title: Life-threatening, giant pneumatoceles in the course of surfactant protein C deficiency.
abstract: 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.
authors: Department of Pediatric Pulmonology, Gazi University Hospital, Ankara, Turkey.
authors: Tugba
authors: T
authors: Sismanlar
authors: Department of Pediatric Pulmonology, Gazi University Hospital, Ankara, Turkey.
authors: Ayse Tana
authors: AT
authors: Aslan
authors: Department of Pediatric Pulmonology, Hauner Children's Hospital, Ludwig Maximilians University, Member of the German Center for Lung Research (DZL), Munich, Germany.
authors: Matthias
authors: M
authors: Griese
mesh_terms: D000388:Air; D003560:Cysts / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D007223:Infant; D008171:Lung Diseases / Q000000981:diagnostic imaging*; D020151:Protein C Deficiency / Q000150:complications*; D011859:Radiography
keywords: children; interstitial lung disease (ILD); pneumatocel; surfactant protein C deficiency