PMID- 37589458
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230818
IS  - 2312-0541 (Print)
IS  - 2312-0541 (Electronic)
IS  - 2312-0541 (Linking)
VI  - 9
IP  - 4
DP  - 2023 Jul
TI  - Unexpected sirolimus-stimulated airway hyperreactivity in 
      lymphangioleiomyomatosis.
LID - 00305-2023 [pii]
LID - 10.1183/23120541.00305-2023 [doi]
AB  - Lymphangioleiomyomatosis (LAM) is a multisystem disease affecting primarily 
      women, characterised in the lung by proliferation of LAM cells, abnormal smooth 
      muscle-like cells with dysfunctional tuberous sclerosis complex genes. This 
      dysfunction results in activation of mechanistic target of rapamycin (mTOR), 
      leading to LAM cell proliferation. Sirolimus (rapamycin) is the only United 
      States Food and Drug Administration-approved treatment for pulmonary LAM, 
      resulting in decreased LAM cell growth/size and stabilised lung function [1].
CI  - Copyright (c)The authors 2023.
FAU - Steagall, Wendy K
AU  - Steagall WK
AD  - Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes 
      of Health, Bethesda, MD, USA.
FAU - Stylianou, Mario
AU  - Stylianou M
AD  - Office of Biostatistics Research, National Heart, Lung, and Blood Institute, 
      National Institutes of Health, Bethesda, MD, USA.
FAU - Pacheco-Rodriguez, Gustavo
AU  - Pacheco-Rodriguez G
AD  - Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes 
      of Health, Bethesda, MD, USA.
FAU - Yu, Zu Xi
AU  - Yu ZX
AD  - Pathology Facility, National Heart, Lung, and Blood Institute, National 
      Institutes of Health, Bethesda, MD, USA.
FAU - Moss, Joel
AU  - Moss J
AD  - Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes 
      of Health, Bethesda, MD, USA.
LA  - eng
PT  - Journal Article
DEP - 20230814
PL  - England
TA  - ERJ Open Res
JT  - ERJ open research
JID - 101671641
PMC - PMC10423980
COIS- Conflict of interest: All authors declare no conflicts of interest and no 
      competing interests.
EDAT- 2023/08/17 12:41
MHDA- 2023/08/17 12:42
PMCR- 2023/08/14
CRDT- 2023/08/17 09:03
PHST- 2023/05/11 00:00 [received]
PHST- 2023/05/20 00:00 [accepted]
PHST- 2023/08/17 12:42 [medline]
PHST- 2023/08/17 12:41 [pubmed]
PHST- 2023/08/17 09:03 [entrez]
PHST- 2023/08/14 00:00 [pmc-release]
AID - 00305-2023 [pii]
AID - 10.1183/23120541.00305-2023 [doi]
PST - epublish
SO  - ERJ Open Res. 2023 Aug 14;9(4):00305-2023. doi: 10.1183/23120541.00305-2023. 
      eCollection 2023 Jul.