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Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse

Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which defects in alveolar macrophage maturation or function lead to the accumulation of proteinaceous surfactant in alveolar space, resulting in impaired gas exchange and hypoxemia. PAP is categorized into three types: hereditary, autoi...

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Autores principales: Li, Meirui, Alowami, Salem, Schell, Miranda, Davis, Clive, Naqvi, Asghar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5828087/
https://www.ncbi.nlm.nih.gov/pubmed/29607238
http://dx.doi.org/10.1155/2018/5202173
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author Li, Meirui
Alowami, Salem
Schell, Miranda
Davis, Clive
Naqvi, Asghar
author_facet Li, Meirui
Alowami, Salem
Schell, Miranda
Davis, Clive
Naqvi, Asghar
author_sort Li, Meirui
collection PubMed
description Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which defects in alveolar macrophage maturation or function lead to the accumulation of proteinaceous surfactant in alveolar space, resulting in impaired gas exchange and hypoxemia. PAP is categorized into three types: hereditary, autoimmune, and secondary. We report a case of secondary PAP in a 47-year-old man, whose risk factors include occupational exposure to inhaled toxins, especially aluminum dust, the use of anabolic steroids, and alcohol abuse, which in mice leads to alveolar macrophage dysfunction through a zinc-dependent mechanism that inhibits granulocyte macrophage-colony stimulating factor (GM-CSF) receptor signalling. Although the rarity and vague clinical presentation of PAP can pose diagnostic challenges, clinician awareness of PAP risk factors may facilitate the diagnostic process and lead to more prompt treatment.
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spelling pubmed-58280872018-04-01 Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse Li, Meirui Alowami, Salem Schell, Miranda Davis, Clive Naqvi, Asghar Case Rep Pulmonol Case Report Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which defects in alveolar macrophage maturation or function lead to the accumulation of proteinaceous surfactant in alveolar space, resulting in impaired gas exchange and hypoxemia. PAP is categorized into three types: hereditary, autoimmune, and secondary. We report a case of secondary PAP in a 47-year-old man, whose risk factors include occupational exposure to inhaled toxins, especially aluminum dust, the use of anabolic steroids, and alcohol abuse, which in mice leads to alveolar macrophage dysfunction through a zinc-dependent mechanism that inhibits granulocyte macrophage-colony stimulating factor (GM-CSF) receptor signalling. Although the rarity and vague clinical presentation of PAP can pose diagnostic challenges, clinician awareness of PAP risk factors may facilitate the diagnostic process and lead to more prompt treatment. Hindawi 2018-01-23 /pmc/articles/PMC5828087/ /pubmed/29607238 http://dx.doi.org/10.1155/2018/5202173 Text en Copyright © 2018 Meirui Li et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Li, Meirui
Alowami, Salem
Schell, Miranda
Davis, Clive
Naqvi, Asghar
Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse
title Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse
title_full Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse
title_fullStr Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse
title_full_unstemmed Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse
title_short Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse
title_sort pulmonary alveolar proteinosis in setting of inhaled toxin exposure and chronic substance abuse
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5828087/
https://www.ncbi.nlm.nih.gov/pubmed/29607238
http://dx.doi.org/10.1155/2018/5202173
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