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.