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Lymphangioleiomyomatosis

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to the members of the Scientific Advisory Board of the LAM Foundation for assistance in the preparation of this report and Joel Moss, MD, PhD at the National Heart, Lung, and Blood Insitute, National Institutes of Health, for reviewing this report.

Synonyms of Lymphangioleiomyomatosis

  • LAM
  • Lymphangioleimyomatosis
  • Lymphangioleiomatosis
  • Pulmonary Lymphangiomyomatosis
  • Sporadic Lymphangioleiomyomatosis

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Lymphangioleiomyomatosis (LAM) is a rare progressive multisystem disorder that predominantly affects women of childbearing age. It occurs in women who have tuberous sclerosis, and also in women who do not have a heritable genetic disorder. LAM is characterized by the spread and uncontrolled growth (proliferation) of specialized cells (smooth muscle-like LAM cells) in certain organs of the body, especially the lungs, kidney and lymphatics. Common symptoms associated with LAM include coughing and/or difficulty breathing (dyspnea), especially following periods of exercise or exertion. Affected individuals may also experience complications including collapse of a lung or fluid accumulation around the lungs (pleural effusion). The disorder is progressive and, in some cases, may result in chronic respiratory failure.

Organizations related to Lymphangioleiomyomatosis

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

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