Atıf İçin Kopyala
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-Expanded)
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Yayın Türü:
Makale / Tam Makale
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Cilt numarası:
125
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Basım Tarihi:
2018
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Doi Numarası:
10.1016/j.ymgme.2018.07.012
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Dergi Adı:
MOLECULAR GENETICS AND METABOLISM
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Derginin Tarandığı İndeksler:
Science Citation Index Expanded (SCI-EXPANDED), Scopus
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Sayfa Sayıları:
ss.168-173
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Anahtar Kelimeler:
Hermansky-Pudlak syndrome, pirfenidone, pulmonary fibrosis, INTERSTITIAL LUNG-DISEASE, SYNDROME TYPE-2, MUTATIONS, GENE, FORM, CT, IMMUNODEFICIENCY, ORGANELLES, ADAPTER
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Orta Doğu Teknik Üniversitesi Adresli:
Evet
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.