Study of the Disease Process of Lymphangioleiomyomatosis

This study is currently recruiting participants. (see Contacts and Locations)
Verified January 2014 by National Institutes of Health Clinical Center (CC)
Sponsor:
Information provided by:
National Institutes of Health Clinical Center (CC)
ClinicalTrials.gov Identifier:
NCT00001465
First received: November 3, 1999
Last updated: March 14, 2014
Last verified: January 2014

November 3, 1999
March 14, 2014
September 1995
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Complete list of historical versions of study NCT00001465 on ClinicalTrials.gov Archive Site
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Study of the Disease Process of Lymphangioleiomyomatosis
Characterization of the Pathogenesis of Lymphangioleiomyomatosis (LAM)

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.

Individuals with pulmonary lymphangioleiomyomatosis develop severe destructive lung disease. Most of them are females of childbearing age. Currently, there is no proven effective therapy and the prognosis is variable. This study is designed to (a) define the clinical course of the disease and (b) elucidate the pathogenesis of the disease at the cellular and molecular levels, in order to develop more effective therapy. To accomplish this, we intend to identify the proteins and genes that contribute to the process of lung destruction in affected individuals.

Observational
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  • Lung Disease
  • Pneumothorax
  • Tuberous Sclerosis
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*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruiting
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  • INCLUSION CRITERIA:

General admission criteria for patients include one or both of the following:

Findings on lung biopsy diagnostic of LAM;

Findings on chest x-ray and/or chest computed axial tomography consistent with LAM.

Patients with TSC and pulmonary LAM will be included in the study.

Normal non-smokers in the control group are defined as individuals who have not smoked for greater than or equal to 1 year and have no systemic or pulmonary disease.

Normal smokers defined as individuals with no systemic or pulmonary disease, who have smoked for greater than or equal to 1 year and have normal chest x-ray and normal pulmonary function tests may be included if needed as controls for a similar population of patients with LAM.

EXCLUSION CRITERIA:

Exclusion criteria for patients include:

Age less than 16.

Advanced stage of a pulmonary or a systemic illness in which the risk of the study is judged to be significant even in the absence of a clear contraindication to the procedures.

Exclusion criteria for patients for the formal exercise study and the stress echocardiogram include patients on continuous oxygen. Patients may perform an exercise test that will assess the patient's exercise capacity with activities of daily living.

Both
16 Years to 80 Years
No
Contact: Mary Haughey, R.N. (301) 496-3632 mhaughey@nhlbi.nih.gov
Contact: Joel Moss, M.D. (301) 496-1597 mossj@nhlbi.nih.gov
United States
 
NCT00001465
950186, 95-H-0186
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National Heart, Lung, and Blood Institute (NHLBI)
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Principal Investigator: Joel Moss, M.D. National Heart, Lung, and Blood Institute (NHLBI)
National Institutes of Health Clinical Center (CC)
January 2014

ICMJE     Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP