A case of diffuse panbronchiolitis, associated with severe pulmonary hypertension, managed with bilateral lung transplantation from a brain-dead donor

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• 作者：Yasutaka Onishi ; M.D.^a ; ^{yonishi@hmj-net.hosp.go.jp" class="auth_mail" title="E-mail the corresponding author} ; Tetsuji Kawamura ; M.D.^a ; ^{kawa-t@hmj-net.hosp.go.jp" class="auth_mail" title="E-mail the corresponding author} ; Akie Morimoto ; M.D.^a ; ^{a.morimoto@hmj-net.hosp.go.jp" class="auth_mail" title="E-mail the corresponding author} ; Yasuharu Nakahara ; M.D.^a ; ^{nakahary@hmj-net.hosp.go.jp" class="auth_mail" title="E-mail the corresponding author} ; Yoshiro Mochizuki ; M.D.^a ; ^{mochi@hmj-net.hosp.go.jp" class="auth_mail" title="E-mail the corresponding author} ; Kentaroh Miyoshi ; M.D.^b ; ^{kentarohmiyoshi@yahoo.co.jp" class="auth_mail" title="E-mail the corresponding author} ; Takahiro Oto ; M.D.^b ; ^{oto@md.okayama-u.ac.jp" class="auth_mail" title="E-mail the corresponding author}
• 关键词：Diffuse panbronchiolitis ; Brain-dead donor ; Lung transplantation ; Chronic sinusitis ; Pulmonary hypertension
• 刊名：Respiratory Investigation
• 出版年：2015
• 出版时间：September 2015
• 年：2015
• 卷：53
• 期：5
• 页码：242-246
• 全文大小：1142 K

文摘

Diffuse panbronchiolitis (DPB) is a chronic respiratory disease that mainly involves the respiratory bronchioles, and has historically been associated with a very poor prognosis. The development of long-term low dose macrolide therapy in the 1980s has dramatically improved the prognosis of DPB. Nevertheless, some cases are resistant to macrolide therapy, and ultimately develop severe respiratory failure and pulmonary hypertension; in such cases lung transplantation is a viable treatment option. Here we report the case of a 40-year-old patient with a 20-year history of DPB, who underwent bilateral lung transplantation due to severe respiratory failure with pulmonary hypertension.