Official Record of Patients Diagnosed With Lymphangioleiomyomatosis (LAM)

Lymphangioleiomyomatosis (LAM) Registry

Status

Completed

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT00001869

Enrollment

400

Registered

1999-11-04

Start date

1998-12-31

Completion date

2003-04-30

Last updated

2008-03-04

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Leiomyomatosis

Keywords

Progesterone, Pulmonary Function Tests, Pneumothorax, Oopherectomy, High Resolution CT, Tuberous Sclerosis Complex, Lymphangioleiomyomatosis (LAM)

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.

Interventions

None listed

Eligibility

Sex/Gender

ALL

Healthy volunteers

Inclusion 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.

Countries

United States

Outcome results