Primary pulmonary lymphangiectasia in Noonan syndrome: apropos of an extremely rare manifestation and a brief literature review
| Autor: | Georgia-Emmanuela Dendrinou, Panagiotis Zagarelos, Angelos Sofronas, Stamatis Katsenos |
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| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: | |
| Zdroj: | Monaldi Archives for Chest Disease, Vol 90, Iss 4 (2020) |
| Druh dokumentu: | article |
| ISSN: | 1122-0643 2532-5264 |
| DOI: | 10.4081/monaldi.2020.1576 |
| Popis: | Noonan syndrome (NS) is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, bleeding difficulties and lymphatic malformations. Although lymphatic dysplasias are present in 20% of patients with NS, however pulmonary lymphangiectasia has rarely been described. In this present paper, we report a 24-year-old male who was diagnosed with Noonan syndrome and primary pulmonary lymphangiectasia by using chest imaging modalities. A brief overview of the current literature is also provided laying emphasis on the clinical, pathogenetic and diagnostic aspects of this uncommon Noonan syndrome complication. |
| Databáze: | Directory of Open Access Journals |
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