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Usual Interstitial Pneumonia in an Adolescent With ABCA3 Mutations
Chest, 07/17/08
Young LR et al. - This is a case report of 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.
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