Official Record of Patients Diagnosed With Lymphangioleiomyomatosis (LAM)

NCT00001869 | Completed

• 2 other identifiers: 99002099-H-0020
• Study Type: observational
• Target: 400
• Locations: 1 country
• Sites: 1

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.

Conditions

Leiomyomatosis

Keywords

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

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

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.

Contact the study team to discuss eligibility requirements. They can help determine if this study is right for you.

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI): lead

Study Sites (1)

National Heart, Lung and Blood Institute (NHLBI)

Bethesda, Maryland, 20892, United States

Location

Related Publications (3)

• 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.

  PMID: 8024411 BACKGROUND

• 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.

  PMID: 2367651 BACKGROUND

• 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.

  PMID: 1000470 BACKGROUND

MeSH Terms

Conditions

Leiomyomatosis; Pneumothorax; Tuberous Sclerosis; Lymphangioleiomyomatosis

Condition Hierarchy (Ancestors)

Leiomyoma; Neoplasms, Muscle Tissue; Neoplasms, Connective and Soft Tissue; Neoplasms by Histologic Type; Neoplasms; Pleural Diseases; Respiratory Tract Diseases; Hamartoma; Neoplasms, Multiple Primary; Neoplastic Syndromes, Hereditary; Malformations of Cortical Development, Group I; Malformations of Cortical Development; Nervous System Malformations; Nervous System Diseases; Neurocutaneous Syndromes; Heredodegenerative Disorders, Nervous System; Neurodegenerative Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn; Lymphangiomyoma; Neoplasm, Lymphatic Tissue; Perivascular Epithelioid Cell Neoplasms; Lymphoproliferative Disorders; Lymphatic Diseases; Hemic and Lymphatic Diseases; Immunoproliferative Disorders; Immune System Diseases

Study Design

• Study Type: observational
• Sponsor Type: NIH

Study Record Dates

• First Submitted: November 3, 1999
• First Posted: November 4, 1999
• Study Start: December 1, 1998
• Study Completion: April 1, 2003
• Last Updated: March 4, 2008

Record last verified: 2003-04

Locations

US (1)