Pulmonary function test and pulmonary interstitial disease / 中华实用儿科临床杂志

Interstitial lung diseases (ILD) are a heterogeneous group of disorders, which are functionally cha-racterized by a restrictive ventilatory defect and exercise induced hypoxemia.Most lung function abnormalities in ILD share common features but are not specific.Pulmonary function is important for the diagnosis of patients with suspected ILD.For the diagnosis of ILD cases, pulmonary function test can assess the severity of the diseases and monitor progre-ssion of diseases.

Correlation between ABCA3 and Pediatric Pulmonary Disease / 临床儿科杂志

Pulmonary surfactant is a lipid-protein complex that lines and stabilizes the respiratory interface in the alveoli, allowing for gas exchange during the breathing cycle. ATP-binding Cassette transporters A3, is a lipid transporter in the limiting membrane of lamellar bodies in alveolar type Ⅱ cells that plays a critical role in the regulation of pulmonary surfactant homeostasis. Mutations in the ABCA3 gene cause respiratory distress syndrome in new-born and childhood interstitial lung disease. An updated view on expression of ABCA3 gene and ABCA3 mutation associated with neonatal respiratory distress syndrome,or childhood interstitial lung disease was reviewed.