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Hermansky-Pudlak Syndrome Complicated by Pulmonary Fibrosis: Radiologic-Pathologic Correlation and Review of Pulmonary Complications

Hermansky–Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous hypopigmentation, platelet dysfunction, and in many cases, life-threatening pulmonary fibrosis. We report the clinical course, imaging, and postmortem findings of a 38-year-old female with HPS-rela...

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Detalles Bibliográficos
Autores principales: Kelil, Tatiana, Shen, Jeanne, O’Neill, Ailbhe C, Howard, Stephanie A
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4220421/
https://www.ncbi.nlm.nih.gov/pubmed/25379352
http://dx.doi.org/10.4103/2156-7514.143437