• Internal medicine · Jun 2020

    Case Reports

    An Autopsy Case of Idiopathic Pleuroparenchymal Fibroelastosis with Left Vocal Cord Paralysis and a Rapid Deterioration without an Acute Exacerbation.

    • Chizuru Futatsuya, Hiroshi Minato, Yurie Okayama, Kazuyoshi Katayanagi, Hiroshi Kurumaya, Mizuki Yuasa, and Koichi Nishi.
    • Department of Diagnostic Pathology, Ishikawa Prefectural Central Hospital, Japan.
    • Intern. Med. 2020 Jun 15; 59 (12): 1541-1547.

    AbstractIdiopathic pleuroparenchymal fibroelastosis (IPPFE) is a generally slow-progressing rare disorder of unknown etiology. The direct cause of death in cases of IPPFE is rarely investigated. We experienced an autopsy case of a Japanese man with IPPFE and found aspiration pneumonia to be the major trigger of death. The individual had left vocal cord paralysis at admission, which may have contributed to aspiration pneumonia, and which probably was affected by the fibrous adhesion of the left apex of the chest wall resulting from IPPFE. The prevention of aspiration pneumonia is important for maintaining the respiratory function, especially in IPPFE patients with repeated pneumothorax.

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