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Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male
Pulmonary alveolar microlithiasis (PAM) is an uncommon hereditary lung disease characterized by widespread deposition of calcium phosphate microliths within the alveolar spaces. It is considered an autosomal recessive disease with a mutation in a gene encoding a sodium phosphate cotransporter. The i...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Hindawi
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7204375/ https://www.ncbi.nlm.nih.gov/pubmed/32411490 http://dx.doi.org/10.1155/2020/6247920 |
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author | Menon, Preethi Dileep Hackman, Sarah |
author_facet | Menon, Preethi Dileep Hackman, Sarah |
author_sort | Menon, Preethi Dileep |
collection | PubMed |
description | Pulmonary alveolar microlithiasis (PAM) is an uncommon hereditary lung disease characterized by widespread deposition of calcium phosphate microliths within the alveolar spaces. It is considered an autosomal recessive disease with a mutation in a gene encoding a sodium phosphate cotransporter. The imaging findings in the early phase of disease can be mistaken for miliary tuberculosis or sarcoidosis. However, the classic radiologic findings in the later phases of disease show numerous opacities causing a “snowstorm” appearance to the lungs that corresponds with widespread deposition of microliths throughout the lung parenchyma. Although the disease often progresses over a slow time course, there are no effective therapies, and bilateral lung transplantation is recommended when there are increasing oxygen requirements or evidence of pulmonary hypertension. |
format | Online Article Text |
id | pubmed-7204375 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Hindawi |
record_format | MEDLINE/PubMed |
spelling | pubmed-72043752020-05-14 Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male Menon, Preethi Dileep Hackman, Sarah Case Rep Pathol Case Report Pulmonary alveolar microlithiasis (PAM) is an uncommon hereditary lung disease characterized by widespread deposition of calcium phosphate microliths within the alveolar spaces. It is considered an autosomal recessive disease with a mutation in a gene encoding a sodium phosphate cotransporter. The imaging findings in the early phase of disease can be mistaken for miliary tuberculosis or sarcoidosis. However, the classic radiologic findings in the later phases of disease show numerous opacities causing a “snowstorm” appearance to the lungs that corresponds with widespread deposition of microliths throughout the lung parenchyma. Although the disease often progresses over a slow time course, there are no effective therapies, and bilateral lung transplantation is recommended when there are increasing oxygen requirements or evidence of pulmonary hypertension. Hindawi 2020-03-27 /pmc/articles/PMC7204375/ /pubmed/32411490 http://dx.doi.org/10.1155/2020/6247920 Text en Copyright © 2020 Preethi Dileep Menon and Sarah Hackman. http://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 Menon, Preethi Dileep Hackman, Sarah Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male |
title | Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male |
title_full | Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male |
title_fullStr | Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male |
title_full_unstemmed | Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male |
title_short | Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male |
title_sort | pulmonary alveolar microlithiasis: an isolated case in a hispanic male |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7204375/ https://www.ncbi.nlm.nih.gov/pubmed/32411490 http://dx.doi.org/10.1155/2020/6247920 |
work_keys_str_mv | AT menonpreethidileep pulmonaryalveolarmicrolithiasisanisolatedcaseinahispanicmale AT hackmansarah pulmonaryalveolarmicrolithiasisanisolatedcaseinahispanicmale |