How to evaluate and treat children's interstitial lung disease

详细信息    查看全文

• 作者：Robin Deterding
• 关键词：pulmonary surfactant ; respiratory distress ; gene mutations
• 刊名：Paediatric Respiratory Reviews
• 出版年：2006
• 出版时间：2006
• 年：2006
• 卷：7
• 期：Supplement 1
• 页码：S245-S247
• 全文大小：81 K

文摘

Pulmonary surfactant reduces surface tension at the air-liquid interface in the alveolus, thereby maintaining lung volumes during the respiratory cycle. In premature newborn infants, the lack of surfactant causes atelectasis and respiratory failure, characteristic of respiratory of distress syndrome. Surfactant is comprised of lipids and associated proteins that are required for surfactant function. Surfactant proteins B and C and a lamellar body associated transport protein, ABCA3 play critical roles in surfactant synthesis and function. Mutations in the genes encoding these proteins cause lethal respiratory distress in newborn infants. This review discusses the clinical and pathological findings associated with these inherited disorders of alveolar homeostasis.