| Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. | |
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MedLine Citation:
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PMID: 11520735 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The true prevalence of pulmonary lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex (TSC) is unknown. The prevalence of LAM, radiological features, and lung function in patients with TSC was measured. The presence of LAM, as defined by the presence of cysts by high-resolution chest computed tomography (HRCT) scan, was determined in patients with TSC without prior pulmonary disease (Group 1). To determine the significance of early detection, severity of disease in screened patients (Group 1) was compared with that in patients with TSC with a prior diagnosis of LAM (Group 2). Forty-eight patients with TSC and no prior history of LAM were screened. Of the 38 females, 13 (34%) had LAM; LAM was absent in males. Lung function was preserved in patients with TSC who were found to have LAM by screening. In patients previously known to have LAM, FEV(1) and DL(CO) correlated inversely with severity of disease as assessed by CT scan. The prevalence of LAM in women with TSC was 34%, approximately 10-fold that previously reported, consistent with a large hitherto unrecognized subclinical population of patients at risk for pulmonary complications. |
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Authors:
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J Moss; N A Avila; P M Barnes; R A Litzenberger; J Bechtle; P G Brooks; C J Hedin; S Hunsberger; A S Kristof |
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Publication Detail:
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Type: Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: American journal of respiratory and critical care medicine Volume: 164 ISSN: 1073-449X ISO Abbreviation: Am. J. Respir. Crit. Care Med. Publication Date: 2001 Aug |
Date Detail:
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Created Date: 2001-08-24 Completed Date: 2001-10-11 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 9421642 Medline TA: Am J Respir Crit Care Med Country: United States |
Other Details:
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Languages: eng Pagination: 669-71 Citation Subset: AIM; IM |
Affiliation:
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Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, Diagnostic Radiology Department, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland 20892-1590, USA. mossj@nhlbi.nih.gov |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Forced Expiratory Volume Humans Lung Neoplasms / epidemiology*, genetics*, physiopathology, radiography Lymphangioleiomyomatosis / epidemiology*, genetics*, physiopathology, radiography Mass Screening Population Surveillance Prevalence Prospective Studies Registries Respiratory Function Tests Severity of Illness Index Smoking / adverse effects Tomography, X-Ray Computed Tuberous Sclerosis / complications* United States / epidemiology |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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