Table 1 Clinical manifestations of Chinese CF patients

Age at onset(y)(n = 63)

Median age 1y

Pulmonary function

0.1–6.0y*

FEV₁(n = 38)

67.8% ± 20.9%

Age at diagnosis(y)(n = 69)

Median age 10y

FVC(n = 20)

80.7% ± 13.8%

4.6–13.3y^*

FEV₁/FVC (n = 19)

71.2% ± 12.3%

Sex(F/M) (n = 69)

37/32

Sweat chloride test (n = 52)

Median value 118.0 mmol/L

Family history(n = 71)

17/71

104.0–135.0 mol/L^*

(n = 59)

normal

8/25

46/59

-1 SD - 0

2/25

13/59

-2 SD - (−1 SD)

6/25

4/59

−3 SD - (− 2 SD)

3/25

KP

3/59

<-3 SD

6/25

Other

3/59

Respiratory system (n = 71)

MRSA

2/59

bronchiectasis

67/71

E.Coli

1/59

ABPA

15/71

MRSE

1/59

sinusitis

33/71

MSSE

1/59

Digestive system (n = 71)

n = 71

SP

1/59

icterus

0

BC

1/59

diarrhea

15/71

HI

1/59

hepatomegaly

1/71

AC

1/59

hypersplenotrophy

1/71

MC

1/59

ileus

3/71

NTM

1/59

hepatolienomegaly

2/71

Misdiagnosis (n = 29)

positive fecal Sudan III

10/71

bronchiectasis

12/29

hepatocirrhosis

2/71

pneumonia

9/29

Reproductive system (n = 38)

Batter syndrome

5/29

ABPA

5/29

CBAVD (adult male, n = 1)

1

other

3/29

TB

4/29

upper respiratory tract infection

2/29