Prolonged treatment with open-label pirfenidone in Hermansky-Pudlak syndrome pulmonary fibrosis


O'Brien K. J. , Introne W. J. , Akal O., AKAL T. , Barbu A., McGowan M. P. , ...Daha Fazla

MOLECULAR GENETICS AND METABOLISM, cilt.125, ss.168-173, 2018 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 125
  • Basım Tarihi: 2018
  • Doi Numarası: 10.1016/j.ymgme.2018.07.012
  • Dergi Adı: MOLECULAR GENETICS AND METABOLISM
  • Sayfa Sayıları: ss.168-173

Özet

Purpose: Limited information is available regarding chronic treatment with pirfenidone, an anti-fibrotic drug. Effects of long-term open-label pirfenidone were evaluated in a small cohort with Hermansky-Pudlak syndrome (HPS), a rare autosomal recessive disorder with highly penetrant pulmonary fibrosis.