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. 2016 Apr 14;11:39. doi: 10.1186/s13023-016-0418-y

Table 8.

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

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

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)

RR risk ratio; CI confidence interval

aAverage prevalence for the 6 time points

bData for females with night waking (Rett syndrome) was only available from 2009 and 2011 questionnaires