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Surfactant Protein Deficiency Syndrome in Childhood Interstitial Lung Disease
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  • Jagdev Singh,
  • Adam Jaffe,
  • André Schultz,
  • Hiran Selvadurai
Jagdev Singh
Children's Hospital at Westmead
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Adam Jaffe
The Sydney Children's Hospitals Network Randwick and Westmead
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André Schultz
Perth Children's Hospital
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Hiran Selvadurai
The Children\'s Hospital at Westmead
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Abstract

Surfactant, which was first identified in the 1920s is pivotal to lower the surface tension in alveoli of the lungs and helps to lower the work of breathing and prevents atelectasis. Surfactant proteins, such as surfactant protein B and surfactant protein C contribute to normal functioning of surfactant. Additionally, Adenosine Triphosphate Binding Cassette 3 and Thyroid Transcription Factor-1 are also integral for the normal structure and functioning of pulmonary surfactant. Through the study and improved understanding of surfactant over the decades, there is increasing interest into the study of childhood interstitial lung diseases (chILD) in the context of surfactant protein deficiencies. Surfactant protein deficiency syndrome (SPDS) is a group of rare diseases within the chILD group that is caused by genetic mutations of SFTPB, SPTPC, ABCA3 and TTF1 genes. This review article seeks to provide an overview of surfactant protein deficiencies in the context of chILD.