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Pediatr Pulmonol. 2015 Jul;50(7):E25-8. doi: 10.1002/ppul.23162. Epub 2015 Feb 9.

Life-threatening, giant pneumatoceles in the course of surfactant protein C deficiency.

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Department of Pediatric Pulmonology, Gazi University Hospital, Ankara, Turkey.
Department of Pediatric Pulmonology, Hauner Children's Hospital, Ludwig Maximilians University, Member of the German Center for Lung Research (DZL), Munich, Germany.


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.


children; interstitial lung disease (ILD); pneumatocel; surfactant protein C deficiency

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