Mediators of Inflammation

Clinical Study

Inflammatory Lung Disease in Rett Syndrome

Table 2

Pulmonary gas exchange abnormality (GEA) in patients with typical Rett syndrome (): relationships between lung ventilation-perfusion () inequalities patterns and respiratory variables.


Variables	Pulmonary ventilation/perfusion () patterns in typical Rett syndrome					value (ANOVA)
Variables	No mismatch ()	“Low” ()	“High” ()	“Mixed” ()	“Simple” Mismatch ()	value (ANOVA)

(L/min)						<0.001
Respiratory rate (breaths/min)						0.162
Alveolar vent. (L/min)						<0.001
PaO₂ (mmHg)						0.025
Std. PaO₂ (mmHg)*						0.082
PaCO₂ (mmHg)						0.753
Blood pH						0.462
(A-a) O₂ (mmHg)						<0.001
Bohr’s DS %						<0.001
(%)						0.224
(mL/min)						<0.001
(mL/min)						0.015
Respiratory ratio						<0.001

Data are expressed as means ± SD. Bold characters indicate statistical significant differences; superscript letters indicate significant pairwise post hoc differences; : total ventilation; (A-a) O₂: Bohr’s DS: physiological dead space, as calculated by the Bohr equation, which, by quantifying the ratio of physiological dead space to the total tidal volume (/ = PaCO₂ − PaCO₂/PaCO₂), gives an indication of the extent of wasted ventilation; (A-a) O₂: O₂ alveolar-arterial gradient; : tidal volume; : pulmonary functional shunting; : oxygen uptake; : carbon dioxide production; *values were calculated according to the formula by Sorbini et al. accounting for hypocapnia: standard PaO₂ = 1.66 PaCO₂ + PaO₂ − 66.4 [52]; respiratory ratio: respiratory exchange ratio, that is, /. See text for further methodology details.