論文

査読有り
2016年1月

Lung Transplantation for Lymphangioleiomyomatosis in Japan

PLOS ONE
  • 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
  • 全て表示

11
1
開始ページ
e0146749
終了ページ
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.1371/journal.pone.0146749
出版者・発行元
PUBLIC LIBRARY SCIENCE

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.

リンク情報
DOI
https://doi.org/10.1371/journal.pone.0146749
PubMed
https://www.ncbi.nlm.nih.gov/pubmed/26771878
Web of Science
https://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=JSTA_CEL&SrcApp=J_Gate_JST&DestLinkType=FullRecord&KeyUT=WOS:000368628300031&DestApp=WOS_CPL
ID情報
  • DOI : 10.1371/journal.pone.0146749
  • ISSN : 1932-6203
  • PubMed ID : 26771878
  • Web of Science ID : WOS:000368628300031

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