Article
Hermansky-Pudlak syndrome-associated pneumothorax with rapid progression of respiratory failure: a case report.
BMC pulmonary medicine - 6 Oct 2020
Kato Yukari, Kato Motoyasu, Ihara Hiroaki, Hayakawa Eri, Shibayama Kohei, Miura Keita, Yamada Tomoko, Mitsuishi Yoichiro, Shukuya Takehito, Ito Jun, Matsunaga Takeshi, Sato Tadashi, Suzuki Kenji, Takahashi Kazuhisa
Abstract excerpt
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, including pirfenidone, many HPS-PF cases are progressive. The development of pneumothorax is known to be rare in HPS-PF....
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.