PMID- 27011924
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20160325
LR  - 20201001
IS  - 2220-3230 (Print)
IS  - 2220-3230 (Electronic)
IS  - 2220-3230 (Linking)
VI  - 6
IP  - 1
DP  - 2016 Mar 24
TI  - Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung 
      transplantation.
PG  - 249-54
LID - 10.5500/wjt.v6.i1.249 [doi]
AB  - Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive lethal lung disease 
      primary afflicting young women. LAM is characterized by proliferation of abnormal 
      smooth muscle cells that target the lungs, causing cystic destruction and 
      eventual respiratory failure leading to death. Recent ten year mortality due to 
      end stage LAM has been reported to be approximately 10%-20%, but may vary. The 
      decline in lung function in LAM is gradual, occurring at a rate of about 3% to 
      15% per year but can vary from patient to patient. But recently therapy with 
      mammalian target of rapamycin (mTOR) inhibitors such as sirolimus has shown 
      promising results in the stabilization of lung function and reduction of chylous 
      effusions in LAM. Lung transplantation is a viable option for patients who 
      continue to have decline in lung function despite mTOR therapy. Unique issues 
      that may occur post-transplant in a recipient with LAM include development of 
      chylous effusion and a risk of recurrence. We describe a case of LAM recurrence 
      in a bilateral lung transplant recipient who developed histological findings of 
      LAM nine years after transplantation.
FAU - Zaki, Khawaja S
AU  - Zaki KS
AD  - Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev, 
      Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, United States.
FAU - Aryan, Zahra
AU  - Aryan Z
AD  - Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev, 
      Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, United States.
FAU - Mehta, Atul C
AU  - Mehta AC
AD  - Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev, 
      Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, United States.
FAU - Akindipe, Olufemi
AU  - Akindipe O
AD  - Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev, 
      Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, United States.
FAU - Budev, Marie
AU  - Budev M
AD  - Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev, 
      Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, United States.
LA  - eng
PT  - Case Reports
PL  - United States
TA  - World J Transplant
JT  - World journal of transplantation
JID - 101608356
PMC - PMC4801802
OTO - NOTNLM
OT  - Lung rejection
OT  - Lung transplantation
OT  - Lymphangioleiomyomatosis
OT  - Mammalian target of rapamycin inhibitors
OT  - Sirolimus
EDAT- 2016/03/25 06:00
MHDA- 2016/03/25 06:01
PMCR- 2016/03/24
CRDT- 2016/03/25 06:00
PHST- 2015/05/09 00:00 [received]
PHST- 2015/12/01 00:00 [revised]
PHST- 2015/12/13 00:00 [accepted]
PHST- 2016/03/25 06:00 [entrez]
PHST- 2016/03/25 06:00 [pubmed]
PHST- 2016/03/25 06:01 [medline]
PHST- 2016/03/24 00:00 [pmc-release]
AID - 10.5500/wjt.v6.i1.249 [doi]
PST - ppublish
SO  - World J Transplant. 2016 Mar 24;6(1):249-54. doi: 10.5500/wjt.v6.i1.249.