Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the accumulation of
alveolar surfactant and dysfunction of alveolar macrophages. PAP results in progressive …

Interstitial lung diseases (ILDs) are a set of heterogeneous lung diseases characterised by
inflammation and, in some cases, fibrosis. These lung conditions lead to dyspnoea, cough …

Monogenic lung diseases that are caused by mutations in surfactant genes of the pulmonary
epithelium are marked by perinatal lethal respiratory failure or chronic diffuse parenchymal …

Genetic disorders of surfactant production are rare but associated with significant morbidity
and mortality. Diagnosis can be made invasively through clinically available genetic testing …

Patients with idiopathic pulmonary fibrosis (IPF) often experience precipitous deteriorations,
termed “acute exacerbations”(AE), marked by diffuse alveolitis and altered gas exchange …

Pulmonary fibrosis refers to the development of diffuse parenchymal abnormalities in the
lung that cause dyspnea, cough, hypoxemia, and impair gas exchange, ultimately leading to …

Pulmonary fibrosis (PF) is a very rare condition in children, which may be observed in
specific forms of interstitial lung disease. None of the clinical, radiological, or histological …

Modeling Idiopathic Pulmonary Fibrosis Pathogenic findings in a mouse model of idiopathic
pulmonary fibrosis confirm the pivotal role played by dysfunction of the type 2 alveolar (AT2) …

Unprecedented successes regarding cancer immunotherapy have been achieved, in which
therapeutic agents are used to target immune cells rather than cancer cells. The most …

Background Mutations in the surfactant protein C gene (SFTPC) result in interstitial lung
disease (ILD). Our objective was to characterize clinical and genetic spectrum of ILD in …