Life-Threatening, Giant Pneumatoceles in the Course of Surfactant Protein C Deficiency


ŞİŞMANLAR EYÜBOĞLU T. , ASLAN A. T. , Griese M.

PEDIATRIC PULMONOLOGY, vol.50, no.7, 2015 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 50 Issue: 7
  • Publication Date: 2015
  • Doi Number: 10.1002/ppul.23162
  • Title of Journal : PEDIATRIC PULMONOLOGY

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. (C) 2015 Wiley Periodicals, Inc.