Life‐threatening, giant pneumatoceles in the course of surfactant protein C deficiency. Issue 7 (9th February 2015)

• Record Type: Journal Article
• Authors: Sismanlar, Tugba; Aslan, Ayse Tana; Griese, Matthias
• Is Part Of: Pediatric pulmonology. Volume 50:Issue 7(2015:Jul.)
• Page Start: E25
• Page End: E28
• Publication Date: 2015-02-09
• Subjects: Pediatric respiratory diseases -- Periodicals; Pediatrics -- Periodicals; 618.922
• DOI: 10.1002/ppul.23162
• Languages: English
• ISSNs: 8755-6863

Abstract

<abstract abstract-type="main" xml:lang="en"> <title>Summary</title> <sec id="ppul23162-sec-0001" sec-type="section"> <p>Chronic interstitial lung diseases are rare in childhood and can present with a wide spectrum of histological abnormalities and radiological‐clinical phenotypes. A 17‐month‐old female infant with malnutrition, recurrent lower respiratory tract infections, and failure to thrive since 3 months of age was diagnosed as surfactant protein C deficiency. Diffuse, giant, and life‐threatening pneumatoceles developed during the course. They were treated with empiric drug treatment and oxygen support, and resolved rapidly. Substantial clinical and radiological improvement was observed 1 year after treatment initiation. Large‐giant pneumatoceles can develop in the course of surfactant protein C deficiency and may be associated with biopsy. They can resolve with medical treatment. If available, genetic testing should be attempted as a first step for diagnosis. <bold>Pediatr Pulmonol. 2015; 50:E25–E28.</bold> © 2015 Wiley Periodicals, Inc.</p> </sec> </abstract>