Hermansky-Pudlak Syndrome and Lung Disease: Pathogenesis and Therapeutics

Hermansky-Pudlak Syndrome (HPS) is a rare, genetic, multisystem disorder characterized by oculocutaneous albinism (OCA), bleeding diathesis, immunodeficiency, granulomatous colitis, and pulmonary fibrosis. HPS pulmonary fibrosis (HPS-PF) occurs in 100% of patients with subtype HPS-1 and has a simila...

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Main Authors: Pamela Velázquez-Díaz (Author), Erika Nakajima (Author), Parand Sorkhdini (Author), Ashley Hernandez-Gutierrez (Author), Adam Eberle (Author), Dongqin Yang (Author), Yang Zhou (Author)
Format: Book
Published: Frontiers Media S.A., 2021-03-01T00:00:00Z.
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3rd Floor Main Library

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