Table 3 Adjusted* associations of covariates with selected RSBQ scores among individuals with Rett syndrome

n

365

365

365

365

362

Adjusted coefficient (95% CI), P-value

Age, in years

 2–12

0.65 (-0.47,1.78),0.25

0.80 (-0.10,1.69),0.08

0.43 (0.02,0.83),0.04

0.65 (0.05,1.25),0.03

4.21 (0.26,8.16),0.04

 13–17

-0.49 (-1.60,0.62),0.38

0.24 (-0.64,1.13),0.59

-0.07 (-0.47,0.33),0.72

0.61 (0.01,1.20),0.04

1.34 (-2.58,5.26),0.50

 18–24

-0.15 (-1.24,0.94),0.79

0.20 (-0.67,1.07),0.66

0.30 (-0.09,0.70),0.13

0.34 (-0.24,0.92),0.25

1.46 (-2.38,5.30),0.45

 25+

Ref

Ref

Ref

Ref

Ref

Variant

 C-terminal deletion

Ref

Ref

Ref

Ref

Ref

 Early truncation

0.57 (-1.28,2.41),0.55

0.40 (-1.08,1.87),0.60

0.33 (-0.34,1.00),0.33

-0.59 (-1.57,0.40),0.24

1.95 (-4.54,8.45),0.55

 Large deletion

0.86 (-0.91,2.63),0.34

-0.04 (-1.46,1.37),0.95

0.30 (-0.34,0.94),0.36

-0.25 (-1.20,0.69),0.60

3.08 (-3.15,9.31),0.33

 p.Arg106Trp

0.21 (-1.89,2.30),0.84

-0.70 (-2.38,0.98),0.41

0.09 (-0.67,0.85),0.82

-0.61 (-1.72,0.51),0.29

-0.06 (-7.44,7.32),0.99

 p.Arg133Cys

1.20 (-0.57,2.98),0.18

-0.21 (-1.63,1.21),0.77

0.22 (-0.42,0.87),0.49

0.26 (-0.68,1.21),0.59

3.71 (-2.53,9.95),0.24

 p.Thr158Met

1.06 (-0.62,2.73),0.21

0.05 (-1.29,1.39),0.95

0.31 (-0.30,0.91),0.32

0.21 (-0.68,1.10),0.64

3.36 (-2.54,9.25),0.26

 p.Arg168*

0.39 (-1.25,2.03),0.64

-0.31 (-1.62,1.00),0.64

0.63 (0.03,1.22),0.04

0.31 (-0.56,1.19),0.48

3.26 (-2.52,9.03),0.27

 p.Arg255*

1.19 (-0.51,2.89),0.17

0.60 (-0.76,1.96),0.39

0.68 (0.06,1.29),0.03

0.23 (-0.68,1.14),0.62

5.50 (-0.48,11.49),0.07

 p.Arg270*

0.26 (-1.71,2.24),0.79

-0.63 (-2.21,0.95),0.43

0.32 (-0.40,1.03),0.38

-0.65 (-1.71,0.40),0.22

0.38 (-6.57,7.33),0.91

 p.Arg294*

2.30 (0.37,4.22),0.02

-0.34 (-1.88,1.20),0.66

0.57 (-0.13,1.27),0.11

0.49 (-0.54,1.52),0.35

4.33 (-2.44,11.11),0.21

 Arg306Cys

1.57 (-0.34,3.48),0.11

-0.59 (-2.12,0.94),0.45

0.69 (-0.00,1.38),0.05

0.69 (-0.33,1.70),0.19

2.77 (-4.04,9.58),0.42

 Other

1.69 (0.01,3.37),0.05

0.51 (-0.84,1.85),0.46

0.30 (-0.31,0.91),0.34

-0.01 (-0.91,0.89),0.99

4.69 (-1.29,10.67),0.12

 Unknown

0.04 (-2.32,2.40),0.97

-0.00 (-1.80,1.79),1.00

-0.40 (-1.26,0.46),0.36

-0.10 (-1.36,1.16),0.88

-0.03 (-8.20,8.14),0.99

Walking ability

 Unable

-0.95 (-1.88,-0.03),0.04

-0.32 (-1.06,0.42),0.40

-0.11 (-0.45,0.23),0.52

-0.01 (-0.51,0.48),0.96

-3.44 (-6.71,-0.17),0.04

 Assisted

0.17 (-0.78,1.13),0.72

0.56 (-0.20,1.33),0.15

0.31 (-0.04,0.66),0.08

0.74 (0.23,1.25),<0.01

3.69 (0.31,7.08),0.03

 Independent

Ref

Ref

Ref

Ref

Ref

Hand function

 Unable

-0.58 (-1.54,0.39),0.24

0.53 (-0.24,1.30),0.18

-0.05 (-0.40,0.30),0.77

0.07 (-0.45,0.58),0.80

2.79 (-0.61,6.20),0.11

 Large objects

-0.50 (-1.62,0.63),0.39

0.78 (-0.12,1.69),0.09

-0.18 (-0.59,0.23),0.40

0.30 (-0.30,0.90),0.33

3.78 (-0.21,7.78),0.06

 Small objects

Ref

Ref

Ref

Ref

Ref

Seizure frequency

 Never or controlled

Ref

Ref

Ref

Ref

Ref

 Monthly or less

-0.66 (-1.58,0.26),0.16

0.68 (-0.05,1.42),0.07

-0.19 (-0.53,0.14),0.26

0.00 (-0.49,0.49),0.99

-0.24 (-3.48,3.01),0.89

 Weekly

-0.35 (-1.55,0.85),0.57

1.43 (0.47,2.39),<0.01

0.26 (-0.17,0.70),0.23

-0.00 (-0.64,0.64),1.00

2.15 (-2.09,6.39),0.32

 Daily

-1.05 (-2.33,0.23),0.11

0.42 (-0.61,1.44),0.42

0.07 (-0.39,0.54),0.76

-0.42 (-1.10,0.26),0.23

-2.37 (-6.92,2.19),0.31

Constipation

 No

Ref

Ref

Ref

Ref

Ref

 Yes

-0.37 (-1.29,0.56),0.44

-0.44 (-1.18,0.30),0.24

-0.16 (-0.50,0.17),0.34

-0.26 (-0.76,0.23),0.29

-2.17 (-5.42,1.07),0.19

Reflux

 No

Ref

Ref

Ref

Ref

Ref

 Yes

0.09 (-0.74,0.93),0.83

-0.86 (-1.53,-0.19),0.01

-0.01 (-0.32,0.29),0.93

0.04 (-0.41,0.49),0.86

-1.32 (-4.29,1.64),0.38

Insomnia

 Normal

Ref

Ref

Ref

Ref

Ref

 Abnormal

1.82 (0.92,2.71),<0.01

0.13 (-0.59,0.85),0.72

1.03 (0.71,1.36),<0.01

0.59 (0.11,1.07),0.02

5.75 (2.58,8.92),<0.01

Excessive daytime sleepiness

 Normal

Ref

Ref

Ref

Ref

Ref

 Abnormal

1.82 (0.84,2.79),<0.01

0.74 (-0.05,1.52),0.07

0.27 (-0.09,0.62),0.14

1.17 (0.65,1.69),<0.01

7.85 (4.39,11.31),<0.01

Data source

 AussieRett

Ref

Ref

Ref

Ref

Ref

 InterRett

-0.78 (-1.73,0.18),0.11

0.12 (-0.65,0.89),0.76

-0.48 (-0.83,-0.13),0.01

-0.47 (-0.98,0.04),0.07

-3.89 (-7.28,-0.51),0.02