Protocol Details

Characterization of the Pathogenesis of Lymphangioleiomyomatosis (LAM)

This study is currently recruiting participants.

Summary | Eligibility | Citations | Contacts

Summary

Number	95-H-0186
Sponsoring Institute	National Heart, Lung and Blood Institute (NHLBI)
Recruitment Detail	Type: Participants currently recruited/enrolled Gender: Male & Female Min Age: 16 Max Age: 80
Referral Letter Required	Yes
Population Exclusion(s)	None
Special Instructions	You will be asked to return to NIH every 6 months. This protocol includes a bronchoscopy that you may or may not elect to have performed. This has no impact on your continued participation in the study.
Keywords	Smooth Muscle Proliferation; Bronchoscopy; Female; Pneumothorax; Tuberous Sclerosis
Recruitment Keyword(s)	Lymphangioleiomyomatosis (LAM)
Condition(s)	Lung Disease; Pneumothorax; Tuberous Sclerosis
Investigational Drug(s)	None
Investigational Device(s)	None
Intervention(s)	None
Supporting Site	National Heart, Lung, and Blood Institute

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.

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Eligibility

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.

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Citations:

Crooks DM, Pacheco-Rodriguez G, DeCastro RM, McCoy JP Jr, Wang JA, Kumaki F, Darling T, Moss J. Molecular and genetic analysis of disseminated neoplastic cells in lymphangioleiomyomatosis. Proc Natl Acad Sci U S A. 2004 Dec 14;101(50):17462-7. Epub 2004 Dec 6.

Pacheco-Rodriguez G, Kristof AS, Stevens LA, Zhang Y, Crooks D, Moss J. Giles F. Filley Lecture. Genetics and gene expression in lymphangioleiomyomatosis. Chest. 2002 Mar;121(3 Suppl):56S-60S.

Taveira-DaSilva AM, Stylianou MP, Hedin CJ, Kristof AS, Avila NA, Rabel A,Travis WD, Moss J. Maximal oxygen uptake and severity of disease in lymphangioleiomyomatosis. Am J Respir Crit Care Med. 2003 Dec 15;168(12):1427-31. Epub 2003 Sep 4.

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Contacts:

Principal Investigator	Referral Contact	For more information:
Joel Moss, M.D. National Heart, Lung and Blood Institute (NHLBI) National Institutes of Health Building 10 Room 6D05 10 Center Drive Bethesda, Maryland 20892 (301) 496-3632 mossj@nhlbi.nih.gov	Mary Haughey, R.N. National Heart, Lung and Blood Institute (NHLBI) National Institutes of Health Building 10 Room 6D03 10 Center Drive Bethesda, Maryland 20892 (301) 496-3632 mhaughey@nhlbi.nih.gov	Patient Recruitment and Public Liaison Office Building 61 10 Cloister Court Bethesda, Maryland 20892-4754 Toll Free: 1-800-411-1222 TTY: 301-594-9774 (local),1-866-411-1010 (toll free) Fax: 301-480-9793 prpl@mail.cc.nih.gov

Clinical Trials Number:

NCT00001465

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