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-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 116 Sayı: 1
  • Basım Tarihi: 2018
  • Doi Numarası: 10.5546/aap.2018.eng.e147
  • Dergi Adı: ARCHIVOS ARGENTINOS DE PEDIATRIA
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Marmara Üniversitesi Adresli: Evet

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.