Genetic disorders of surfactant deficiency and neonatal lung disease
Pulmonary surfactant is a heterogeneous combination of lipids and proteins, which prevents
alveolar collapse at the end of expiration cycle by decreasing the alveolar surface tension at …
alveolar collapse at the end of expiration cycle by decreasing the alveolar surface tension at …
The clinical and genetic spectrum of the interstitial lung diseases in infancy associated with surfactant protein disorders: A retrospective study
Background: Childhood interstitial lung diseases (chILD) include a large spectrum of
diseases. Besides the genetically defined surfactant dysfunction disorders, most present …
diseases. Besides the genetically defined surfactant dysfunction disorders, most present …
Genetic disorders of surfactant dysfunction
SE Wert, JA Whitsett, LM Nogee - Pediatric and …, 2009 - journals.sagepub.com
Mutations in the genes encoding the surfactant proteins B and C (SP-B and SP-C) and the
phospholipid transporter, ABCA3, are associated with respiratory distress and interstitial …
phospholipid transporter, ABCA3, are associated with respiratory distress and interstitial …
Inherited surfactant protein-B deficiency and surfactant protein-C associated disease: clinical features and evaluation
A Hamvas - Seminars in perinatology, 2006 - Elsevier
The pulmonary surfactant is a mixture of phospholipids and proteins synthesized, packaged,
and secreted by alveolar type II cells that lowers surface tension and prevents atelectasis at …
and secreted by alveolar type II cells that lowers surface tension and prevents atelectasis at …
Genetic disorders of surfactant homeostasis
JA Whitsett - Paediatric Respiratory Reviews, 2006 - Elsevier
Pulmonary surfactant reduces surface tension at the air-liquid interface in the alveolus,
thereby maintaining lung volumes during the respiratory cycle. In premature newborn …
thereby maintaining lung volumes during the respiratory cycle. In premature newborn …
Lung diseases in children associated with inherited disorders of surfactant metabolism
C Delestrain, F Flamein, L Jonard… - Revue de …, 2013 - europepmc.org
Pulmonary surfactant is a unique mixture of lipids and specific proteins that reduces surface
tension at the air-liquid interface, preventing collapse of the lung at the end of expiration …
tension at the air-liquid interface, preventing collapse of the lung at the end of expiration …
Surfactant Function Disorders: Genetics Is Just the Tip of the Iceberg
K Keegan, K Ruff, G Burg, LE Berklite… - … AND DIFFUSE LUNG …, 2024 - atsjournals.org
Introduction: Surfactant function disorders are an increasingly recognized cause of pediatric
interstitial lung disease. With improvements in genetic analysis speed and accuracy, these …
interstitial lung disease. With improvements in genetic analysis speed and accuracy, these …
Surfactant deficiency disorders: SP-B and ABCA3
LM Nogee - Molecular Basis of Pulmonary Disease: Insights from …, 2010 - Springer
Single gene disorders disrupting surfactant metabolism and resulting in acute and chronic
lung disease have been identified in recent years. This review focuses on lung disease …
lung disease have been identified in recent years. This review focuses on lung disease …
Surfactant dysfunction mutations in children's interstitial lung disease and beyond
R Deterding, LL Fan - … journal of respiratory and critical care …, 2005 - search.proquest.com
Surfactant has been an important focus in pediatrics since the recognition of surfactant
deficiency in premature infants (1). It seemed logical that derangements in this system could …
deficiency in premature infants (1). It seemed logical that derangements in this system could …