Table 1 Clinical characteristics and laboratory results of Chinese PCD patients categorized by age at diagnosis

Age at diagnosis (n = 237), mean (SD)

13.1 ± 11.3

8.2 ± 4.1(n = 189)

32.1 ± 10.1(n = 48)

< 0.0001

Gender, female (%)

116(47.5)

90 (47.6)

26 (47.3)

0.96

Age at symptom onset

 Infancy

120 (49.2)

112 (59.3)

8 (14.5)

< 0.0001

 Childhood

85 (34.8)

47 (24.9)

38 (69.1)

< 0.0001

 Adulthood

3(1.2)

NA

3 (5.5)

NA

 NA

36 (14.8)

30 (15.9)

6 (10.9)

0.36

Situs inversus totalis/heterotaxy

120 (49.2)

82 (43.4)

38 (69.1)

0.001

Family history

 Consanguineous patients

24 (9.8)

4 (2.1)

20 (36.4)

< 0.0001

 PCD family history

26 (10.7)

11 (5.8)

15 (27.3)

< 0.0001

Infertility history

 Female

7 (2.9)

NA

7 (12.7)

NA

 Male

16 (6.6)

NA

16 (29.1)

NA

Symptoms and Comorbidities

 Neonatal respiratory distress

60 (24.6)

60 (31.8)

0 (0)

< 0.0001

 Chronic wet cough

225 (92.2)

170 (89.9)

55 (100)

0.03

 Sinusitis

205 (84.0)

153 (81.0)

52 (94.5)

0.02

 Otitis media

67 (27.5)

59 (31.2)

8 (14.6)

0.02

 Hearing impairment

51 (20.9)

40 (21.2)

11 (20.0)

0.85

 Asthma

28 (11.5)

22 (11.6)

6 (10.9)

0.88

 Congenital heart disease

11 (4.5)

10 (5.3)

1 (1.8)

0.47

 Thoracic deformity

17 (7.0)

16 (8.5)

1 (1.8)

0.16

 Diffuse panbronchiolitis

2 (0.8)

0 (0)

2 (3.6)

0.05

 Post infectious bronchiolitis obliterans

6 (2.5)

6 (3.2)

0 (0)

0.34

 Gastroesophageal reflux

9 (3.7)

9 (4.8)

0 (0)

0.21

 Congenital deafness

3 (1.2)

2 (1.1)

1 (1.8)

0.54

Nasal NO test (n = 102)

 Below diagnostic cut-off

93 (91.2)

89 (93.3)

9 (75)

0.12

Respiratory culture (n = 133)

119

14

 Haemophilus influenzae

22 (16.5)

22 (18.6)

0 (0)

0.17

 Pseudomonas aeruginosa

26 (19.5)

18 (15.1)

8 (57.1)

0.001

 Streptococcus pneumoniae

36 (27.1)

36 (30.3)

0 (0)

0.04

 Streptococcus viridans

1 (0.8)

0 (0)

1 (7.1)

0.11

 Staphylococcus aureus

7 (5.3)

6 (5.0)

1 (7.7)

0.55

 Moraxella catarrhalis

4 (3.0)

4 (3.4)

0 (0)

1

 Legionella pneumophila

1 (0.8)

1 (0.8)

0 (0)

1

 Acinetobacter baumannii

1 (0.8)

1 (0.8)

0 (0)

1

 Monilia albican

2 (1.5)

2 (1.7)

0 (0)

1

 Aspergillus

1 (0.8)

0 (0)

1 (7.1)

0.11

 normal flora

35 (26.3)

32 (26.9)

3 (21.4)

0.91

Radiographic feature (n = 233)

178

55

 Bronchiectasis

164 (70.4)

110 (61.8)

54 (98.2)

< 0.0001

 Atelectasis

88 (37.8)

85 (47.8)

3 (5.5)

< 0.0001

 Diffuse nodules

29 (12.5)

22 (12.4)

7 (12.7)

0.94

 Pneumonia

19 (8.2)

15 (8.4)

4 (7.3)

1

 Nearly normal

7 (3.0)

7 (4.0)

0 (0)

0.29

Lung function

 FEV₁% of predicted, mean (SD)

70.6 (25.5) (n = 45)

83.7 (15.6) (n = 27)

51.0 (25.1) (n = 18)

< 0.0001

 FVC % of predicted, mean (SD)

80.9 (20.6) (n = 32)

82.3 (17.5) (n = 25)

75.9 (30.6) (n = 7)

0.61