Table 4

Ciliary ultrastructural analysis of PCD patients with identified CCDC39 and CCDC40 mutations (CCDC39 and CCDC40 groups)

CCDC39 groupCCDC40 groupp Value
Total number of patients with available quantitative analysis257
Ciliary length (μm) median (25th; 75th IQR)5.59 (5.47; 5.86)5.45 (5.38; 5.75)NS
Ciliary orientation, n (%)NS
 Normal3 (12)2 (28.6)
 Abnormal22 (88)4 (57.1)
 Non-evaluable0 (0)1 (14.3)
Compound cilia, n (%)NS
 Absent19 (76)4 (57.1)
 Present6 (24)3 (42.9)
  • NS, non-significant; PCD, primary ciliary dyskinesia.