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Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia
Pulmonary lymphangiectasia (PL) is a rare congenital disorder of pulmonary lymphatic development. Although it was traditionally a fatal disorder of infancy, some cases in later childhood have been reported, suggesting a spectrum of severity. We present an unusual case of unilateral, congenital pulmo...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2021
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8100182/ https://www.ncbi.nlm.nih.gov/pubmed/33968857 http://dx.doi.org/10.3389/fped.2021.657473 |
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author | Adair, Dionne Rabah, Raja Ladino-Torres, Maria Saba, Thomas G. |
author_facet | Adair, Dionne Rabah, Raja Ladino-Torres, Maria Saba, Thomas G. |
author_sort | Adair, Dionne |
collection | PubMed |
description | Pulmonary lymphangiectasia (PL) is a rare congenital disorder of pulmonary lymphatic development. Although it was traditionally a fatal disorder of infancy, some cases in later childhood have been reported, suggesting a spectrum of severity. We present an unusual case of unilateral, congenital pulmonary lymphangiectasia. Our patient presented with neonatal respiratory distress, a chronic wet cough and recurrent episodes of bronchitis. Chest CT revealed thickening of the interlobular septae of the right lung. A lung biopsy confirmed the diagnosis of lymphangiectasia. His clinical course is characterized by chronic coughing and recurrent bronchitis but normal growth and development. This case illustrates a relatively mild presentation of unilateral PL, which, along with other reports, suggests variability in the presentation and severity of this disorder. In the absence of imaging and histological examination, mild presentations may go undiagnosed. |
format | Online Article Text |
id | pubmed-8100182 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-81001822021-05-07 Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia Adair, Dionne Rabah, Raja Ladino-Torres, Maria Saba, Thomas G. Front Pediatr Pediatrics Pulmonary lymphangiectasia (PL) is a rare congenital disorder of pulmonary lymphatic development. Although it was traditionally a fatal disorder of infancy, some cases in later childhood have been reported, suggesting a spectrum of severity. We present an unusual case of unilateral, congenital pulmonary lymphangiectasia. Our patient presented with neonatal respiratory distress, a chronic wet cough and recurrent episodes of bronchitis. Chest CT revealed thickening of the interlobular septae of the right lung. A lung biopsy confirmed the diagnosis of lymphangiectasia. His clinical course is characterized by chronic coughing and recurrent bronchitis but normal growth and development. This case illustrates a relatively mild presentation of unilateral PL, which, along with other reports, suggests variability in the presentation and severity of this disorder. In the absence of imaging and histological examination, mild presentations may go undiagnosed. Frontiers Media S.A. 2021-04-22 /pmc/articles/PMC8100182/ /pubmed/33968857 http://dx.doi.org/10.3389/fped.2021.657473 Text en Copyright © 2021 Adair, Rabah, Ladino-Torres and Saba. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Pediatrics Adair, Dionne Rabah, Raja Ladino-Torres, Maria Saba, Thomas G. Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia |
title | Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia |
title_full | Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia |
title_fullStr | Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia |
title_full_unstemmed | Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia |
title_short | Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia |
title_sort | case report: a relatively mild presentation of unilateral congenital pulmonary lymphangiectasia |
topic | Pediatrics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8100182/ https://www.ncbi.nlm.nih.gov/pubmed/33968857 http://dx.doi.org/10.3389/fped.2021.657473 |
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