A case of diffuse panbronchiolitis, associated with severe pulmonary hypertension, managed with bilateral lung transplantation from a brain-dead donor.
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.
Department of Respiratory Medicine, National Hospital Organization, Himeji Medical Center, Honnmachi 68, Himeji City 670-8520, Hyogo, Japan. Electronic address: yonishi@hmj-net.hosp.go.jp.
Yasutaka
Y
Onishi
Department of Respiratory Medicine, National Hospital Organization, Himeji Medical Center, Honnmachi 68, Himeji City 670-8520, Hyogo, Japan. Electronic address: kawa-t@hmj-net.hosp.go.jp.
Tetsuji
T
Kawamura
Department of Respiratory Medicine, National Hospital Organization, Himeji Medical Center, Honnmachi 68, Himeji City 670-8520, Hyogo, Japan. Electronic address: a.morimoto@hmj-net.hosp.go.jp.
Akie
A
Morimoto
Department of Respiratory Medicine, National Hospital Organization, Himeji Medical Center, Honnmachi 68, Himeji City 670-8520, Hyogo, Japan. Electronic address: nakahary@hmj-net.hosp.go.jp.
Yasuharu
Y
Nakahara
Department of Respiratory Medicine, National Hospital Organization, Himeji Medical Center, Honnmachi 68, Himeji City 670-8520, Hyogo, Japan. Electronic address: mochi@hmj-net.hosp.go.jp.
Yoshiro
Y
Mochizuki
Department of Thoracic Surgery, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama 700-8558, Japan. Electronic address: kentarohmiyoshi@yahoo.co.jp.
Kentaroh
K
Miyoshi
Department of Thoracic Surgery, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama 700-8558, Japan. Electronic address: oto@md.okayama-u.ac.jp.
Takahiro
T
Oto
D000328:Adult; D001926:Brain Death; D001988:Bronchiolitis / Q000150:complications* / Q000601:surgery*; D005260:Female; D006192:Haemophilus Infections / Q000150:complications* / Q000601:surgery*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000601:surgery*; D016040:Lung Transplantation / Q000379:methods*; D012131:Respiratory Insufficiency / Q000209:etiology / Q000601:surgery; D012720:Severity of Illness Index; D014019:Tissue Donors; D016896:Treatment Outcome
Brain-dead donor; Chronic sinusitis; Diffuse panbronchiolitis; Lung transplantation; Pulmonary hypertension