB/NTM

 + 0

6

69

F

26.2

\(\Delta\)F508

5 T/9 T

0.32 (16)

N

Y

0.8

NTM/PA

N/Ac

7

70

F

24.9

\(\Delta\)F508

7 T/9 T

2.28 (88)

Y

Y

37.8

HiB/MC/NTM/PA/SP

− 3

8

75

F

26.0

c.1175 T>A

7 T/7 T

1.03 (61)

N

N

2

− 1

9

77

F

23.2

R117H

5 T/7 T

1.54 (75)

Y

Y

19

 + 0

10

54

F

40.0

621 + 1G > T

7 T/9 T

1.17 (48)

Y

N

14.3

N/Ac

11

62

M

31.6

c.2249C>T

7 T/9 T

1.60 (47)

N

Y

1.5

− 3

12

76

M

21.9

c.1540G>A

5 T/7 T

3.15 (90)

Y

Y

6.3

 + 0

13

90

F

23.1

S549N

7 T/9 T

1.52 (73)

N

Y

0

N/A

  1. HiB, Haemophilus influenzae type b; HTS, hypertonic saline; NC, Nocardia; NTM, non-tubercular mycobacterium; PA, Pseudomonas aeruginosa; SA, Staphylococcus aureus; SP, Streptococcus pneumoniae; ST, Stenotrophomonas
  2. aRepresents presence of current asthma at the time of evaluation based on bronchodilator reversibility (FEV1 > 12% and > 200 mL improvement with bronchodilator administration) or methacholine PC20 < 8 mg/mL
  3. bPatient had tracheostomy placed due to hereditary angioedema with recurrent laryngeal attacks, so spirometry was not obtained
  4. cPatient 6 was not treated with hypertonic saline due to low FEV1 and Patient 10 did not tolerate it due to bronchoconstriction