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Table 2 Results of the diagnostic testing

From: Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia

 

All (%)

Children (%)

Adults (%)

nNO (nL/min)

45(100)

13(100)

32(100)

Low(<  77)

34(75.6)

11(84.6)

23(31.9)

Normal(≥ 77)

11(24.4)

2(15.4)

9(28.1)

Ciliary motility

50(100.0)

17(100.0)

33(100.0)

Complete immotility

11(22.0)

1(5.9)

10(30.3)

Reduced CBF (<  5 Hz)

22(44.0)

9(52.9)

13(39.4)

Reduced CBF (5–10 Hz)

4(8.0)

1(5.9)

3(9.1)

Within normal range (>  10 Hz)

2(4.0)

1(5.9)

1(3.0)

Absence of cilia

11(22.0)

5(29.4)

6(18.2)

Ciliary beat pattern

50(100.0)

17(100.0)

33(100.0)

Complete immotility

11(22.0)

1(5.9)

10(30.3)

Minimal residual movement

12(24)

5(29.4)

7(21.2)

Extremely stiff

8(16)

2(11.8)

6(18.2)

Reduced proximal bending

6(12)

3(17.6)

3(9.1)

Circular beating cilia

2(4)

1(5.9)

1(3)

Absence of cilia

11(22.0)

5(29.4)

6(18.2)

TEM ultrastructure

54(100.0)

19(100.0)

35(100.0)

Only ODA absence

12(22.2)

2(10.5)

10(28.6)

ODA + IDA absence

10(18.5)

1(5.3)

9(25.7)

ODA + IDA absence + CC defect

2(3.7)

1(5.3)

1(2.9)

IDA absence + MTD

12(22.2)

9(47.4)

3(8.6)

CC defect

6(11.1)

2(10.5)

4(11.4)

Only IDA absence

3(5.6)

0(0)

3(8.6)

Normal

9(16.7)

4(21)

5(14.2)

Genetic analysis

42(100.0)

15(100.0)

27(100.0)

No mutations

16(38.1)

6(40.0)

10(37.0)

DNAH5 Mutations

15(35.7)

5(33.3)

10(37.0)

DNAH11 Mutations

6(14.3)

2(13.3)

4(14.9)

DNAI1 Mutations

3(7.1)

1(6.7)

2(7.4)

Other mutations

2(4.8)

1(6.7)

1(3.7)

  1. ODA = Outer Dynein Arm; IDA = Inner Dynein Arm; CC = Central Complex;MTD= Microtubular disorganization
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BMC Pediatrics

ISSN: 1471-2431

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