Cargando…
Lung Transplantation for Lymphangioleiomyomatosis in Japan
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 thi...
Autores principales: | , , , , , , , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2016
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4714890/ https://www.ncbi.nlm.nih.gov/pubmed/26771878 http://dx.doi.org/10.1371/journal.pone.0146749 |
_version_ | 1782410384199647232 |
---|---|
author | Ando, Katsutoshi Okada, Yoshinori Akiba, Miki Kondo, Takashi Kawamura, Tomohiro Okumura, Meinoshin Chen, Fengshi Date, Hiroshi Shiraishi, Takeshi Iwasaki, Akinori Yamasaki, Naoya Nagayasu, Takeshi Chida, Masayuki Inoue, Yoshikazu Hirai, Toyohiro Seyama, Kuniaki Mishima, Michiaki |
author_facet | Ando, Katsutoshi Okada, Yoshinori Akiba, Miki Kondo, Takashi Kawamura, Tomohiro Okumura, Meinoshin Chen, Fengshi Date, Hiroshi Shiraishi, Takeshi Iwasaki, Akinori Yamasaki, Naoya Nagayasu, Takeshi Chida, Masayuki Inoue, Yoshikazu Hirai, Toyohiro Seyama, Kuniaki Mishima, Michiaki |
author_sort | Ando, Katsutoshi |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-4714890 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Public Library of Science |
record_format | MEDLINE/PubMed |
spelling | pubmed-47148902016-01-30 Lung Transplantation for Lymphangioleiomyomatosis in Japan Ando, Katsutoshi Okada, Yoshinori Akiba, Miki Kondo, Takashi Kawamura, Tomohiro Okumura, Meinoshin Chen, Fengshi Date, Hiroshi Shiraishi, Takeshi Iwasaki, Akinori Yamasaki, Naoya Nagayasu, Takeshi Chida, Masayuki Inoue, Yoshikazu Hirai, Toyohiro Seyama, Kuniaki Mishima, Michiaki PLoS One Research Article 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. Public Library of Science 2016-01-15 /pmc/articles/PMC4714890/ /pubmed/26771878 http://dx.doi.org/10.1371/journal.pone.0146749 Text en © 2016 Ando et al http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Research Article Ando, Katsutoshi Okada, Yoshinori Akiba, Miki Kondo, Takashi Kawamura, Tomohiro Okumura, Meinoshin Chen, Fengshi Date, Hiroshi Shiraishi, Takeshi Iwasaki, Akinori Yamasaki, Naoya Nagayasu, Takeshi Chida, Masayuki Inoue, Yoshikazu Hirai, Toyohiro Seyama, Kuniaki Mishima, Michiaki Lung Transplantation for Lymphangioleiomyomatosis in Japan |
title | Lung Transplantation for Lymphangioleiomyomatosis in Japan |
title_full | Lung Transplantation for Lymphangioleiomyomatosis in Japan |
title_fullStr | Lung Transplantation for Lymphangioleiomyomatosis in Japan |
title_full_unstemmed | Lung Transplantation for Lymphangioleiomyomatosis in Japan |
title_short | Lung Transplantation for Lymphangioleiomyomatosis in Japan |
title_sort | lung transplantation for lymphangioleiomyomatosis in japan |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4714890/ https://www.ncbi.nlm.nih.gov/pubmed/26771878 http://dx.doi.org/10.1371/journal.pone.0146749 |
work_keys_str_mv | AT andokatsutoshi lungtransplantationforlymphangioleiomyomatosisinjapan AT okadayoshinori lungtransplantationforlymphangioleiomyomatosisinjapan AT akibamiki lungtransplantationforlymphangioleiomyomatosisinjapan AT kondotakashi lungtransplantationforlymphangioleiomyomatosisinjapan AT kawamuratomohiro lungtransplantationforlymphangioleiomyomatosisinjapan AT okumurameinoshin lungtransplantationforlymphangioleiomyomatosisinjapan AT chenfengshi lungtransplantationforlymphangioleiomyomatosisinjapan AT datehiroshi lungtransplantationforlymphangioleiomyomatosisinjapan AT shiraishitakeshi lungtransplantationforlymphangioleiomyomatosisinjapan AT iwasakiakinori lungtransplantationforlymphangioleiomyomatosisinjapan AT yamasakinaoya lungtransplantationforlymphangioleiomyomatosisinjapan AT nagayasutakeshi lungtransplantationforlymphangioleiomyomatosisinjapan AT chidamasayuki lungtransplantationforlymphangioleiomyomatosisinjapan AT inoueyoshikazu lungtransplantationforlymphangioleiomyomatosisinjapan AT hiraitoyohiro lungtransplantationforlymphangioleiomyomatosisinjapan AT seyamakuniaki lungtransplantationforlymphangioleiomyomatosisinjapan AT mishimamichiaki lungtransplantationforlymphangioleiomyomatosisinjapan AT lungtransplantationforlymphangioleiomyomatosisinjapan |