Hermansky-Pudlak syndrome-associated pneumothorax with rapid progression of respiratory failure: a case report

Hermansky-Pudlak syndrome-associated pneumothorax with rapid progression of respiratory failure: a case report

Abstract Background Hermansky-Pudlak syndrome (HPS) is an extremely rare disease with pulmonary fibrosis (PF), oculocutaneous albinism, induced platelet dysfunction, and granulomatous colitis. Although patients with HPS-associated PF (HPS-PF) often receive treatment with anti-fibrotic agents, includ...

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Bibliographic Details
Main Authors: Yukari Kato, Motoyasu Kato, Hiroaki Ihara, Eri Hayakawa, Kohei Shibayama, Keita Miura, Tomoko Yamada, Yoichiro Mitsuishi, Takehito Shukuya, Jun Ito, Takeshi Matsunaga, Tadashi Sato, Kenji Suzuki, Kazuhisa Takahashi
Format: Article
Language:English
Published: BMC 2020-10-01
Series:BMC Pulmonary Medicine
Subjects:
Chest drainage
Hermansky-Pudlak syndrome
Pneumothorax
Pulmonary fibrosis
Pirfenidone
Online Access:http://link.springer.com/article/10.1186/s12890-020-01302-8

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http://link.springer.com/article/10.1186/s12890-020-01302-8

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