|
Number (n)
|
17
|
16
|
13
|
|
Age years (mean range)
|
38 (25-54)
|
26.9 (13-59)
|
14.9 (6-43)
|
|
Male (%)
|
38%
|
63%
|
53%
|
|
BMI (kg/m2) (mean range)
| |
19.07 (17.1-24.4)
|
19.9 (14.8-37.2)
|
|
Respiratory function: FEV1%
| |
44.92 (13-93)
|
77.15 (48-99)
|
|
FVC%
| |
59.51 (1.14-102)
|
91.1 (55-120)
|
|
Genetic analysis
| |
Sweat test (3)
|
Sweat test (3)
|
| | |
ΔF508/ΔF508 (9)
|
ΔF508/ΔF508 (5)
|
| | |
ΔF508/G542X (3)
|
ΔF508/G542X (1)
|
| | |
ΔF508/c.1339delA (1)
|
c.F508/c.1766+1g (1)
|
| | | |
ΔF508/3849+10kbct (1)
|
| | | |
c.1624g > t(p.g542x)/c.1
|
| | | |
624g > t(p.g542x) (1)
|
| | | |
ΔF508/P67L (1)
|
|
Diagnoses
^
| | | |
|
Pancreatic insufficiency
| |
15
|
12
|
|
Chronic rhinosinuitis
| |
9
|
0
|
|
Osteopenia
| |
8
|
0
|
|
Gastro-oesophageal disease
| |
7
|
0
|
|
CF related diabetes
| |
4
|
3
|
|
Vitamin A, D & E deficiency
| |
5
|
1
|
|
Asthma
| |
5
|
0
|
|
Allergic bronchopulmonary
| |
0
|
3
|
|
aspergillosis
| | | |
|
Distal intestinal obstruction
| |
1
|
2
|
|
syndrome
| | | |
|
Previous tuberculosis
| |
1
|
0
|
|
Pancreatitis
| |
1
|
0
|
|
Chronic nasal polyps
| |
0
|
1
|
|
Haemoptysis
| |
0
|
0
|
|
Chronic eczema & ichthyosis
| |
1
|
1
|
