Lung transplantation for lymphangioleiomyomatosis in Japan

Katsutoshi Ando, Yoshinori Okada, Miki Akiba, Takashi Kondo, Tomohiro Kawamura, Meinoshin Okumura, Fengshi Chen, Hiroshi Date, Takeshi Shiraishi, Akinori Iwasaki, Naoya Yamasaki, Takeshi Nagayasu, Masayuki Chida, Yoshikazu Inoue, Toyohiro Hirai, Kuniaki Seyama, Michiaki Mishima, Masaharu Nishimura, Koh Nakata, Koichiro TatsumiTsuneto Akashiba, Tomoko Betsuyaku, Takahide Nagase, Jun Tamaoki, Keishi Kubo, Hiroyuki Taniguchi, Kazuo Chin, Yoshihiro Urade, Norifumi Nakanishi, Yasutaka Nakano, Hiroshi Kimura, Akihito Yokoyama, Tomoaki Hoshino, Kentaro Watanabe

研究成果: Article査読

28 被引用数 (Scopus)

抄録

Background: Lung transplantation has been established as the definitive treatment option for patients with advanced lymphangioleiomyomatosis (LAM). However, the prognosis after registration and the circumstances of lung transplantation with sirolimus therapy have never been reported. Methods: In this national survey, we analyzed data from 98 LAM patients registered for lung transplantation in the Japan Organ Transplantation Network. Results: Transplantation was performed in 57 patients as of March 2014. Survival rate was 86.7% at 1 year, 82.5% at 3 years, 73.7% at 5 years, and 73.7% at 10 years. Of the 98 patients, 21 had an inactive status and received sirolimus more frequently than those with an active history (67% vs. 5%, p<0.001). Nine of twelve patients who remained inactive as of March 2014 initiated sirolimus before or while on a waiting list, and remained on sirolimus thereafter. Although the statistical analysis showed no statistically significant difference, the survival rate after registration tended to be better for lung transplant recipients than for those who awaited transplantation (p = 0.053). Conclusions: Lung transplantation is a satisfactory therapeutic option for advanced LAM, but the circumstances for pre-transplantation LAM patients are likely to alter with the use of sirolimus.

本文言語English
論文番号e0146749
ジャーナルPloS one
11
1
DOI
出版ステータスPublished - 2016 1月 1

ASJC Scopus subject areas

  • 生化学、遺伝学、分子生物学一般
  • 農業および生物科学一般
  • 一般

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