Yanagiya Masahiro, Sato Masaaki, Kawashima Shun, Kuwano Hideki, Nagayama Kazuhiro, Nitadori Jun-Ichi, Anraku Masaki, Nakajima Jun
Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.
Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.
Ann Thorac Surg. 2016 Oct;102(4):e347-9. doi: 10.1016/j.athoracsur.2016.02.092.
A patient with pleuroparenchymal fibroelastosis (PPFE) was successfully treated with living-donor lobar lung transplantation. A 27-year-old woman with a 3-month history of dyspnea received a diagnosis of PPFE. Her chest wall was extremely flattened over time, and her respiratory condition progressively deteriorated. She underwent semielective bilateral living-donor lobar lung transplantation. Her chest wall rigidity, which was secondary to PPFE, required intensive pulmonary rehabilitation postoperatively. By 6 months after transplantation, the flattening of her chest wall was reversed. Living-donor lobar lung transplantation was a life-saving procedure for this patient and improved the chest wall deformity of PPFE.
一名胸膜实质纤维弹性组织增生症（PPFE）患者通过活体供体肺叶移植获得成功治疗。一名有3个月呼吸困难病史的27岁女性被诊断为PPFE。随着时间推移，她的胸壁极度扁平，呼吸状况逐渐恶化。她接受了半择期双侧活体供体肺叶移植。她因PPFE继发的胸壁僵硬在术后需要强化肺部康复治疗。移植后6个月，她胸壁的扁平状态得到了逆转。活体供体肺叶移植对该患者来说是一种挽救生命的手术，并改善了PPFE的胸壁畸形。
