Study of the Disease Process of Lymphangioleiomyomatosis
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Purpose
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 |
|---|
|
Lung Disease Pneumothorax Tuberous Sclerosis |
| Study Type: | Observational |
| Official Title: | Characterization of the Pathogenesis of Lymphangioleiomyomatosis (LAM) |
| Estimated Enrollment: | 99999999 |
| Study Start Date: | September 1995 |
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.
Eligibility| Ages Eligible for Study: | 16 Years to 80 Years |
| Genders Eligible for Study: | Both |
| Accepts Healthy Volunteers: | No |
- 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.
Contacts and Locations| 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, 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 Patient Recruitment and Public Liaison Office (PRPL) 800-411-1222 ext TTY8664111010 prpl@mail.cc.nih.gov | |
| Principal Investigator: | Joel Moss, M.D. | National Heart, Lung, and Blood Institute (NHLBI) |
More Information
Additional Information:
Publications:
| ClinicalTrials.gov Identifier: | NCT00001465 History of Changes |
| Other Study ID Numbers: | 950186, 95-H-0186 |
| Study First Received: | November 3, 1999 |
| Last Updated: | May 1, 2013 |
| Health Authority: | United States: Federal Government |
Keywords provided by National Institutes of Health Clinical Center (CC):
|
Smooth Muscle Proliferation Bronchoscopy Female |
Pneumothorax Tuberous Sclerosis Lymphangioleiomyomatosis (LAM) |
Additional relevant MeSH terms:
|
Lung Diseases Pneumothorax Sclerosis Tuberous Sclerosis Lymphangioleiomyomatosis Respiratory Tract Diseases Pleural Diseases Pathologic Processes Hamartoma Neoplasms Malformations of Cortical Development Nervous System Malformations Nervous System Diseases Neurocutaneous Syndromes |
Heredodegenerative Disorders, Nervous System Neurodegenerative Diseases Congenital Abnormalities Genetic Diseases, Inborn Lymphangiomyoma Lymphatic Vessel Tumors Neoplasms by Histologic Type Perivascular Epithelioid Cell Neoplasms Neoplasms, Connective and Soft Tissue Lymphoproliferative Disorders Lymphatic Diseases Immunoproliferative Disorders Immune System Diseases |
ClinicalTrials.gov processed this record on June 18, 2013