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 Vol. 9 No. 10, October 2008
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NeoReviews Vol.9 No.10 2008 e458
© 2008 American Academy of Pediatrics

Inherited Surfactant Disorders

W. Adam Gower, MD*
Susan E. Wert, PhD{dagger}
Lawrence M. Nogee, MD{ddagger}

* Eudowood Division of Pediatric Respiratory Sciences, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Md
{dagger} Divisions of Neonatology and Pulmonary Biology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center & University of Cincinnati College of Medicine, Cincinnati, Ohio
{ddagger} Eudowood Neonatal Pulmonary Division, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Md

Inherited disorders of surfactant metabolism are rare causes of respiratory disease in newborns but are associated with significant morbidity and mortality. This review outlines the molecular basis and pathophysiology of the three currently identified single-gene disorders of surfactant metabolism as well as the clinical presentations and evaluation of potentially affected infants. Implications for the understanding of normal surfactant metabolism and the potential roles of surfactant dysfunction mutations in more common neonatal disorders, such as respiratory distress syndrome, also are discussed.

Abbreviations: ABCA3: adenosine triphosphate-binding cassette member A3 • DPPC: dipalmitoyl phosphatidylcholine • DSPC: disaturated phosphatidylcholine • ILD: interstitial lung disease • PC: phosphatidylcholine • PG: phosphatidylglycerol • RDS: respiratory distress syndrome • SP: surfactant protein • TTF-1: thyroid transcription factor-1


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