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

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

PEDIATRIC PULMONOLOGY, cilt.50, sa.7, 2015 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 50 Sayı: 7
  • Basım Tarihi: 2015
  • Doi Numarası: 10.1002/ppul.23162
  • Dergi Adı: PEDIATRIC PULMONOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Gazi Üniversitesi Adresli: Evet

Özet

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.