Back to resultsOfficial Record of Patients Diagnosed With Lymphangioleiomyomatosis (LAM)
Primary Purpose
Leiomyomatosis
Status
Completed
Phase
Locations
United States
Study Type
Observational
Intervention
Sponsored by
About this trial
This is an observational trial for Leiomyomatosis focused on measuring Progesterone, Pulmonary Function Tests, Pneumothorax, Oopherectomy, High Resolution CT, Tuberous Sclerosis Complex, Lymphangioleiomyomatosis (LAM)
Eligibility Criteria
Informed consent must be obtained from the patient. Patients must be female. Patients must be age 18 or older. Patients with prevalent and incident cases are eligible. Patients with the presence or absence of underlying diagnosis or evidence of Tuberous Sclerosis Complex (TSC) are eligible. Patients must have a diagnosis of LAM confirmed by any of the following criteria: Lung biopsy (transbronchial, surgical, transthoracic) judged to be diagnostic by the Tissue Core pathologists; OR Biopsy of lymph node or other mass judged to be diagnostic by the Tissue Core pathologists; OR High resolution CT scan of the chest which is judged to be diagnostic of LAM with a high degree of certainty by all three of the expert radiologists making up the Imaging Core. Patients may be enrolled in other protocols.
Sites / Locations
- National Heart, Lung and Blood Institute (NHLBI)
Outcomes
Primary Outcome Measures
Secondary Outcome Measures
Full Information
NCT ID
NCT00001869
First Posted
November 3, 1999
Last Updated
March 3, 2008
Sponsor
National Heart, Lung, and Blood Institute (NHLBI)
1. Study Identification
Unique Protocol Identification Number
NCT00001869
Brief Title
Official Record of Patients Diagnosed With Lymphangioleiomyomatosis (LAM)
Official Title
Lymphangioleiomyomatosis (LAM) Registry
Study Type
Observational
2. Study Status
Record Verification Date
April 2003
Overall Recruitment Status
Completed
Study Start Date
December 1998 (undefined)
Primary Completion Date
undefined (undefined)
Study Completion Date
April 2003 (undefined)
3. Sponsor/Collaborators
Name of the Sponsor
National Heart, Lung, and Blood Institute (NHLBI)
4. Oversight
5. Study Description
Brief Summary
Pulmonary lymphoangioleiomyomatosis (LAM) is a rare destructive lung disease typically affecting women of childbearing age. Currently, there is no effective therapy for the disease and the prognosis is poor.
In order to better study this disease, the National Heart, Lung, and Blood Institute (NHLBI) has developed a registry to keep an official record of patients diagnosed with LAM. This research project will collect data from 6 health care centers as well as outside physicians. Researchers hope to provide valuable information about the rate of lung destruction and quality of life in patients with LAM.
Patients participating in this study will be followed for 5 years. Tissue collected from these patients may contribute to the development of future studies on the disease processes of LAM.
Detailed Description
LAM is a rare disease that predominantly affects women of child-bearing age. To study in more detail this rare disease, this multi-center project will establish a registry of persons with LAM. By combining data from 6 centers and outside physicians, this study may yield valuable information regarding the rate of decline in pulmonary function and quality of life in individuals with LAM. These patients will be followed over a five-year period. Tissue collected from study participants may facilitate future studies into the molecular basis of LAM.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Leiomyomatosis
Keywords
Progesterone, Pulmonary Function Tests, Pneumothorax, Oopherectomy, High Resolution CT, Tuberous Sclerosis Complex, Lymphangioleiomyomatosis (LAM)
7. Study Design
Enrollment
400 (false)
10. Eligibility
Sex
All
Accepts Healthy Volunteers
No
Eligibility Criteria
Informed consent must be obtained from the patient.
Patients must be female.
Patients must be age 18 or older.
Patients with prevalent and incident cases are eligible.
Patients with the presence or absence of underlying diagnosis or evidence of Tuberous Sclerosis Complex (TSC) are eligible.
Patients must have a diagnosis of LAM confirmed by any of the following criteria:
Lung biopsy (transbronchial, surgical, transthoracic) judged to be diagnostic by the Tissue Core pathologists;
OR
Biopsy of lymph node or other mass judged to be diagnostic by the Tissue Core pathologists;
OR
High resolution CT scan of the chest which is judged to be diagnostic of LAM with a high degree of certainty by all three of the expert radiologists making up the Imaging Core.
Patients may be enrolled in other protocols.
Facility Information:
Facility Name
National Heart, Lung and Blood Institute (NHLBI)
City
Bethesda
State/Province
Maryland
ZIP/Postal Code
20892
Country
United States
12. IPD Sharing Statement
Citations:
PubMed Identifier
8024411
Citation
Abdulla M, Bui HX, del Rosario AD, Wolf BC, Ross JS. Renal angiomyolipoma. DNA content and immunohistochemical study of classic and multicentric variants. Arch Pathol Lab Med. 1994 Jul;118(7):735-9.
Results Reference
background
PubMed Identifier
2367651
Citation
Aberle DR, Hansell DM, Brown K, Tashkin DP. Lymphangiomyomatosis: CT, chest radiographic, and functional correlations. Radiology. 1990 Aug;176(2):381-7. doi: 10.1148/radiology.176.2.2367651.
Results Reference
background
PubMed Identifier
1000470
Citation
Basset F, Soler P, Marsac J, Corrin B. Pulmonary lymphangiomyomatosis: three new cases studied with electron microscopy. Cancer. 1976 Dec;38(6):2357-66. doi: 10.1002/1097-0142(197612)38:63.0.co;2-a.
Results Reference
background
Learn more about this trial
Official Record of Patients Diagnosed With Lymphangioleiomyomatosis (LAM)
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