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Infant respiratory distress syndrome (IRDS), also known as surfactant deficiency disorder (SDD), [2] and previously called hyaline membrane disease (HMD), is a syndrome in premature infants caused by developmental insufficiency of pulmonary surfactant production and structural immaturity in the lungs.
It is characterized by symmetric fine granular opacities, air bronchograms. It does not have pleural effusions because the lung dysfunction is not due to excess fluid (as can occur with a non-compressive Cesaerean section), but decreased surfactant, causing the lungs to be inelastic and crumpled. [6]
Most disease-causing mutations in SFTPB result in a complete lack of mature SP-B protein 265120. Lung disease is inherited in an autosomal recessive manner, requiring mutations in both alleles. Surfactant produced by infants with SP-B deficiency is abnormal in composition and does not function normally in lowering surface tension. [citation needed]
The edema contributes to the deposition of a hyaline membrane (composed of dead cells, surfactant, and proteins) along the alveolar walls. Hyaline membranes are characteristic of DAD. The edema interferes with the naturally occurring surfactant, which is critical for reducing surface tension and allowing alveoli to remain open and allow air in ...
The major surfactant deficiency in premature infants relates to the lack of phosphatidylglycerol, even though it comprises less than 5% of pulmonary surfactant phospholipids. It is synthesized by head group exchange of a phosphatidylcholine enriched phospholipid using the enzyme phospholipase D .
Surfactant Dysfunction Disorder is a disease that affects newborn children whose pulmonary surfactant is insufficient for adequate breathing, resulting in respiratory distress syndrome (RDS). [ 16 ] Despite DPPC being one of the major components of lung surfactant, most of the genetic errors that are linked with surfactant dysfunction disorder ...
My heart broke the first time I told a patient they had a terminal disease. I cried myself to sleep the night when, as a third-year medical student, the man I assured would do fine during his ...
Surfactant deficiency is a common cause of respiratory disease. Respiratory distress syndrome (RDS) is a particularly well-known instance of surfactant deficiency because it has a high mortality rate among preterm babies, a variety of other conditions are related to surfactant levels and composition. [21]