An adolescent with idiopathic pleuroparenchymal fibroelastosis. Case report


Atag E., Ikizoglu N. B. , Gökdemir Y. , Erdem Eralp E. , Kıyan G. , Yilmazbayhan D., ...Daha Fazla

ARCHIVOS ARGENTINOS DE PEDIATRIA, cilt.116, sa.1, 2018 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 116 Konu: 1
  • Basım Tarihi: 2018
  • Doi Numarası: 10.5546/aap.2018.eng.e147
  • Dergi Adı: ARCHIVOS ARGENTINOS DE PEDIATRIA

Özet

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades.