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Table 1 Comparison of ciliary beat pattern (CBP) and ciliary beat frequency (CBF) in eight patients with primary ciliary dyskinesia (PCD) and seven healthy non-smoking controls measured by conventional ciliary function analysis (cCFA) performed immediately after brush biopsy and after patient-specific 3D-explant spheroid (3D-E) formation

From: Patient-specific three-dimensional explant spheroids derived from human nasal airway epithelium: a simple methodological approach for ex vivo studies of primary ciliary dyskinesia

Healthy

Mean CBF, cCFA (Hz)

Mean CBF, 3D-E (Hz)

CBP, cCFA

CBP, 3D-E

No. of days in culture

EM defects

IF defects

PCD mutation

nNO (ppb)

1

10.7

10.0

Synchronous

Synchronous

4

2

10.0

9.8

Synchronous

Synchronous

5

3

10.0

11.5

Synchronous

Synchronous

4

4

8.8

9.0

Synchronous

Synchronous

5

5

8.3

8.0

Synchronous

Synchronous

1

6

7.8

9.0

Synchronous

Synchronous

3

7

5.3

7.0

Synchronous

Synchronous

3

PCD

 1

0

0

Immotile

Immotile

5

ODA

DNAH5 (polyclonal) abnormal

Awaiting results

36

 2

0

0

Immotile/stiff flickery

Immotile/stiff flickery

2

Radial spoke head defect +IDA defect

GAS8/11 abnormal and DNALI1 (monoclonal) inconclusive

CCDC40

52

 3

0

0

Immotile

Immotile

1

ODA

DNAH5 (polyclonal) inconclusive and DNALI1 (LC28,polyclonal) abnormal

Awaiting results

30

 4

3.0

4.0

Asynchronous

Asynchronous

2

ODA

DNAH5 (polyclonal) abnormal

Awaiting results

283

 5

3.6

5.0

Asynchronous

Asynchronous

2

ODA

DNAH5 (polyclonal) abnormal

Awaiting results

102

 6

5.3

5.0

Asynchronous

Asynchronous

5

Normal

None found

Unknown

101

 7

3.6

5.0

Asynchronous

Asynchronous

2

Inconclusive

None found

Unknown

 8

3.0

2.0

Stiff and asynchronous

Stiff and asynchronous

2

Sample poor, not repeated

Not done

Unknown

36