ArticleJournal of thoracic disease2020
Pleural changes in patients with pneumothoraces and Marfan syndrome.
Article in Journal of thoracic disease, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed, 3 citations in OpenAlex.
- Latent Vasculopathy of a Peripheral Pulmonary Artery in FBN1-Related Marfan Syndrome.Pathology international · 2026Article
- Postoperative pneumothorax occurrence in patients with Marfan syndrome.General thoracic and cardiovascular surgery · 2025Article
- Tracheal Stenosis Associated With Operation for Pneumothorax With Marfan Syndrome: A Case Report.Cureus · 2024Article
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPatients with Marfan syndrome (MFS) often develop pneumothorax, but the features of pneumothorax in the context of MFS have not been well described in the literature. We clarified the clinical and histopathological characteristics of this condition in these patients.
methodsPatients with MFS were selected from among all patients who underwent surgery for pneumothorax, between December 1991 and January 2015, in our hospital. We studied the histopathological characteristics of the resected lungs as well as the clinical features of the selected patients, including surgical findings and postoperative recurrence status.
resultsThere were 966 operations underwent pneumothorax-related surgeries in our hospital. A total of 16 operations (1.66%) were performed on patients with MFS in 11 cases. In this study, 9 patients (6 men, 3 women) were included. Clinically, 7 patients (77.8%) had bilateral pneumothoraces and 4 (44.4%) exhibited postoperative recurrent pneumothoraces. Pathologically, the resected pulmonary bullae exhibited blood vessel cystic medial degeneration (55.6% of cases), calcification (55.6% of cases), and demonstrated elastic fiber fragmentation and degeneration (all cases).
conclusionsAs in few previous reports, many patients with MFS develop bilateral or postoperative recurrent pneumothoraces. In many patients, characteristic changes in the pulmonary bullae, possibly caused by degenerated elastic fibers, were observed.
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