The lung interfaces with the environment across a continuous epithelium composed of
various cell types along the proximal and distal airways. At the alveolar structure level, the …

Childhood interstitial lung diseases (chILDs) are rare and heterogeneous diseases with
significant morbidity and mortality. An accurate and quick aetiological diagnosis may …

Integrin α3 is a transmembrane integrin receptor subunit that mediates signals between the
cells and their microenvironment. We identified three patients with homozygous mutations in …

Rationale: Beginning in 2006, epidemics of a fatal lung injury of unknown cause in children
were observed in Korea every spring. A recent study demonstrated that this type of children's …

Background Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of alveolar
hemorrhage in children and its pathophysiology remains obscure. Classically, diagnosis is …

Objectives Childhood interstitial lung disease (chILD) is a group of rare chronic and complex
disorders of variable pathology. There has been no systematic review of published chILD …

Rationale: Children's Interstitial and Diffuse Lung Disease (chILD) is a heterogeneous group
of disorders that is challenging to categorize. In previous study, a classification scheme was …

We retrospectively studied the clinical presentation, treatment modalities and outcome in 16
patients with heterozygous NKX2-1 mutation associated with chronic lung disease. Twelve …

Abstract Background ABCA3 transporter (A TP-b inding cassette transporter of the A
subfamily) is localized to the limiting membrane of lamellar bodies, organelles for assembly …

Abstract Background Interstitial lung diseases (ILDs) in children represent a heterogeneous
group of rare respiratory disorders that affect the lung parenchyma. After the launch of the …