Large multicenter clinical trials have led to two recently approved drugs for patients with
idiopathic pulmonary fibrosis (IPF); yet, both of these therapies only slow disease …

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 …

Idiopathic pulmonary fibrosis (IPF) is a lethal interstitial lung disease characterized by airway
remodeling, inflammation, alveolar destruction, and fibrosis. We utilized single-cell RNA …

Evidence of endoplasmic reticulum (ER) stress has been found in lungs of patients with
familial and sporadic idiopathic pulmonary fibrosis. We tested whether ER stress causes or …

Familial pulmonary fibrosis is a heterogeneous group of interstitial lung diseases of
unknown cause that is associated with multiple pathologic subsets. Mutations in the …

Rationale: Acquired pulmonary alveolar proteinosis (PAP) is a syndrome characterized by
pulmonary surfactant accumulation occurring in association with granulocyte/macrophage …

Rationale: Considerable confusion exists regarding nomenclature, classification, and
management of pediatric diffuse lung diseases due to the relative rarity and differences in …

Pulmonary surfactant is essential for life and is composed of a complex lipoprotein-like
mixture that lines the inner surface of the lung to prevent alveolar collapse at the end of …

The matrix metalloproteinases (MMPs), a family of 25 secreted and cell surface-bound
neutral proteinases, process a large array of extracellular and cell surface proteins under …

All tissues and organs can be classified according to their ability to repair and regenerate
during adult homeostasis and after injury. Some exhibit a high rate of constant cell turnover …