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Table 8 Comparison of various comorbidities in females with CDKL5 disorder (n = 143) and Rett syndrome (n = 321)

From: Prevalence and onset of comorbidities in the CDKL5 disorder differ from Rett syndrome

Comorbidity type

Rett syndromea

CDKL5 disorder

RR (95 % CI)

p value

n (%)

n (%)

GI health

    

 Any GI Problem

936/1181 (79.3)

82/120 (68.3)

0.85 (0.75–0.98)

0.02

 Constipation

871/1181 (73.8)

65/123 (52.9)

0.72 (0.60–0.87)

<0.001

 Reflux

214/793 (27.0)

44/110 (40.0)

1.41 (1.03–1.92)

0.03

Respiratory

    

 Hyperventilation

639/1175 (54.4)

15/109 (13.8)

0.26 (0.16–0.41)

<0.001

 Breath-holding

724/1176 (61.6)

26/109 (23.9)

0.40 (0.28–0.57)

<0.001

Sleep

    

 Night wakingb

422/591 (71.4)

96/110 (87.3)

1.18 (1.1–1.31)

0.03

 Night screaming

483/1139 (42.4)

60/118 (50.9)

1.1 (0.86–1.32)

0.57

 Night laughing

774/1139 (68.0)

70/122 (57.4)

0.76 (0.64–0.90)

0.001

  1. Each individual in the AussieRett database could have contributed from one to six observations according to their participation in the initial and follow-up questionnaires, which accounts for the large number of total observation (Rett syndrome)
  2. RR risk ratio; CI confidence interval
  3. aAverage prevalence for the 6 time points
  4. bData for females with night waking (Rett syndrome) was only available from 2009 and 2011 questionnaires