Table 3—

Phenotypic features according to the main ciliary defects as defined by transmission electron microscopy(group III, n = 245)

	Main ultrastructural defect			p-value
	ODA	IDA	CC	p-value
Patients n	159	40	46
Associated defects
IDA
Normal	81
Defect	56
Questionable	22
Radial spoke
Normal		9
Defect		31
Abnormal cilia %
Median	100^#	100^#	37	<0.0001
IQR	78–100	100–100	26.7–60.2
Range	21–100	30–100	20.5–85
Sex ratio F/M	0.91	0.66	0.65	ns
Children/adults ratio	2.74	3	2.83	ns
Situs inversus	65 (40.9)^¶	13 (32.5)	0 (0)^¶	<0.001
Patients with 100% abnormal cilia	110 (69.2)	32 (80)	0 (0)⁺	<0.001

Data are presented as n (%), unless otherwise indicated. ODA: outer dynein arm; IDA: inner dynein arm; CC: central complex; IQR: interquartile range; F/M: female/male; ns: nonsignificant. p-values are for global comparison between the four groups; if p<0.05, two-by-two comparisons were performed. ^#: indicates that the two groups were not different from each other but were significantly different from the other two groups; ^¶: indicates that the two groups were different from each other and were also significantly different from the other two groups; ⁺: indicates that only this group was significantly different from the other groups.