Inflammatory Lung Disease in Rett Syndrome

Table 1

Relevant demographic and clinical characteristics of female subjects with Rett syndrome.

Variables
Patients (𝑁)	228
Age (years)	12.9±7.9^§
Body weight (RTT 𝑧-score for age)^1	0.025±1.12
Body height (RTT 𝑧-score for age)^1	−0.05±1.13
Head circumference (RTT 𝑧-score)^1	−0.22±1.11
Body mass index (BMI) (RTT 𝑧-score for age)^1	−0.36±1.45
Clinical severity score (CSS)^2	17.4±7.3
Tachypnea^a	59 (25.9 %)
Respiratory dysfunction on a clinical basis^b
+	42 (18.4 %)
++	127 (55.7 %)
+++	59 (25.9 %)
Additional clinical features
Air-S^c	64 (28.1 %)
Severe GERD^d	27 (11.8 %)

1Calculated 𝑧-scores for age are referred to a validated Rett syndrome-specific growth charts [42]. ²Clinical severity score was defined according to Neul et al., 2008 [7]. ^aTachypnea was defined as a respiratory rate >1.8 times (i.e., above the upper quartile) of the expected respiratory rate for age and gender; ^brespiratory dysfunction was categorized based on the corresponding Percy’s clinical severity scale item [41]; ^§mean  ± SD; ^cAir-S: abnormal air swallowing; ^dGERD: gastroesophageal reflux disease.