The lung's unique extracellular matrix (ECM), while providing structural support for cells, is
critical in the regulation of developmental organogenesis, homeostasis and injury-repair …

Background: There is growing recognition and understanding of the entities that cause
interstitial lung disease (ILD) in infants. These entities are distinct from those that cause ILD …

Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic
diseases in neonates, infants, and older children and contributes to significant morbidity and …

We describe a human lung disease caused by autosomal recessive, complete deficiency of
the monocyte chemokine receptor CC motif chemokine receptor 2 (CCR2). Nine children …

Interstitial lung disease in children (chILD) is rare, and most centres will only see a few
cases/year. There are numerous possible underlying diagnoses, with specific and non …

Objective To investigate the characteristics and risk factors of a novel parenchymal lung
disease (LD), increasingly detected in systemic juvenile idiopathic arthritis (sJIA). Methods In …

In acquired pulmonary alveolar proteinosis, lipids and proteins accumulate within the alveoli
because alveolar macrophages cannot catabolize surfactants. Surprisingly, alveolar …

The alveolar region of the lung creates an extensive epithelial surface that mediates the
transfer of oxygen and carbon dioxide required for respiration after birth. Maintenance of …

Autoimmune pulmonary alveolar proteinosis (PAP) is a rare disease characterized by
myeloid cell dysfunction, abnormal pulmonary surfactant accumulation, and innate immune …

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar
accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte …