Abstract
Background: Homozygous ABCA3-deficiency is associated with a decreased surfactant function and results in irreversible respiratory failure or in chronic interstitial lung disease. Heterozygous individuals are susceptible for respiratory failure when an additional surfactant-protein-C-deficiency is present.
Aim: Determination of lung mechanics and inflammation in heterozygous ABCA3 mice (HZ) after exposure to 95%-oxygen or room-air as compared to wild type mice (WT).
Methods: HZ and WT were randomly assigned to 95%-oxygen (n=51) or room air (n=40). One set of mice was ventilated for 25 min and conventional mechanics were measured (Crs, Cstat, Rrs, total lung capacity (TLC), additionally lungimpedance was measured by forced oscillation technique (FOT è Rn, HL, GL, eta). Cell count and interleukins in BAL and wet to dry ratio were determined in ventilated and non-ventilated animals.
Results: There was no difference between ventilated HZ and WT mice with regard to respiratory mechanics, impedance measurements and BAL parameters after exposure to room air. After exposure to 95% oxygen TLC was significantly higher in HZ whereas Rn was lower as compared to WT (TLC: 86.6 vs 60.2, p=0.011; Rn: 0.29 vs 0.34, p=0.034). Leucocytes were higher in non-ventilated, room-air housed HZ as compared to WT (310 vs 170/μl, p=0,021). Oxygen exposure followed by ventilation resulted in a significant increase of interleukines HZ (II-6: 3.04 vs 2.55pg/ml, p=0.003; MIP-2: 23.99 vs 11.19, p=0.007).
Summary: Our data suggest that lung mechanics and inflammation in HZ are not affected a priori. However, ventilation or oxygen exposure resulted in a phenotypical difference between genotypes.
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Herber-Jonat, S., Huppmann, M., Mittal, R. et al. 36 Characterisation of Lung Mechanics and Inflammation in Heterozygous Abca3 Mice After Exposure to 95% Oxygen or Room Air. Pediatr Res 68 (Suppl 1), 21–22 (2010). https://doi.org/10.1203/00006450-201011001-00036
Issue date:
DOI: https://doi.org/10.1203/00006450-201011001-00036
