PMID- 27514586
OWN - NLM
STAT- MEDLINE
DCOM- 20170214
LR  - 20181202
IS  - 1557-8216 (Electronic)
IS  - 0272-5231 (Linking)
VI  - 37
IP  - 3
DP  - 2016 Sep
TI  - Lymphangioleiomyomatosis.
PG  - 389-403
LID - S0272-5231(16)30039-9 [pii]
LID - 10.1016/j.ccm.2016.04.002 [doi]
AB  - Lymphangioleiomyomatosis is a rare multisystem disease predominantly affecting 
      women that can occur sporadically or in association with tuberous sclerosis. Lung 
      cysts progressively replace the lung parenchyma, which leads to dyspnea, 
      recurrent pneumothorax, and in some cases respiratory failure. Patients may also 
      have lymphatic disease in the thorax, abdomen, and pelvis, and renal 
      angiomyolipomas. Treatment includes supportive care, bronchodilators, and for 
      those with progressive disease, mammalian target of rapamycin (mTOR) inhibitors.
CI  - Copyright (c) 2016 Elsevier Inc. All rights reserved.
FAU - Johnson, Simon R
AU  - Johnson SR
AD  - Division of Respiratory Medicine, National Centre for Lymphangioleiomyomatosis, 
      University Hospitals NHS Trust, University of Nottingham, Nottingham, NG7 2UH, 
      UK. Electronic address: simon.johnson@nottingham.ac.uk.
FAU - Taveira-DaSilva, Angelo M
AU  - Taveira-DaSilva AM
AD  - Cardiovascular and Pulmonary Branch, National Institutes of Health, National 
      Heart Lung and Blood Institute, Building 10, Room 6D05, Bethesda, MD 20814, USA.
FAU - Moss, Joel
AU  - Moss J
AD  - Cardiovascular and Pulmonary Branch, National Institutes of Health, National 
      Heart Lung and Blood Institute, Building 10, Room 6D03, Bethesda, MD 20814, USA.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Clin Chest Med
JT  - Clinics in chest medicine
JID - 7907612
RN  - 0 (Antibiotics, Antineoplastic)
RN  - 0 (Bronchodilator Agents)
RN  - 9HW64Q8G6G (Everolimus)
RN  - W36ZG6FT64 (Sirolimus)
SB  - IM
MH  - Angiomyolipoma/complications/*diagnostic imaging/pathology/therapy
MH  - Antibiotics, Antineoplastic/therapeutic use
MH  - Bronchodilator Agents/therapeutic use
MH  - Everolimus/therapeutic use
MH  - Humans
MH  - Kidney Neoplasms/complications/*diagnostic imaging/pathology/therapy
MH  - Lung Neoplasms/complications/*diagnostic imaging/pathology/therapy
MH  - Lymphangioleiomyomatosis/complications/*diagnostic imaging/pathology/therapy
MH  - Pleurodesis
MH  - Pneumothorax/etiology/therapy
MH  - Sirolimus/therapeutic use
MH  - Tomography, X-Ray Computed
MH  - Tuberous Sclerosis/complications/*diagnostic imaging/pathology
MH  - Ultrasonography
OTO - NOTNLM
OT  - Angiomyolipoma
OT  - Cystic lung disease
OT  - Lymphatic disease
OT  - Pneumothorax
OT  - mTOR inhibitor
EDAT- 2016/08/16 06:00
MHDA- 2017/02/15 06:00
CRDT- 2016/08/13 06:00
PHST- 2016/08/13 06:00 [entrez]
PHST- 2016/08/16 06:00 [pubmed]
PHST- 2017/02/15 06:00 [medline]
AID - S0272-5231(16)30039-9 [pii]
AID - 10.1016/j.ccm.2016.04.002 [doi]
PST - ppublish
SO  - Clin Chest Med. 2016 Sep;37(3):389-403. doi: 10.1016/j.ccm.2016.04.002.