▪ Abstract The hydrophobic surfactant proteins, SP-B and SP-C, have important roles in
surfactant function. The importance of these proteins in normal lung function is highlighted …

A set of lung diseases share the tendency for the development of progressive fibrosis
ultimately leading to respiratory failure. This review examines the common pathogenetic …

Background Pulmonary surfactant forms a lipid-rich monolayer that coats the airways of the
lung and is essential for proper inflation and function of the lung. Surfactant is produced by …

Background: While idiopathic pulmonary fibrosis (IPF) is one of the most common forms of
interstitial lung disease, the aetiology of IPF is poorly understood. Familial cases of …

Chronic interstitial lung diseases in children represent a heterogeneous group of disorders
of both known and unknown causes that share common histological features. Despite many …

Thyroid transcription factor-1 (TTF-1/Nkx-2.1) is required for formation of the lung and
differentiation of peripheral respiratory epithelial cells. TTF-1 activates transcription of target …

The spectrum of pediatric interstitial lung disease (PILD) includes a diverse group of rare
disorders characterized by diffuse infiltrates and disordered gas exchange. Children with …

Adaptation to air breathing at birth is dependent on formation and function of the lung. Lung
morphogenesis is a complex process dependent on precise temporal–spatial control of cell …

Mutations in the surfactant protein C gene (SFTPC) were recently reported in patients with
interstitial lung disease. In a 13‐month-old infant with severe respiratory insufficiency, a lung …

Pulmonary surfactant protein C (SP-C) has been originally identified as a highly
hydrophobic protein of $3.5-kDa purified from broncho-alveolar lavage (BAL) fluid from a …