Pulmonary surfactant is critically important to prevent atelectasis by lowering the surface
tension of the alveolar lining liquid. While respiratory distress syndrome (RDS) is common in …

Surfactant protein C (SP-C) is an important player in enhancing the interfacial adsorption of
lung surfactant lipid films to the alveolar air-liquid interface. Doing so, surface tension drops …

Objective To compare outcomes of infants and children who underwent lung transplantation
for genetic disorders of surfactant metabolism (SFTPB, SFTPC, ABCA3, and NKX2-1) over 2 …

Genetic disorders of surfactant dysfunction result in significant morbidity and mortality,
among infants, children, and adults. Available medical interventions are limited, nonspecific …

Diffuse lung diseases in children, also called children's interstitial lung disease, are a
diverse group of rare disorders that cause disturbances of gas exchange in the lungs …

In this up-to-date study, we first aimed to highlight the genetic and non-genetic factors
associated with respiratory distress syndrome (RDS) while also focusing on the genomic …

Introduction Therapeutics exist to treat fibrotic lung disease in adults, but these have not
been investigated in children. Defining biomarkers for pediatric fibrotic lung disease in …

Summary Mutations of the Surfactant Protein C (SPC) gene (SFTPC) have been associated
with childhood interstitial lung disease (chILD) with variable age of onset, severity of lung …

Although recent decades have seen the identification, classification and discovery of the
genetic basis of many children's interstitial and rare lung disease (chILD) disorders, detailed …

Fibrotic interstitial pneumonias are a group of rare diseases characterised by distortion of
lung interstitium. Patients with mutations in surfactant-processing genes, such as surfactant …