Document Detail

Maximal oxygen uptake and severity of disease in lymphangioleiomyomatosis.
MedLine Citation:
PMID:  12958050     Owner:  NLM     Status:  MEDLINE    
Lymphangioleiomyomatosis (LAM), a disease that occurs primarily in women, is characterized by cystic lung lesions causing respiratory failure, which may require lung transplantation. Lung diffusion (DLCO) and/or FEV1 are decreased, but frequently not in parallel with each other. Because cardiopulmonary exercise testing (CPET) provides information that is not obtainable from resting cardiopulmonary tests, we performed CPET in 217 LAM patients and correlated exercise data with clinical markers of severity, computed tomography scans, lung function, and histology. VO2max was decreased in 162 patients, of whom 28 did not reach anaerobic threshold; 29 had low oxygen uptake at anaerobic threshold, and 54 developed hypoxemia. Hypoxemia occurred even in patients with near normal DLCO and FEV1. VO2max decreased with an increasing score of histologic LAM severity and was correlated with computed tomography scans, the use of oxygen, and resting PaO2. DLCO and FEV1, however, were the only significant predictors of VO2max. We conclude that CPET uncovers the presence of exercise-induced hypoxemia and assists in grading the severity of disease and determining supplemental oxygen requirements in patients with LAM.
Angelo M Taveira-DaSilva; Mario P Stylianou; Carolyn J Hedin; Arnold S Kristof; Nilo A Avila; Antoinette Rabel; William D Travis; Joel Moss
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.     Date:  2003-09-04
Journal Detail:
Title:  American journal of respiratory and critical care medicine     Volume:  168     ISSN:  1073-449X     ISO Abbreviation:  Am. J. Respir. Crit. Care Med.     Publication Date:  2003 Dec 
Date Detail:
Created Date:  2003-12-11     Completed Date:  2004-02-12     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9421642     Medline TA:  Am J Respir Crit Care Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1427-31     Citation Subset:  AIM; IM    
Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892-1590, USA.
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MeSH Terms
Anoxia / etiology,  therapy
Exercise / physiology
Exercise Test / methods*
Lung / pathology,  radiography
Lymphangioleiomyomatosis / complications,  metabolism*,  physiopathology*,  radiography
Middle Aged
Oxygen Consumption / physiology*
Respiratory Function Tests
Severity of Illness Index*
Tomography, X-Ray Computed
Comment In:
Am J Respir Crit Care Med. 2003 Dec 15;168(12):1405-6   [PMID:  14668248 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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