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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Frontiers Media S.A.,
2021-03-01T00:00:00Z.
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A1234.567 |
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