• Chest computed tomography (CT) revealed cystic formations throughout the lungs, consistent with lymphangioleiomyomatosis (Figure 1 ), and the presence of a pericardial effusion. (biomedcentral.com)
  • Lymphangioleiomyomatosis (LAM) may occur sporadically or in association with tuberous sclerosis complex (TSC). (medscape.com)
  • Lymphangioleiomyomatosis, or LAM, can also occur in women who have a rare disease called tuberous sclerosis complex (TSC). (nationaljewish.org)
  • Lymphangioleiomyomatosis (LAM) is a rare progressive cystic lung disease affecting primarily females, characterised by the proliferation of neoplastic LAM cells with mutations in the tuberous sclerosis complex ( TSC )1 or TSC 2 genes. (ersjournals.com)
  • Most of the women who were diagnosed with Tuberous Sclerosis Complex (TSC-LAM) can expect to develop lymphangioleiomyomatosis. (epainassist.com)
  • Men diagnosed with lymphangioleiomyomatosis get it only due to pre-existing Tuberous Sclerosis Complex. (epainassist.com)
  • Lymphangioleiomyomatosis is highly similar to a condition called Tuberous Sclerosis Complex (TSC) and it often comes in pair with that illness (TSC-LAM). (epainassist.com)
  • ABSTRACT: Lymphangioleiomyomatosis (LAM) is a rare lung-metastasizing neoplasm caused by the proliferation of smooth muscle-like cells that commonly carry loss-of-function mutations in either the tuberous sclerosis complex 1 or 2 (TSC1 or TSC2) genes. (thecannabisadvisory.com)
  • The advent of pharmacological therapies for lymphangioleiomyomatosis (LAM) has made early diagnosis important in women with tuberous sclerosis complex (TSC), although the lifelong cumulative radiation exposure caused by chest computer tomography (CT) should not be underestimated. (cdc.gov)
  • Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial. (medscape.com)
  • Background: TSC2-deficient cells can proliferate in the lungs, kidneys, and other organs causing devastating progressive multisystem disorders such as lymphangioleiomyomatosis (LAM) and tuberous sclerosis complex (TSC). (harvard.edu)
  • Costello LC, Hartman TE and Ryu JH (2000) High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex. (els.net)
  • Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. (wikipedia.org)
  • Lymphangioleiomyomatosis (LAM), a disease that occurs primarily in women, is characterized by cystic lung lesions causing respiratory failure, which may require lung transplantation. (nih.gov)
  • Cystic lung disease with no pulmonary nodules is consistent with lymphangioleiomyomatosis. (radiopaedia.org)
  • Lymphangioleiomyomatosis (lim-FAN-je-o-LI-o-MI-o-ma-TO-sis), or LAM, is a rare cystic lung disease that mostly affects women in their mid-forties. (nih.gov)
  • Lymphangioleiomyomatosis (LAMP) is a progressive cystic lung disease that predominantly affects young women [ 1 ]. (omicsonline.org)
  • Lymphangioleiomyomatosis (LAM) is a rare, progressive, cystic lung disease that mostly affects women of child-bearing age and is characterized by abnormal smooth muscle cell proliferation. (biomedcentral.com)
  • Lymphangioleiomyomatosis (LAM), a multisystem disease of women, is manifest by the proliferation of smooth muscle-like cells in the lung resulting in cystic lung destruction. (harvard.edu)
  • A high index of clinical suspicion for lymphangioleiomyomatosis (LAM) should be maintained in women of childbearing age who present with recurrent pneumothoraces, when chylous effusion or an interstitial pattern on chest radiograph is not identified. (medscape.com)
  • A 28-year-old woman with lymphangioleiomyomatosis had severe changes in the chest radiograph and recurrent pneumothoraces. (nih.gov)
  • Recurrent lymphangioleiomyomatosis after living-donor lobar lung transplantation. (qxmd.com)
  • Abnormal growth of smooth muscle-like cells in lymphangioleiomyomatosis: Role for tumor suppressor TSC2. (nih.gov)
  • The TSC1 and TSC2 proteins, which function as a TSC1/TSC2 tumor suppressor complex, are associated with lymphangioleiomyomatosis (LAM), a genetic disorder characterized by the abnormal growth of smooth muscle-like cells in the lungs. (nih.gov)
  • Together with tuberous sclerosis, mutations in TSC2 can cause Lymphangioleiomyomatosis, a disease caused by the enlargement of tissue in the lungs, creating cysts and tumours and causing difficulty breathing. (wikipedia.org)
  • Lymphangioleiomyomatosis (LAM) is an often fatal disease primarily affecting young women in which tuberin (TSC2)-null cells metastasize to the lungs. (pnas.org)
  • Chest computed tomography (CT) revealed cystic formations throughout the lungs, consistent with lymphangioleiomyomatosis (Figure 1 ), and the presence of a pericardial effusion. (biomedcentral.com)
  • A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. (go.jp)
  • Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. (semanticscholar.org)
  • Studies published in the Jan. 10 edition of the New England Journal of Medicine (NEJM) are providing clues into the treatment and diagnosis of LAM, or lymphangioleiomyomatosis, a progressive and deadly lung disease that affects women in their childbearing years. (news-medical.net)
  • Lymphangioleiomyomatosis, also known as LAM, is a rare, progressive lung disease that most commonly affects women in their 30s and 40s, and almost never occurs in men. (brighamandwomens.org)
  • Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease affecting women that was first described by von Stossel in 1937. (medscape.com)
  • Lymphatic involvement is seen in lymphangioleiomyomatosis (LAM) and idiopathic pulmonary fibrosis (IPF). (oalib.com)
  • Lymphangioleiomyomatosis (LAM) is a rare, idiopathic hamartomatosis characterized by smooth muscle cell hyperplasia along the terminal bronchioles in the lung, the lymphatic and blood vessels of the thorax and the retroperitoneum. (eurorad.org)
  • METHODOLOGY: This study was a retrospective review of the medical records of 50 patients, who were assessed for lymphangioleiomyomatosis from 1978 to 2002 at Mayo Clinic in Rochester, Minnesota, and Jacksonville, Florida, USA. (biomedsearch.com)
  • Sphingolipid, fatty acid and phospholipid metabolites are associated with disease severity and mTOR inhibition in lymphangioleiomyomatosis. (onmedica.com)
  • for the NIH Rare Lung Disease Consortium 2019, ' Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis ', European Respiratory Journal , vol. 53, no. 4, 1802066. (elsevier.com)
  • Chest radiograph and pulmonary function test (PFT) findings, while suggestive of lymphangioleiomyomatosis (LAM), can be nonspecific and may be normal despite the presence of symptoms. (medscape.com)
  • Chylous ascites is a symptom of lymphangioleiomyomatosis as it is a condition when the flow of vital lymph liquid is blocked by errant smooth muscle cells. (epainassist.com)