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    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 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. 10.1371/journal.pone.0146749
    Experience of Lung Transplantation in Patients with Lymphangioleiomyomatosis at a Brazilian Reference Centre. Baldi Bruno Guedes,Samano Marcos Naoyuki,Campos Silvia Vidal,de Oliveira Martina Rodrigues,Junior José Eduardo Afonso,Carraro Rafael Medeiros,Teixeira Ricardo Henrique Oliveira Braga,Minguini Isabela Pasqualini,Burlina Roni,Pato Eduardo Zinoni Silva,Carvalho Carlos Roberto Ribeiro,Costa André Nathan Lung INTRODUCTION:Lung transplantation (LT) is the standard of care for patients with advanced lung diseases, including lymphangioleiomyomatosis (LAM). LAM accounts for only 1% of all LTs performed in the international registry. As a result, the global experience, including the use of mechanistic target of rapamycin (mTOR) inhibitors before and after LT in LAM, is still limited. METHODS:We conducted a retrospective review of all LAM patients who underwent LT at our centre between 2003 and 2016. Pre- and post-transplant data were assessed. RESULTS:Eleven women with LAM underwent LT, representing 3.3% of all procedures. Ten (91%) patients underwent double-LT. The mean age at diagnosis was 39 ± 6 years and the mean FEV before LT was 28 ± 14%. Only one patient underwent pleurodesis for recurrent pneumothorax. Pulmonary hypertension was confirmed in 3 (27%) patients. Four (36%) patients received sirolimus preoperatively; three of them received it until the day of LT, and there was no occurrence of bronchial anastomotic dehiscence after the procedure. Four patients (36%) received mTOR inhibitors post-transplant. The median follow-up from LT was 44 months. There were 3 deaths (27%) during the study and survival probabilities at 1, 3, and 5 years after LT were, 90, 90, and 77%, respectively. CONCLUSIONS:This data reinforces the role of LT for LAM patients with end-stage disease. The use of sirolimus seems to be safe before LT and the occurrence of complications after LT, including those LAM-related, should be continuously monitored. 10.1007/s00408-017-0045-y
    Single lung transplantation for lymphangioleiomyomatosis: a single-center experience in Japan. Oishi Hisashi,Watanabe Tatsuaki,Matsuda Yasushi,Noda Masafumi,Ejima Yutaka,Saiki Yoshikatsu,Seyama Kuniaki,Kondo Takashi,Okada Yoshinori Surgery today PURPOSE:Lung transplantation is accepted as an effective modality for patients with end-stage pulmonary lymphangioleiomyomatosis (LAM). Generally, bilateral lung transplantation is preferred to single lung transplantation (SLT) for LAM because of native lung-related complications, such as pneumothorax and chylothorax. It remains controversial whether SLT is a suitable surgical option for LAM. The objective of this study was to evaluate the morbidity, mortality and outcome after SLT for LAM in a lung transplant center in Japan. METHODS:We reviewed the records of 29 patients who underwent SLT for LAM in our hospital between March, 2000 and November, 2017. The data collected included the pre-transplant demographics of recipients, surgical characteristics, complications, morbidity, mortality and survival after SLT for LAM. RESULTS:The most common complication after SLT for LAM was contralateral pneumothorax (n = 7; 24.1%). Six of these recipients were treated successfully with chest-tube placement and none required surgery for the pneumothorax. The second-most common complication was chylous pleural effusion (n = 6; 20.7%) and these recipients were all successfully treated by pleurodesis. The 5-year survival rate after SLT for LAM was 79.5%. CONCLUSION:LAM-related complications after SLT for this disease can be managed. SLT is a treatment option and may improve access to lung transplantation for patients with end-stage LAM. 10.1007/s00595-018-1678-z
    Long-Term Results of Bilateral Lung Transplantation in Patients With End-Stage Pulmonary Lymphangioleiomyomatosis. Salman Jawad,Ius Fabio,Sommer Wiebke,Siemeni Thierry,Fleissner Felix,Alhadidi Hani,Kugler Christiane,Avsar Murat,Haverich Axel,Warnecke Gregor,Tudorache Igor,Kuhn Christian Progress in transplantation (Aliso Viejo, Calif.) INTRODUCTION:Lymphangioleiomyomatosis (LAM) is a rare disease in women, leading to progressive deterioration of lung function and respiratory failure. We describe the outcome of patients with end-stage LAM who underwent lung transplantation at our center. MATERIALS AND METHODS:The records of patients with LAM transplanted at our institution between February 1997 and May 2015 were reviewed retrospectively. Morbidity and mortality were analyzed, and actuarial survival was calculated using Kaplan-Meier methods. The cumulative survival of transplant patients with LAM at our center was compared with survival after transplantation due to different diseases at our center and the results of the International Society for Heart and Lung Transplantation. Quality of life was assessed by a patient self-report at the end of the first postoperative year. RESULTS:During the study period, 25 patients underwent lung transplantation for LAM. All patients were women with a mean age of 50 (9) years. Thirteen patients (52%) had undergone previous thoracotomy. All patients (100%) received bilateral lung transplantation. One (4%) case of in-hospital mortality occurred and 9 (36%) late deaths. Two (8%) cases of late death were due to chronic lung allograft dysfunction. The 1-, 3-, and 5-year survival rates were 92%, 84%, and 76%, respectively. Quality-of-life ratings were above the normal in all eight 36-Item Short Form Health Survey subscales 1 year after transplantation. CONCLUSIONS:Lung transplantation offers a valuable therapy for patients with end-stage pulmonary LAM. 10.1177/1526924819835815
    Clinical outcomes and survival following lung transplantation in patients with lymphangioleiomyomatosis. Khawar Muhammad Umair,Yazdani Dina,Zhu Zheng,Jandarov Roman,Dilling Daniel F,Gupta Nishant The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation BACKGROUND:The primary aim of our study was to derive disease-specific outcomes following lung transplantation (LT) in patients with lymphangioleiomyomatosis (LAM). METHODS:We queried the Organ Procurement and Transplant Network database to identify LAM patients that have undergone LT in the United States. The overall survival was analyzed with Kaplan-Meier curves. Survival estimates between subgroups of interest were compared using the log-rank method. Cox proportional hazard models were employed to determine the pre-transplant variables that impact post-LT survival. RESULTS:One hundred and thirty-eight women with LAM underwent LT at 31 centers between January 2003 and June 2017. The median age at listing and transplant was 44 (IQR: 36-51) and 45 (IQR: 38-52) years, respectively. The median time spent on the LT waitlist was 257 (IQR: 85-616) days. The majority of the patients (109/134, 81%) received bilateral sequential LT. The median ischemic time was 4.9 (IQR: 4.1-6.1) hours. The actuarial Kaplan-Meier survival following LT for LAM patients at 1-, 5-, and 10 years was 94%, 73% and 56%, respectively. The post-LT survival was significantly better in LAM than in other lung diseases (10-year survival 56% vs. 32%, p < 0.01), and this advantage persisted after age- and gender-matched analysis (10-year survival 54% vs. 37%, p < 0.01). Pre-transplant parameters, such as the presence of pulmonary hypertension, six-minute walk distance, age at transplant, ischemic time during transplant, or type of transplant (single vs bilateral sequential LT), did not affect post-transplant survival. CONCLUSIONS:The median survival after LT in LAM is 12 years and is substantially better than in other lung diseases. 10.1016/j.healun.2019.06.015
    Favorable survival even with high disease-specific complication rates in lymphangioleiomyomatosis after lung transplantation-long-term follow-up of a Japanese center. Kurosaki Takeshi,Otani Shinji,Miyoshi Kentaroh,Okazaki Mikio,Sugimoto Seiichiro,Suno Manabu,Yamane Masaomi,Kobayashi Motomu,Oto Takahiro,Toyooka Shinichi The clinical respiratory journal BACKGROUND:Lung transplantation (LT) is a reliable therapeutic option for end-stage pulmonary lymphangioleiomyomatosis (LAM). Long-term outcome of LAM recipients after LT remains unknown. The aim of this study was to describe the outcomes of LT for LAM with a long-term follow-up, comparing those for other diseases in the same period. METHODS:We retrospectively reviewed consecutive 145 LT recipients between 1998 and 2015 at Okayama University Hospital with minimum 3-year follow-up. RESULTS:Twelve LAM recipients including nine sporadic-LAM and three tuberous sclerosis complex -LAM were identified. Nine of 12 underwent bilateral LT including four living-donor lobar LT. There was no significant difference in overall survival between the two groups. (P = 0.15). Chronic lung allograft dysfunction free survival rate in LAM compared with other diseases tended to be better (P = 0.058). However, the rate of requiring hemodialysis was significantly higher in LAM recipients than in the recipients of other diseases (P = 0.047). Notably, 8 of 12 (67%) LAM patients encountered LAM-related complication including chylothorax and pneumothorax, seven (58%) had proliferative diseases consisting of renal angiomyolipoma and recurrent LAM. Nine patients required mTOR inhibitors for LAM-related problems, contributing to improved control of LAM-related problems. While all nine recipients of bilateral LT have still survived, two patients died of diseases in their native lungs and one required re-LT among three recipients of single LT. CONCLUSION:Although the rates of LAM-related complications were unexpectedly high in the long term, LT is a feasible therapeutic option for patients with advanced pulmonary LAM. 10.1111/crj.13108