Lung Transplantation for Lymphangioleiomyomatosis in Japan

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. In this national survey, we...

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Veröffentlicht in:PloS one 2016-01, Vol.11 (1), p.e0146749-e0146749
Hauptverfasser: 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
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container_start_page e0146749
container_title PloS one
container_volume 11
creator 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
description 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. In this national survey, we analyzed data from 98 LAM patients registered for lung transplantation in the Japan Organ Transplantation Network. 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
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However, the prognosis after registration and the circumstances of lung transplantation with sirolimus therapy have never been reported. In this national survey, we analyzed data from 98 LAM patients registered for lung transplantation in the Japan Organ Transplantation Network. 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&lt;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). 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.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>26771878</pmid><doi>10.1371/journal.pone.0146749</doi><oa>free_for_read</oa></addata></record>
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subjects Adult
Aging
Anti-Bacterial Agents - administration & dosage
Anti-Bacterial Agents - therapeutic use
Cancer
Care and treatment
Data processing
Disease
Female
Health aspects
Heart
Humans
Japan
Lung Neoplasms - surgery
Lung Transplantation
Lymphangioleiomyomatosis - surgery
Lymphatic diseases
Male
Medicine
Middle Aged
Organ transplantation
Patients
Pediatrics
Pulmonary arteries
Rapamycin
Retrospective Studies
Sirolimus - administration & dosage
Sirolimus - therapeutic use
Statistical analysis
Surgery
Survival
Survival Rate
Thoracic surgery
Transplantation
Transplants & implants
University graduates
Young Adult
title Lung Transplantation for Lymphangioleiomyomatosis in Japan
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