Table 4 Adjusted* associations of covariates with selected RSBQ scores in children with Rett syndrome

n

186

186

186

186

184

Adjusted coefficient (95% CI), P-value

Age, in years

 2–12

1.38 (0.29,2.46),0.01

0.60 (-0.32,1.51),0.20

0.57 (0.19,0.95),<0.01

0.23 (-0.36,0.83),0.44

3.65 (-0.61,7.90),0.09

 13–17

Ref

Ref

Ref

Ref

Ref

Variant

 C-terminal deletion

Ref

Ref

Ref

Ref

Ref

 Early truncation

1.41 (-1.03,3.85),0.26

0.80 (-1.25,2.86),0.44

0.23 (-0.63,1.09),0.60

-0.04 (-1.38,1.30),0.95

4.77 (-4.81,14.35),0.33

 Large deletion

0.87 (-1.56,3.29),0.48

-0.04 (-2.07,2.00),0.97

0.23 (-0.62,1.09),0.59

0.67 (-0.66,1.99),0.32

4.48 (-5.01,13.97),0.35

 p.Arg106Trp

2.94 (-0.48,6.37),0.09

0.27 (-2.61,3.15),0.85

0.66 (-0.55,1.86),0.28

-0.20 (-2.07,1.68),0.83

6.52 (-6.92,19.97),0.34

 p.Arg133Cys

3.19 (0.61,5.78),0.02

0.62 (-1.55,2.80),0.57

0.61 (-0.30,1.52),0.18

0.47 (-0.94,1.89),0.51

11.14 (1.02,21.27),0.03

 p.Thr158Met

1.69 (-0.69,4.07),0.16

0.73 (-1.28,2.73),0.47

0.53 (-0.31,1.36),0.22

0.36 (-0.94,1.66),0.59

7.10 (-2.23,16.43),0.13

 p.Arg168*

-0.01 (-2.44,2.43),0.99

-0.35 (-2.40,1.70),0.74

0.40 (-0.46,1.26),0.36

0.37 (-0.96,1.71),0.58

2.04 (-7.51,11.59),0.67

 p.Arg255*

0.85 (-1.66,3.36),0.51

1.24 (-0.87,3.36),0.25

0.14 (-0.74,1.03),0.75

0.37 (-1.00,1.75),0.59

5.24 (-4.60,15.08),0.29

 p.Arg270*

0.19 (-2.41,2.78),0.89

-0.12 (-2.31,2.06),0.91

0.44 (-0.48,1.35),0.35

-0.77 (-2.20,0.65),0.29

2.58 (-7.61,12.77),0.62

 p.Arg294*

3.40 (0.37,6.43),0.03

0.55 (-2.00,3.10),0.67

0.78 (-0.29,1.85),0.15

0.53 (-1.13,2.19),0.53

9.20 (-2.67,21.06),0.13

 Arg306Cys

1.66 (-1.17,4.49),0.25

-1.16 (-3.54,1.22),0.34

0.64 (-0.35,1.64),0.20

0.93 (-0.62,2.48),0.24

1.88 (-9.21,12.97),0.74

 Other

1.57 (-0.95,4.08),0.22

0.05 (-2.07,2.16),0.97

0.09 (-0.79,0.98),0.84

0.25 (-1.13,1.63),0.72

4.14 (-5.94,14.23),0.42

 Unknown

0.57 (-2.57,3.71),0.72

-0.32 (-2.96,2.32),0.81

-0.38 (-1.48,0.73),0.50

0.02 (-1.70,1.74),0.98

2.46 (-9.84,14.76),0.69

Walking ability

 Unable

-1.00 (-2.29,0.28),0.13

-0.70 (-1.78,0.39),0.21

-0.34 (-0.79,0.11),0.14

-0.10 (-0.80,0.61),0.79

-3.16 (-8.21,1.89),0.22

 Assisted

-0.67 (-2.10,0.76),0.36

0.13 (-1.08,1.33),0.84

0.06 (-0.44,0.57),0.81

0.77 (-0.01,1.55),0.05

0.71 (-4.95,6.37),0.81

 Independent

Ref

Ref

Ref

Ref

Ref

Hand function

 Unable

-0.77 (-2.18,0.65),0.29

-0.25 (-1.44,0.94),0.68

-0.15 (-0.65,0.34),0.54

0.16 (-0.61,0.94),0.68

1.30 (-4.26,6.86),0.65

 Large objects

-0.32 (-1.82,1.19),0.68

0.64 (-0.63,1.90),0.32

0.17 (-0.36,0.70),0.52

0.28 (-0.55,1.10),0.51

3.84 (-2.14,9.81),0.21

 Small objects

Ref

Ref

Ref

Ref

Ref

Seizure frequency

 Never or controlled

Ref

Ref

Ref

Ref

Ref

 Monthly or less

0.06 (-1.27,1.38),0.93

0.01 (-1.11,1.13),0.99

0.15 (-0.32,0.62),0.53

0.28 (-0.44,1.01),0.44

0.42 (-4.80,5.65),0.87

 Weekly

0.02 (-1.66,1.69),0.98

0.96 (-0.45,2.37),0.18

0.42 (-0.17,1.01),0.16

0.36 (-0.55,1.28),0.44

3.10 (-3.47,9.67),0.35

 Daily

-0.60 (-2.37,1.17),0.50

0.11 (-1.38,1.61),0.88

0.24 (-0.38,0.87),0.45

0.05 (-0.92,1.02),0.92

0.72 (-6.23,7.66),0.84

Constipation

 No

Ref

Ref

Ref

Ref

Ref

 Yes

-0.63 (-1.91,0.66),0.34

-1.38 (-2.46,-0.30),0.01

-0.48 (-0.93,-0.02),0.04

-0.69 (-1.39,0.02),0.06

-5.96 (-10.99,-0.92),0.02

Reflux

 No

Ref

Ref

Ref

Ref

Ref

 Yes

-0.18 (-1.36,1.00),0.77

-0.30 (-1.29,0.69),0.55

0.09 (-0.33,0.50),0.68

-0.20 (-0.85,0.45),0.54

-1.67 (-6.31,2.96),0.48

Insomnia

 Normal

Ref

Ref

Ref

Ref

Ref

 Abnormal

1.50 (0.23,2.77),0.02

0.18 (-0.89,1.25),0.74

0.93 (0.48,1.38),<0.01

0.60 (-0.09,1.30),0.09

4.83 (-0.15,9.81),0.06

Excessive daytime sleepiness

 Normal

Ref

Ref

Ref

Ref

Ref

 Abnormal

2.10 (0.78,3.41),<0.01

1.65 (0.54,2.75),<0.01

0.35 (-0.11,0.82),0.14

1.58 (0.86,2.30),<0.01

11.12 (5.97,16.27),<0.01

Data source

 AussieRett

Ref

Ref

Ref

Ref

Ref

 InterRett

0.06 (-1.30,1.43),0.93

0.55 (-0.59,1.70),0.34

-0.54 (-1.02,-0.06),0.03

0.03 (-0.72,0.78),0.94

-1.46 (-6.81,3.90),0.59