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Study of the Disease Process of Lymphangioleiomyomatosis
This study is currently recruiting participants.
Verified by National Institutes of Health Clinical Center (CC), June 2008
Sponsored by: National Heart, Lung, and Blood Institute (NHLBI)
Information provided by: National Institutes of Health Clinical Center (CC)
ClinicalTrials.gov Identifier: NCT00001465
  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

Genetics Home Reference related topics: familial encephalopathy with neuroserpin inclusion bodies tuberous sclerosis
MedlinePlus related topics: Tuberous Sclerosis
U.S. FDA Resources
Study Type: Observational
Official Title: Characterization of the Pathogenesis of Lymphangioleiomyomatosis (LAM)

Further study details as provided by National Institutes of Health Clinical Center (CC):

Estimated Enrollment: 999
Study Start Date: September 1995
Detailed Description:

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:   18 Years to 80 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Criteria
  • 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 18.

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
Please refer to this study by its ClinicalTrials.gov identifier: NCT00001465

Contacts
Contact: Patient Recruitment and Public Liaison Office (800) 411-1222 prpl@mail.cc.nih.gov
Contact: TTY 1-866-411-1010

Locations
United States, Maryland
National Institutes of Health Clinical Center, 9000 Rockville Pike Recruiting
Bethesda, Maryland, United States, 20892
Sponsors and Collaborators
  More Information

NIH Clinical Center Detailed Web Page  This link exits the ClinicalTrials.gov site

Publications:
Study ID Numbers: 950186, 95-H-0186
Study First Received: November 3, 1999
Last Updated: August 1, 2008
ClinicalTrials.gov Identifier: NCT00001465  
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)

Study placed in the following topic categories:
Immunoproliferative Disorders
Nervous System Malformations
Pleural Diseases
Lymphangiomyoma
Sclerosis
Neurodegenerative Diseases
Bourneville syndrome
Lymphatic Diseases
Lymphangioleiomyomatosis
Tuberous Sclerosis
Heredodegenerative Disorders, Nervous System
Genetic Diseases, Inborn
Respiratory Tract Diseases
Tuberous sclerosis
Lung Diseases
Lymphoproliferative Disorders
Malformations of Cortical Development
Congenital Abnormalities
Pneumothorax
Neurocutaneous Syndromes

Additional relevant MeSH terms:
Lymphatic Vessel Tumors
Neoplasms
Neoplasms by Histologic Type
Pathologic Processes
Immune System Diseases
Nervous System Diseases
Hamartoma

ClinicalTrials.gov processed this record on January 15, 2009