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 Vol. 9 No. 10, October 2008
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NeoReviews Vol.9 No.10 2008 e468
© 2008 American Academy of Pediatrics

Lung Transplantation for Inherited Disorders of Surfactant Metabolism

Albert Faro, MD*
Aaron Hamvas, MD*

* Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, Mo

Inherited disorders of surfactant metabolism are rare diseases that provide unique opportunities for understanding the mechanisms of surfactant function and metabolism. Presenting both as acute respiratory dysfunction in the neonatal period with a predictably lethal outcome as well as chronic respiratory insufficiency of variable onset and unpredictable natural histories, these disorders present unique challenges for clinicians and families because the treatment options are limited to lung transplantation and compassionate care. We briefly review the known inherited surfactant disorders, the challenges associated with offering lung transplantation for these disorders, and the outcomes.

Abbreviations: ABCA3: adenosine triphosphate-binding cassette member A3 • ECMO: extracorporeal membrane oxygenation • RDS: respiratory distress syndrome • SP: surfactant protein


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