Severe and progressive platythorax disproportionate to lung fibrosis : A rare variant of idiopathic pleuroparenchymal fibroelastosis
© 2021 The Author(s)..
A 57-year-old man was referred to our department because of progressive shortness of breath and emaciation. He had experienced pneumothorax three times in the past five years. The patient radiologically showed mild upper-lobe predominant airspace consolidation and severe platythorax and was clinically diagnosed with idiopathic pleuroparenchymal fibroelastosis (PPFE). Although the wedge-shaped shadows in the bilateral lung apexes did not significantly progress, his platythorax gradually worsened during the clinical course. He ultimately died of chronic respiratory failure 1.2 years after the diagnosis. This case demonstrates a rare variant of idiopathic PPFE with progressive platythorax disproportionate to the extent of upper-lobe fibroelastosis.
Medienart: |
E-Artikel |
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Erscheinungsjahr: |
2021 |
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Erschienen: |
2021 |
Enthalten in: |
Zur Gesamtaufnahme - volume:33 |
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Enthalten in: |
Respiratory medicine case reports - 33(2021) vom: 27., Seite 101395 |
Sprache: |
Englisch |
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Beteiligte Personen: |
Ikeda, Takato [VerfasserIn] |
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Links: |
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Themen: |
Case Reports |
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Anmerkungen: |
Date Revised 06.04.2021 published: Electronic-eCollection Citation Status PubMed-not-MEDLINE |
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doi: |
10.1016/j.rmcr.2021.101395 |
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funding: |
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Förderinstitution / Projekttitel: |
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PPN (Katalog-ID): |
NLM323715109 |
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520 | |a A 57-year-old man was referred to our department because of progressive shortness of breath and emaciation. He had experienced pneumothorax three times in the past five years. The patient radiologically showed mild upper-lobe predominant airspace consolidation and severe platythorax and was clinically diagnosed with idiopathic pleuroparenchymal fibroelastosis (PPFE). Although the wedge-shaped shadows in the bilateral lung apexes did not significantly progress, his platythorax gradually worsened during the clinical course. He ultimately died of chronic respiratory failure 1.2 years after the diagnosis. This case demonstrates a rare variant of idiopathic PPFE with progressive platythorax disproportionate to the extent of upper-lobe fibroelastosis | ||
650 | 4 | |a Case Reports | |
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700 | 1 | |a Kushima, Hisako |e verfasserin |4 aut | |
700 | 1 | |a Ishii, Hiroshi |e verfasserin |4 aut | |
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