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doi:10.1378/chest.07-2652
(Chest. 2008; 134:192-195)
© 2008 American College of Chest Physicians
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Usual Interstitial Pneumonia in an Adolescent With ABCA3 Mutations*

Lisa R. Young, MD; Lawrence M. Nogee, MD, FCCP; Bruce Barnett, MD; Ralph J. Panos, MD; Thomas V. Colby, MD, FCCP and Gail H. Deutsch, MD

* From the Division of Pulmonary Medicine (Dr. Young) and Department of Pathology (Dr. Deutsch), Cincinnati Children’s Hospital Medical Center, Cincinnati, OH; Division of Pulmonary, Critical Care, and Sleep Medicine (Dr. Panos), University of Cincinnati, Cincinnati, OH; Division of Neonatology (Dr. Nogee), Department of Pediatrics, Johns Hopkins University, Baltimore, MD; Division of Pulmonary Medicine (Dr. Barnett), Toledo Children’s Hospital, Toledo, OH; and Department of Pathology (Dr. Colby), Mayo Clinic, Scottsdale, AZ.

Correspondence to: Lisa R. Young, MD, Division of Pulmonary Medicine, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, MLC 2021, Cincinnati, OH 45229-3039; e-mail: Lisa.Young{at}cchmc.org

Many diverse and frequently idiopathic disorders cause interstitial lung disease (ILD) in children. Although the histologic patterns of ILD in children and adults share similar features, important differences exist in etiology, clinical manifestations, and outcome. Usual interstitial pneumonia (UIP) is the most frequent histologic pattern in adult ILD; however, the characteristic histologic features of UIP have yet to be demonstrated in a child. We report a 15-year-old boy with the UIP pattern of pulmonary fibrosis who had mutations in the adenosine triphosphate-binding-cassette-A3 gene. Discovery of how genetic mutations of proteins involved in surfactant biosynthesis lead to progressive fibrosis will have implications for the understanding of the pathogenesis and clinical manifestations of ILD in both adults and children.

Key Words: ABCA3 • children • interstitial lung disease • surfactant • usual interstitial pneumonia






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