Study of the Disease Process of Lymphangioleiomyomatosis
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|ClinicalTrials.gov Identifier: NCT00001465|
Recruitment Status : Recruiting
First Posted : November 4, 1999
Last Update Posted : May 27, 2020
Pulmonary lymphangioleiomyomatosis (LAM) is a destructive lung disease typically affecting women of childbearing age. Currently, there is no effective therapy for the disease and the prognosis is poor.
This study is designed to determine the disease processes involved at the level of cells and molecules, in order to develop more effective therapy.
Researchers intend to identify the proteins and genes that contribute to the process of lung destruction in affected individuals.
|Condition or disease||Intervention/treatment||Phase|
|Lung Disease Pneumothorax Tuberous Sclerosis Lymphangioleiomyomatosis||Device: Toshibia Aquilion One CT||Not Applicable|
|Study Type :||Interventional (Clinical Trial)|
|Estimated Enrollment :||99999999 participants|
|Intervention Model:||Single Group Assignment|
|Masking:||None (Open Label)|
|Official Title:||Characterization of the Pathogenesis of Lymphangioleiomyomatosis (LAM)|
|Actual Study Start Date :||December 18, 1995|
|Estimated Primary Completion Date :||September 30, 2030|
|Estimated Study Completion Date :||September 30, 2030|
Experimental: LAM patients
The patients with tissue diagnosis of LAM may be admitted for evaluation every six months, or as deemed necessary for research.
Device: Toshibia Aquilion One CT
The Toshiba Aquilion ONE CT system is currently being used for studies in both general CT radiology and CT cardiac imaging. One of the unique aspects of the Aquilion ONE CT system is its ability to acquire whole organ volume images in a single rotation by utilizing an x-ray detector that is configured as 320 detector rows with a 0.5 mm width, providing a z-axis coverage of 16 cm of anatomy. In line with the evolutionary changes to CT systems, the Aquilion ONE will be upgraded with new technology that will expand its capabilities. The changes being made to the Aquilion ONE will provide enhancements to image acquisition capabilities, reduce ionizing radiation dose, and improve subject access to the system. All of these features assist in enhancing the safety of the currently installed Aquilion ONE CT system.
- Define the clinical course of the disease LAM and elucidate the pathogenesis of LAM at cellular and molecular levels [ Time Frame: ongoinig ]To define the molecular basis of the remarkable proliferation of immature appearing smooth muscle cells, which is the cause of many of the clinical manifestations, and perhaps thereby to improve our understanding of the mechanism of smooth muscle cell proliferation in other diseases, e.g., interstitial lung diseases, asthma, atherosclerosis, hypertension, and post-angioplastic coronary restenosis.To assess the contribution of proteins and genetic factors to cyst formation, airway obstruction, and clinical course.To evaluate the role of TSC genes in the pathogenesis of LAM.
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00001465
|Contact: Nora M Quade||(301) email@example.com|
|Contact: Joel Moss, M.D.||(301) firstname.lastname@example.org|
|United States, Maryland|
|National Institutes of Health Clinical Center, 9000 Rockville Pike||Recruiting|
|Bethesda, Maryland, United States, 20892|
|Contact: For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR) 800-411-1222 ext TTY8664111010 email@example.com|
|Principal Investigator:||Joel Moss, M.D.||National Heart, Lung, and Blood Institute (NHLBI)|