The finding of smaller lamellar body size in our model is consistent with the abnormally small and dense lamellar body morphologies found in the lung tissues of patients with ABCA3 mutations (Figure 1) (1, 2, 9, 59) and suggests that these smaller lamellar bodies are a direct consequence of dysfunctional mutant ABCA3 rather than the epiphenomena of other perturbations, such as lung infections or ventilator induced lung injuries, which frequently occur in patients with chILD. The gene discussed is ABCA3; the disease is interstitial lung disease specific to childhood.