Lymphangioleiomyomatosis. Medical search. FAQ

Lymphangioleiomyomatosis (LAM) is a rare disease that generally affects young women and involves the abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs (pulmonary LAM) and extrapulmonary sites (extrapulmonary LAM). (dovepress.com)
Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disease reported to occur exclusively in women. (elsevier.com)
Tazelaar, H. D. / Pulmonary lymphangioleiomyomatosis in a man . (elsevier.com)
Lymphangioleiomyomatosis (LAM) is a low-grade neoplasm characterized by the pulmonary infiltration of smooth muscle-like cells (LAM cells) and cystic destruction. (biomedcentral.com)
RETRACTED ARTICLE: Tuberous sclerosis with pulmonary lymphangioleiomyomatosis and renal angiomyolipomas. (biomedcentral.com)
Computed tomography scan of the chest showed bilateral, diffuse, small thin-walled cysts scattered throughout the lungs characteristic for pulmonary lymphangioleiomyomatosis. (biomedcentral.com)
Pulmonary lymphangioleiomyomatosis and bilateral renal angiomyolipomas are some presentations of tuberous sclerosis and the coexistence of both conditions may cause devastating morbidity and mortality. (biomedcentral.com)

Background: Lymphangioleiomyomatosis is a progressive interstitial lung disease that affects young women. (elsevier.com)

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 present with spontaneous pneumothorax. (epainassist.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)

abstract = "We present two cases that illustrate the appearance of pulmonary lymphangioleiomyomatosis on high resolution CT. (elsevier.com)

Sporadic lymphangioleiomyomatosis (LAM) is a rare form of diffuse parenchymal lung disease. (springer.com)
However, most of the patients with lymphangioleiomyomatosis are not diagnosed with TSC-LAM type of this disease, but rather with the sporadic type of lymphangioleiomyomatosis called S-LAM. (epainassist.com)
Most of the women diagnosed with lymphangioleiomyomatosis have the sporadic type of that illness- S-LAM. (epainassist.com)
Lymphangioleiomyomatosis is caused by mutations (usually sporadic, sometimes in tuberous sclerosis) of the TSC1 or TSC2 genes and results in cystic destruction of the lungs, with CT features being sufficiently characteristic to establish the diagnosis in many cases. (oxfordmedicine.com)
Mohamed Faisal AH, Sharmila N, Yuhanisa A (2017) Sporadic Lymphangioleiomyomatosis Presented with Bilateral Pneumothorax. (scitechnol.com)
The prevalence of Birt-Hogg-Dube syndrome is estimated at 1:200,000, and tuberous sclerosis 1:11,300 -25,000, sporadic lymphangioleiomyomatosis (LAM) affects around 1:125,000 - 500,000 adult women in Europe. (blueprintgenetics.com)

Sadly, the diagnosis of Lymphangioleiomyomatosis is most of the time delayed by 6 years. (epainassist.com)
Transbronchial cryobiopsy of the lung confirmed the diagnosis of lymphangioleiomyomatosis. (scitechnol.com)
Based on history and HRCT findings, a working diagnosis of lymphangioleiomyomatosis (LAM) was made. (scitechnol.com)
The American Thoracic Society and the Japanese Respiratory Society have published additional clinical practice guidelines regarding four specific questions related to the diagnosis of lymphangioleiomyomatosis (LAM) and management of pneumothoraces in patients with LAM. (news-medical.net)

Lymphangioleiomyomatosis (LAM) is the result of disorderly smooth muscle proliferation throughout the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax ) and lymphatics (leading to chylous pleural effusion ). (bionity.com)
The safety of air travel in patients with pneumothorax-prone pulmonary diseases, such as lymphangioleiomyomatosis (LAM), has not been studied to any great extent. (bmj.com)
Lymphangioleiomyomatosis (LAM) is a rare pulmonary condition often presenting with spontaneous pneumothorax. (hindawi.com)
Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that is associated with progressive dyspnea on exertion, recurrent pneumothorax, chylous thoracic and abdominal effusions, and thoracic and abdominal tumors, including lymphangioleiomyomas and angiomyolipomas. (clinicaladvisor.com)
The initial symptoms of lymphangioleiomyomatosis include exertional dyspnea ( shortness of breath ), and spontaneous pneumothorax . (epainassist.com)
Spontaneous pneumothorax is a very serious symptom of lymphangioleiomyomatosis. (epainassist.com)
In the case of lymphangioleiomyomatosis, sudden pneumothorax occurs when some of the cysts which define lymphangioleiomyomatosis suddenly rupture near lung's edge. (epainassist.com)
Pneumothorax is one of the most common symptoms in patients with lymphangioleiomyomatosis (LAM). (biomedcentral.com)
Lymphangioleiomyomatosis (LAM)--an uncommon cause of bilateral spontaneous pneumothorax]. (qxmd.com)
Hereditary cystic lung diseases including alpha-1-antitrypsin deficiency, autosomal recessive (ARCL1) and autosomal dominant cutis laxa (ADCL), Birt-Hogg-Dube syndrome, lymphangioleiomyomatosis (LAM) and tuberous sclerosis are characterized by cystic lung lesions that can result in breathing difficulty, pain and pneumothorax. (blueprintgenetics.com)

RETRACTED ARTICLE: Tuberous sclerosis with pulmonary lymphangioleiomyomatosis and renal angiomyolipomas. (biomedcentral.com)

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)

The average age when symptoms of lymphangioleiomyomatosis occur in people is any age from the early to mid 30s. (epainassist.com)

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)

The differential diagnosis is broad and includes pulmonary lymphangioleiomyomatosis (LAM), langerhans cell histiocytosis, lymphocytic interstitial pneumonia, Birt-Hogg- Dube disease, neurofibromatosis and papillomatosis. (scitechnol.com)

Lymphangioleiomyomatosis (LAM) is a multi-system disease, affecting predominantly pre-menopausal women, that is characterized by proliferation of abnormal smooth muscle-like cells (LAM cells) leading to the formation of lung cysts, fluid-filled cystic tumors in the axial lymphatics (e.g., lymphangioleiomyomas ), and abdominal tumors, primarily in the kidneys, comprising adipose cells, vascular structures and LAM cells (e.g., angiomyolipomas). (atlasgeneticsoncology.org)
Angiomyolipomas are common in people with lymphangioleiomyomatosis. (epainassist.com)
Lymphangioleiomyomatosis (LAM) is a rare multisystem disease almost exclusively affecting women which causes loss of lung function, lymphatic abnormalities and angiomyolipomas. (onmedica.com)
Pulmonary lymphangioleiomyomatosis and bilateral renal angiomyolipomas are some presentations of tuberous sclerosis and the coexistence of both conditions may cause devastating morbidity and mortality. (biomedcentral.com)
Tuberous sclerosis syndrome (TSC) is an autosomal dominant tumor suppressor gene syndrome affecting multiple organs, including renal angiomyolipomas and pulmonary lymphangioleiomyomatosis (LAM). (harvard.edu)

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)

Estrogen maintains myometrial tumors in a lymphangioleiomyomatosis model. (rochester.edu)

Earlier reports indicated a grim prognosis for lymphangioleiomyomatosis (LAM), with progressive respiratory failure and death within 10 years of diagnosis. (medscape.com)
Lymphangioleiomyomatosis diagnosis and prognosis - Get information about diagnosis and prognosis of Lymphangioleiomyomatosis, onlymyhealth.com is providing articles related to Lymphangioleiomyomatosis diagnosis and prognosis. (onlymyhealth.com)

Lymphangioleiomyomatosis (LAM) is a rare lung disease that is caused by genetic mutations. (clinicaltrials.gov)

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)

Although primarily a disease of women of childbearing age, lymphangioleiomyomatosis (LAM) has also been reported in postmenopausal patients. (medscape.com)
BACKGROUND: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease occurring in women of reproductive age and leading to progressive respiratory failure in spite of treatment. (biomedsearch.com)
Lymphangioleiomyomatosis is one of the rarest lung illnesses that usually affects the women in their child bearing period of life. (epainassist.com)
The reason for that is the fact that only in extremely rare cases women before puberty or after the menopause develop lymphangioleiomyomatosis. (epainassist.com)
Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disease reported to occur exclusively in women. (elsevier.com)
Background: Lymphangioleiomyomatosis is a progressive interstitial lung disease that affects young women. (elsevier.com)
Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease that affects primarily women, usually ages 20-40 and during their childbearing years. (utswmed.org)
A rare and potentially deadly lung disease called Lymphangioleiomyomatosis (LAM) strikes women during their childbearing years. (news-medical.net)
Lymphangioleiomyomatosis (LAM) is a progressive neoplastic disorder that leads to lung destruction and respiratory failure primarily in women. (rupress.org)

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)

Lymphangioleiomyomatosis (LAM) is an indolent, progressive growth of smooth muscle cells throughout the lungs, pulmonary blood vessels, lymphatics, and pleurae. (kudratiayurved.com)

An ardent patient advocate, Dr. Trapnell has worked with patient foundations for cystic fibrosis (CF), alpha-1-antitrypsin deficiency, pulmonary alveolar proteinosis, lymphangioleiomyomatosis, and childhood interstitial lung diseases. (cincinnatichildrens.org)

The international frequency of lymphangioleiomyomatosis (LAM) is unknown, although Europe and Japan report case series. (medscape.com)
An early case of pulmonary lymphangioleiomyomatosis diagnosed by video-assisted thoracoscopic surgery. (qxmd.com)

Pulmonary lymphangioleiomyomatosis: high-resolution CT findings in four cases. (semanticscholar.org)

Journal Article] Lymphangiogenesis-Mediated Shedding of LAM Cell Clusters as a Mechanism for Dissemination in Lymphangioleiomyomatosis. (nii.ac.jp)

Histologic examination of lung and lymph node biopsies demonstrated lymphangioleiomyomatosis. (nih.gov)

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