A disease characterized by the progressive invasion of SMOOTH MUSCLE CELLS into the LYMPHATIC VESSELS, and the BLOOD VESSELS. The majority of the cases occur in the LUNGS of women of child-bearing age, eventually blocking the flow of air, blood, and lymph. The common symptom is shortness of breath (DYSPNEA).
A tumorlike condition characterized by SMOOTH MUSCLE and ENDOTHELIUM proliferation of LYMPHATIC VESSELS and LYMPH NODES in the MEDIASTINUM and retroperitoneum, also in the lung. It may be manifested by chylous PLEURAL EFFUSION and ASCITES.
A benign tumor containing vascular, adipose, and muscle elements. It occurs most often in the kidney with smooth muscle elements (angiolipoleiomyoma) in association with tuberous sclerosis. (Dorland, 27th ed)
Autosomal dominant neurocutaneous syndrome classically characterized by MENTAL RETARDATION; EPILEPSY; and skin lesions (e.g., adenoma sebaceum and hypomelanotic macules). There is, however, considerable heterogeneity in the neurologic manifestations. It is also associated with cortical tuber and HAMARTOMAS formation throughout the body, especially the heart, kidneys, and eyes. Mutations in two loci TSC1 and TSC2 that encode hamartin and tuberin, respectively, are associated with the disease.
An accumulation of air or gas in the PLEURAL CAVITY, which may occur spontaneously or as a result of trauma or a pathological process. The gas may also be introduced deliberately during PNEUMOTHORAX, ARTIFICIAL.
An opaque, milky-white fluid consisting mainly of emulsified fats that passes through the lacteals of the small intestines into the lymphatic system.
A large group of diseases which are characterized by a low prevalence in the population. They frequently are associated with problems in diagnosis and treatment.
Cellular antigens that are specific for MELANOMA cells.
Tumors or cancer of the LUNG.
A vascular endothelial growth factor that specifically binds to VASCULAR ENDOTHELIAL GROWTH FACTOR RECEPTOR-2 and VASCULAR ENDOTHELIAL GROWTH FACTOR RECEPTOR-3. In addition to being an angiogenic factor it can act on LYMPHATIC VESSELS to stimulate LYMPHANGIOGENESIS. It is similar in structure to VASCULAR ENDOTHELIAL GROWTH FACTOR C in that they both contain N- and C-terminal extensions that were not found in other VEGF family members.
Proteins that are normally involved in holding cellular growth in check. Deficiencies or abnormalities in these proteins may lead to unregulated cell growth and tumor development.
Presence of milky lymph (CHYLE) in the PERITONEAL CAVITY, with or without infection.
The co-occurrence of pregnancy and NEOPLASMS. The neoplastic disease may precede or follow FERTILIZATION.
Measurement of the various processes involved in the act of respiration: inspiration, expiration, oxygen and carbon dioxide exchange, lung volume and compliance, etc.
A serine threonine kinase that controls a wide range of growth-related cellular processes. The protein is referred to as the target of RAPAMYCIN due to the discovery that SIROLIMUS (commonly known as rapamycin) forms an inhibitory complex with TACROLIMUS BINDING PROTEIN 1A that blocks the action of its enzymatic activity.
The production of adhesions between the parietal and visceral pleura. The procedure is used in the treatment of bronchopleural fistulas, malignant pleural effusions, and pneumothorax and often involves instillation of chemicals or other agents into the pleural space causing, in effect, a pleuritis that seals the air leak. (From Fishman, Pulmonary Diseases, 2d ed, p2233 & Dorland, 27th ed)
Tumors or cancers of the KIDNEY.
Pathological processes involving any part of the LUNG.
A macrolide compound obtained from Streptomyces hygroscopicus that acts by selectively blocking the transcriptional activation of cytokines thereby inhibiting cytokine production. It is bioactive only when bound to IMMUNOPHILINS. Sirolimus is a potent immunosuppressant and possesses both antifungal and antineoplastic properties.
A ribosomal protein that may play a role in controlling cell growth and proliferation. It is a major substrate of RIBOSOMAL PROTEIN S6 KINASES and plays a role in regulating the translation (TRANSLATION, GENETIC) of RNAs that contain an RNA 5' TERMINAL OLIGOPYRIMIDINE SEQUENCE.
The transference of either one or both of the lungs from one human or animal to another.
Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.
Tubular vessels that are involved in the transport of LYMPH and LYMPHOCYTES.
Tomography using x-ray transmission and a computer algorithm to reconstruct the image.
Endoscopic surgery of the pleural cavity performed with visualization via video transmission.
A cysteine protease that is highly expressed in OSTEOCLASTS and plays an essential role in BONE RESORPTION as a potent EXTRACELLULAR MATRIX-degrading enzyme.

Pulmonary lymphangioleiomyomatosis in Korea. (1/169)

BACKGROUND: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease occurring in women of reproductive age and leading to progressive respiratory failure in spite of treatment. In Korea the first case was reported in 1984 and by 1997 a total of 23 cases had been reported. The clinical findings of these Korean cases are reviewed. METHODS: The details of 10 cases of LAM on file at Seoul National University Hospital were reviewed together with those of 13 cases previously reported from other Korean institutes. Two, including the only one to be reported in a man, were excluded after reviewing the clinical, radiological, and pathological findings, leaving a total of 21 cases in the present study. RESULTS: All 21 patients were women and in all cases the disease was proven pathologically. The mean (SD) age at onset of symptoms was 32 (8.6) years. The most common symptoms were dyspnoea and pneumothorax which were seen in 19 (90%) and 13 (76%) patients, respectively. Pulmonary function tests showed decreased transfer factor (TLCO) (100%) and airflow limitation (67%). All the cases had characteristic cysts on high resolution computed tomographic (HRCT) scanning. The overall severity score based on HRCT scans correlated with the percentage predicted TLCO/VA (p = 0.03) and FEV1/FVC (p = 0.02). The patients were all treated with medroxyprogesterone and/or tamoxifen. Follow up was possible in 10 cases. Two of these patients appeared to stabilise with no appreciable change clinically or in lung function on medroxyprogesterone and/or tamoxifen, but the remaining patients all deteriorated with two dying of respiratory insufficiency and one of infection following lung transplantation. CONCLUSIONS: As in other countries, in Korea LAM occurs exclusively in women and progresses despite hormonal treatment.  (+info)

Metastatic endometrial stromal sarcoma masquerading as pulmonary lymphangioleiomyomatosis. (2/169)

A 39 year old female presented with bilateral pneumothoraces and interstitial shadowing on chest x ray. A diagnosis of lymphangioleiomyomatosis was made following an open lung biopsy. Over the next eight years she developed respiratory failure leading to single lung transplantation but she died in the immediate postoperative period. Necropsy examination and review of the previous open lung biopsy revealed multiple pulmonary metastases from a low grade endometrial stromal sarcoma of the uterus. This case high-lights the importance of an accurate diagnosis before transplantation.  (+info)

Mutational analysis of the tuberous sclerosis gene TSC2 in patients with pulmonary lymphangioleiomyomatosis. (3/169)

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder limited almost exclusively to women of reproductive age. LAM affects about 5% of women with tuberous sclerosis complex (TSC). LAM also occurs in women who do not have TSC (sporadic LAM). TSC is a tumour suppressor gene syndrome characterised by seizures, mental retardation, and tumours in the brain, heart, and kidney. Angiomyolipomas, which are benign tumours with smooth muscle, fat, and dysplastic vascular components, are the most common renal tumour in TSC. Renal angiomyolipomas also occur in 63% of sporadic LAM patients. We recently found that 54% of these angiomyolipomas have TSC2 loss of heterozygosity, leading to the hypothesis that sporadic LAM is genetically related to TSC. In this study, we screened DNA from 21 women with sporadic LAM for mutations in all 41 exons of TSC2. Twelve of the patients had known renal angiomyolipomas. No TSC2 mutations were detected. We did find three silent TSC2 polymorphisms. We conclude that patients with sporadic LAM, including those with renal angiomyolipomas, do not have a high frequency of germline mutations in the coding region of TSC2.  (+info)

Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis. (4/169)

Lymphangioleiomyomatosis (LAM) is a progressive and often fatal interstitial lung disease characterized by a diffuse proliferation of abnormal smooth muscle cells in the lungs. LAM is of unusual interest biologically because it affects almost exclusively young women. LAM can occur as an isolated disorder (sporadic LAM) or in association with tuberous sclerosis complex. Renal angiomyolipomas, which are found in most tuberous sclerosis patients, also occur in 60% of sporadic LAM patients. We previously found TSC2 loss of heterozygosity in 7 of 13 (54%) of angiomyolipomas from sporadic LAM patients, suggesting that LAM and TSC could have a common genetic basis. In this study, we report the identification of somatic TSC2 mutations in five of seven angiomyolipomas from sporadic LAM patients. In all four patients from whom lung tissue was available, the same mutation found in the angiomyolipoma was present in the abnormal pulmonary smooth muscle cells. In no case was the mutation present in normal kidney, morphologically normal lung, or lymphoblastoid cells. Our data demonstrate that somatic mutations in the TSC2 gene occur in the angiomyolipomas and pulmonary LAM cells of women with sporadic LAM, strongly supporting a direct role of TSC2 in the pathogenesis of this disease.  (+info)

Oestrogen metabolism in lymphangioleiomyomatosis: catechol-O-methyltransferase pathway is not involved. (5/169)

BACKGROUND: Lymphangioleiomyomatosis (LAM) is an uncommon lung disease for which no effective method of treatment has been found. The predilection of LAM for premenopausal women has led to the assumption that hormonal factors play an important role in the pathogenesis of this disease. The aim of this study was to determine if women with LAM manifest alterations in the catechol-O-methyltransferase (COMT) pathway which is essential for preventing the generation of oestrogen derived reactive oxygen species (ROS). METHODS: Blood samples were collected from 15 women with LAM and compared with appropriate controls. The distribution of high and low activity alleles of COMT was determined with a PCR based RFLP assay. The enzymatic activity of COMT was measured in each sample and the potential presence of a circulating inhibitor of COMT was determined. Since an alteration in the COMT pathway could increase the oxidative stress, the plasma concentration of malondialdehyde (MDA), a secondary product generated from lipid peroxidation, has been used as an internal marker. RESULTS: The distribution of high and low activity alleles of COMT (named COMT(HH), COMT(LL), and COMT(HL)) was similar in the two groups with proportions of 40%, 7%, and 53%, respectively, in the women with LAM and 38%, 6%, and 56% in the control subjects. The mean (SD) COMT activity was 24.2 (12.3) pmol/min/mg protein in women with LAM and 24.1 (6.3) pmol/min/mg protein in the control group. Incubation of plasma from women in the two groups with a preparation of commercial COMT showed that no detectable COMT inhibitor was present. The plasma concentration of MDA in the women with LAM was also not significantly different from control subjects. CONCLUSIONS: This study shows that there are no significant alterations in the COMT pathway of women with LAM. It is therefore unlikely that alterations in oestrogen mediated cell signalling pathways are mediated by oxidants derived from an excess of catecholoestrogens in LAM.  (+info)

Pulmonary lymphangiomyomatosis (LAM) developing chylothorax. (6/169)

We describe a case of pulmonary lymphangiomyomatosis (LAM) with chylothorax that developed in a 46-year-old Japanese woman. This patient exhibited clinical symptoms of dyspnea and chest X-ray showed right pleural effusion. Thoracocentesis demonstrated chylous effusion. Chest computed tomography (CT) scan revealed multiple cystic lesions. Subsequent thoracoscopy revealed the chylorrhea from swelled vessels on the diaphragm. The clinical diagnosis, based on histological examinations with biopsy specimens obtained by thoracoscopy, was pulmonary LAM. Although the hormone therapy was not effective, chylous effusion was improved by the pleurodesis. Pulmonary LAM developing chylothorax is rare in Japan.  (+info)

Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. (7/169)

A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. The clinical features include dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, and chylous ascites. It is characterized by: 1) proliferation of abnormal smooth muscle cells (LAM cells) in pulmonary interstitium and along the axial lymphatics of the thorax and abdomen; 2) thin-walled pulmonary cysts, and 3) a high incidence of angiomyolipomas. The pulmonary cystic lesions have a characteristic appearance on high resolution computed tomography. The most specific method for diagnosing LAM is lung biopsy to demonstrate the presence of LAM cells, either by their characteristic histological appearance or by specific immunostaining with HMB-45 antibody. LAM cells differ in several important respects from the types of smooth muscle cells normally present in lung. Their reactivity with HMB-45 antibody is localized in stage I and stage II melanosomes. LAM cells show additional evidence of incomplete melanogenesis, and the significance of these observations remains to be determined. Two types of LAM cells are recognized: 1) small, spindle-shaped cells that are centrally located in the LAM nodules and are highly immunoreactive for matrix metalloproteinase-2 (MMP-2), its activating enzyme (MT-1-MMP), and proliferating cell nuclear antigen (PCNA), and 2) large, epithelioid cells that are distributed along the periphery of the nodules and show a high degree of immunoreactivity with HMB-45 antibody and with antibodies against estrogen and progesterone receptors. Types of treatment used for LAM include oophorectomy, administration of Lupron or progesterone and in very severe cases, pulmonary transplantation (following the onset of respiratory insufficiency, not relieved by O(2)).  (+info)

Clinical experience of lymphangioleiomyomatosis in the UK. (8/169)

BACKGROUND: Lymphangioleiomyomatosis is a rare lung disease that affects only women. No controlled trials of management have been performed and, until such data are available, management must be based on clinical experience. This study provides data on the natural history of lymphangioleiomyomatosis in the UK and compares this with experience from other centres. METHODS: We tried to identify all cases of lymphangioleiomyomatosis in the UK over a five year period by contacting all chest physicians. Cases were confirmed by lung biopsy or history and high resolution computed tomographic (CT) scanning. Details of disease and management were obtained from hospital notes. RESULTS: The 50 patients who fitted the diagnostic criteria for lymphangioleiomyomatosis had a median age at onset of 35 years (range 22-50). Five presented when postmenopausal (four taking hormone replacement therapy). Pneumothorax and dyspnoea were the most common presenting features. Extrapulmonary presentations included renal angiomyolipomas (3) and lymphangiomyomas (2). Only half the patients were assessed for renal angiomyolipoma and six were identified. Thirty patients had had one or more pneumothoraces, of which two thirds recurred if treated conservatively. Chylous effusions occurred in 11 patients, five requiring surgery. Pregnancy was uncommon once the diagnosis was made (n=7), but was associated with an increase in complications. Half the patients were taking a beta agonist and many showed a bronchodilator response in the laboratory. Thirty six patients had received hormone treatment. CONCLUSIONS: Our UK five year period prevalence was one per 1.1 million population. Since prophylactic interventions are sometimes indicated for renal angiomyolipoma, these data suggest that screening for angiomyolipoma, ideally by CT scanning, may be underused. Patients need to be aware of the increase in complications associated with pregnancy. Recurrence rate of pneumothorax was high in those not treated surgically. Hormone treatment was used variably and controlled trials are needed to determine their role and the optimum duration and dose.  (+info)

Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease that primarily affects women of childbearing age. It is characterized by the abnormal growth of smooth muscle cells in the airways, blood vessels, and lymphatic system of the lungs. These cells can form cysts and lesions that can obstruct the airways and cause lung function to decline over time.

LAM can also affect other organs, such as the kidneys, where it can cause angiomyolipomas (benign tumors composed of blood vessels, muscle cells, and fat). In some cases, LAM may be associated with tuberous sclerosis complex (TSC), a genetic disorder that causes benign tumors to grow in various organs of the body.

The exact cause of LAM is not fully understood, but it is believed to be related to mutations in the TSC1 or TSC2 genes, which regulate cell growth and division. There is currently no cure for LAM, but treatments such as lung transplantation and medications that suppress the growth of smooth muscle cells may help manage symptoms and slow disease progression.

Lymphangiomyoma is a rare, benign tumor that typically arises in the soft tissues of the body. It is composed of smooth muscle cells and lymphatic vessels. These tumors most commonly occur in the cervix, mediastinum, and retroperitoneum. They can cause various symptoms depending on their location, such as abdominal pain or distention, difficulty breathing, or abnormal vaginal bleeding.

Lymphangiomyomas are thought to arise from aberrant smooth muscle proliferation around lymphatic vessels. While they are generally considered benign, they can still cause problems due to their mass effect and potential for local invasion. Treatment typically involves surgical excision of the tumor.

It is important to note that while I strive to provide accurate information, my responses should not be used as a substitute for professional medical advice or treatment.

Angiomyolipoma is a type of benign tumor that occurs most commonly in the kidney. It is composed of blood vessels (angio-), smooth muscle cells (myo-), and fat cells (lipo-). Angiomyolipomas are usually associated with the genetic disorder tuberous sclerosis complex, but they can also occur spontaneously or as a result of other genetic conditions.

These tumors can vary in size and may cause symptoms such as pain, blood in the urine, or a palpable mass in the abdomen if they grow large enough. In some cases, angiomyolipomas may also be at risk for rupture and bleeding, particularly if they are larger than 4 cm in size.

Treatment options for angiomyolipomas include surveillance with imaging tests, medication to reduce the risk of bleeding, or surgical removal of the tumor. The choice of treatment depends on factors such as the size and location of the tumor, the presence of symptoms, and the patient's overall health.

Tuberous Sclerosis Complex (TSC) is a rare genetic disorder that causes non-cancerous (benign) tumors to grow in many parts of the body. These tumors can affect the brain, skin, heart, kidneys, eyes, and lungs. The signs and symptoms of TSC can vary widely, depending on where the tumors develop and how severely a person is affected.

The condition is caused by mutations in either the TSC1 or TSC2 gene, which regulate a protein that helps control cell growth and division. When these genes are mutated, the protein is not produced correctly, leading to excessive cell growth and the development of tumors.

TSC is typically diagnosed based on clinical symptoms, medical imaging, and genetic testing. Treatment for TSC often involves a multidisciplinary approach, with specialists in neurology, dermatology, cardiology, nephrology, pulmonology, and ophthalmology working together to manage the various symptoms of the condition. Medications, surgery, and other therapies may be used to help control seizures, developmental delays, skin abnormalities, and other complications of TSC.

Pneumothorax is a medical condition that refers to the presence of air in the pleural space, which is the potential space between the lungs and the chest wall. This collection of air can result in a partial or complete collapse of the lung. The symptoms of pneumothorax may include sudden chest pain, shortness of breath, cough, and rapid heartbeat.

The two main types of pneumothorax are spontaneous pneumothorax, which occurs without any apparent cause or underlying lung disease, and secondary pneumothorax, which is caused by an underlying lung condition such as chronic obstructive pulmonary disease (COPD), asthma, or lung cancer.

Treatment for pneumothorax may include observation, oxygen therapy, needle aspiration, or chest tube insertion to remove the excess air from the pleural space and allow the lung to re-expand. In severe cases, surgery may be required to prevent recurrence.

Chyle is a milky, slightly opaque fluid that is present in the lymphatic system. It is formed in the small intestine during the digestion of food, particularly fats. Chyle consists of emulsified fat droplets (chylomicrons), proteins, electrolytes, and lymphocytes suspended in a watery solution. It is transported through the lacteals in the villi of the small intestine into the cisterna chyli and then to the thoracic duct, where it empties into the left subclavian vein. From there, it mixes with blood and circulates throughout the body. Chyle formation plays a crucial role in fat absorption and transportation in the human body.

A rare disease, also known as an orphan disease, is a health condition that affects fewer than 200,000 people in the United States or fewer than 1 in 2,000 people in Europe. There are over 7,000 rare diseases identified, and many of them are severe, chronic, and often life-threatening. The causes of rare diseases can be genetic, infectious, environmental, or degenerative. Due to their rarity, research on rare diseases is often underfunded, and treatments may not be available or well-studied. Additionally, the diagnosis of rare diseases can be challenging due to a lack of awareness and understanding among healthcare professionals.

Melanoma-specific antigens are proteins or other molecules that are present on melanoma cells but not normally found on healthy cells in the body. These antigens can be recognized by the immune system as foreign and trigger an immune response, making them potential targets for immunotherapy treatments for melanoma.

There are two main types of melanoma-specific antigens: tumor-specific antigens (TSAs) and tumor-associated antigens (TAAs). TSAs are unique to cancer cells and are not found on normal cells, while TAAs are overexpressed or mutated versions of proteins that are also present in normal cells.

Examples of melanoma-specific antigens include Melan-A/MART-1, gp100, and tyrosinase. These antigens have been studied extensively as targets for cancer vaccines, adoptive cell therapy, and other immunotherapy approaches to treat melanoma.

Lung neoplasms refer to abnormal growths or tumors in the lung tissue. These tumors can be benign (non-cancerous) or malignant (cancerous). Malignant lung neoplasms are further classified into two main types: small cell lung carcinoma and non-small cell lung carcinoma. Lung neoplasms can cause symptoms such as cough, chest pain, shortness of breath, and weight loss. They are often caused by smoking or exposure to secondhand smoke, but can also occur due to genetic factors, radiation exposure, and other environmental carcinogens. Early detection and treatment of lung neoplasms is crucial for improving outcomes and survival rates.

Vascular Endothelial Growth Factor D (VEGFD) is a protein that belongs to the family of vascular endothelial growth factors. It plays an essential role in the process of angiogenesis, which is the formation of new blood vessels from pre-existing ones. Specifically, VEGFD stimulates the growth and proliferation of lymphatic endothelial cells, thereby promoting the development and maintenance of the lymphatic system.

VEGFD binds to its specific receptor, VEGFR-3, which is primarily expressed on the surface of lymphatic endothelial cells. This binding triggers a cascade of intracellular signaling events that ultimately lead to the activation of various genes involved in cell proliferation, migration, and survival.

Dysregulation of VEGFD and its receptor has been implicated in several pathological conditions, including lymphatic malformations, cancer, and inflammatory diseases. In these contexts, the overexpression or aberrant activation of VEGFD can contribute to excessive angiogenesis and lymphangiogenesis, leading to tissue edema, tumor growth, and metastasis. Therefore, targeting the VEGFD signaling pathway has emerged as a promising therapeutic strategy for various diseases.

Tumor suppressor proteins are a type of regulatory protein that helps control the cell cycle and prevent cells from dividing and growing in an uncontrolled manner. They work to inhibit tumor growth by preventing the formation of tumors or slowing down their progression. These proteins can repair damaged DNA, regulate gene expression, and initiate programmed cell death (apoptosis) if the damage is too severe for repair.

Mutations in tumor suppressor genes, which provide the code for these proteins, can lead to a decrease or loss of function in the resulting protein. This can result in uncontrolled cell growth and division, leading to the formation of tumors and cancer. Examples of tumor suppressor proteins include p53, Rb (retinoblastoma), and BRCA1/2.

Chylous ascites is a medical condition characterized by the accumulation of milky, fat-containing fluid in the peritoneal cavity, which is the space within the abdomen that contains the intestines, liver, and other organs. The fluid, called chyle, is normally found in the lymphatic system and is formed when dietary fats are absorbed from the small intestine.

Chylous ascites can occur as a result of damage to the lymphatic vessels that transport chyle from the intestines to the bloodstream. This damage can be caused by various conditions, such as trauma, surgery, tumors, inflammation, or congenital abnormalities. When the lymphatic vessels are damaged, chyle leaks into the peritoneal cavity and accumulates there, leading to ascites.

Symptoms of chylous ascites may include abdominal distension, pain, nausea, vomiting, and weight loss. The condition can be diagnosed through various tests, such as imaging studies or analysis of the fluid in the peritoneal cavity. Treatment typically involves addressing the underlying cause of the condition, as well as managing symptoms and preventing complications. This may include dietary modifications, medications to reduce lymphatic flow, or surgical interventions to repair damaged lymphatic vessels.

Neoplastic pregnancy complications refer to the abnormal growth of cells (neoplasia) that can occur during pregnancy. These growths can be benign or malignant and can arise from any type of tissue in the body. However, when they occur in pregnant women, they can pose unique challenges due to the potential effects on the developing fetus and the changes in the mother's body.

Some common neoplastic pregnancy complications include:

1. Gestational trophoblastic disease (GTD): This is a group of rare tumors that occur in the uterus during pregnancy. GTD can range from benign conditions like hydatidiform mole to malignant forms like choriocarcinoma.
2. Breast cancer: Pregnancy-associated breast cancer (PABC) is a type of breast cancer that occurs during pregnancy or within one year after delivery. It can be aggressive and challenging to diagnose due to the changes in the breast tissue during pregnancy.
3. Cervical cancer: Cervical cancer can occur during pregnancy, and its management depends on the stage of the disease and the gestational age. In some cases, treatment may need to be delayed until after delivery.
4. Lung cancer: Pregnancy does not increase the risk of lung cancer, but it can make diagnosis and treatment more challenging.
5. Melanoma: Melanoma is the most common malignant skin cancer during pregnancy. It can spread quickly and requires prompt treatment.

The management of neoplastic pregnancy complications depends on several factors, including the type and stage of the tumor, gestational age, and the patient's wishes. In some cases, surgery, chemotherapy, or radiation therapy may be necessary. However, these treatments can have potential risks to the developing fetus, so a multidisciplinary team of healthcare providers is often involved in the care of pregnant women with neoplastic complications.

Respiratory Function Tests (RFTs) are a group of medical tests that measure how well your lungs take in and exhale air, and how well they transfer oxygen and carbon dioxide into and out of your blood. They can help diagnose certain lung disorders, measure the severity of lung disease, and monitor response to treatment.

RFTs include several types of tests, such as:

1. Spirometry: This test measures how much air you can exhale and how quickly you can do it. It's often used to diagnose and monitor conditions like asthma, chronic obstructive pulmonary disease (COPD), and other lung diseases.
2. Lung volume testing: This test measures the total amount of air in your lungs. It can help diagnose restrictive lung diseases, such as pulmonary fibrosis or sarcoidosis.
3. Diffusion capacity testing: This test measures how well oxygen moves from your lungs into your bloodstream. It's often used to diagnose and monitor conditions like pulmonary fibrosis, interstitial lung disease, and other lung diseases that affect the ability of the lungs to transfer oxygen to the blood.
4. Bronchoprovocation testing: This test involves inhaling a substance that can cause your airways to narrow, such as methacholine or histamine. It's often used to diagnose and monitor asthma.
5. Exercise stress testing: This test measures how well your lungs and heart work together during exercise. It's often used to diagnose lung or heart disease.

Overall, Respiratory Function Tests are an important tool for diagnosing and managing a wide range of lung conditions.

TOR (Target Of Rapamycin) Serine-Threonine Kinases are a family of conserved protein kinases that play crucial roles in the regulation of cell growth, proliferation, and metabolism in response to various environmental cues such as nutrients, growth factors, and energy status. They are named after their ability to phosphorylate serine and threonine residues on target proteins.

Mammalian cells express two distinct TOR kinases, mTORC1 and mTORC2, which have different protein compositions and functions. mTORC1 is rapamycin-sensitive and regulates cell growth, proliferation, and metabolism by phosphorylating downstream targets such as p70S6 kinase and 4E-BP1, thereby controlling protein synthesis, autophagy, and lysosome biogenesis. mTORC2 is rapamycin-insensitive and regulates cell survival, cytoskeleton organization, and metabolism by phosphorylating AGC kinases such as AKT and PKCα.

Dysregulation of TOR Serine-Threonine Kinases has been implicated in various human diseases, including cancer, diabetes, and neurological disorders. Therefore, targeting TOR kinases has emerged as a promising therapeutic strategy for the treatment of these diseases.

Pleurodesis is a medical procedure that involves the intentional inflammation and subsequent fusion of the pleural surfaces, which are the thin layers of tissue that separate the lungs from the chest wall. This procedure is typically performed to prevent the recurrence of pneumothorax (a collapsed lung) or pleural effusions (abnormal fluid accumulation in the pleural space).

During the pleurodesis procedure, an irritant such as talc, doxycycline, or silver nitrate is introduced into the pleural space. This causes an inflammatory response, leading to the formation of adhesions between the visceral and parietal pleura. These adhesions obliterate the potential space between the pleural layers, preventing the accumulation of air or fluid within that space.

There are two primary approaches to performing pleurodesis: thoracoscopic (using a video-assisted thoracoscopic surgery or VATS) and chemical (instilling a sclerosing agent through a chest tube). Both methods aim to achieve the same goal of creating adhesions between the pleural layers.

It is essential to note that, while pleurodesis can be an effective treatment for preventing recurrent pneumothorax or pleural effusions, it is not without risks and potential complications. These may include pain, fever, infection, empyema (pus in the pleural space), or acute respiratory distress syndrome (ARDS). Patients should discuss these risks with their healthcare provider before undergoing the procedure.

Kidney neoplasms refer to abnormal growths or tumors in the kidney tissues that can be benign (non-cancerous) or malignant (cancerous). These growths can originate from various types of kidney cells, including the renal tubules, glomeruli, and the renal pelvis.

Malignant kidney neoplasms are also known as kidney cancers, with renal cell carcinoma being the most common type. Benign kidney neoplasms include renal adenomas, oncocytomas, and angiomyolipomas. While benign neoplasms are generally not life-threatening, they can still cause problems if they grow large enough to compromise kidney function or if they undergo malignant transformation.

Early detection and appropriate management of kidney neoplasms are crucial for improving patient outcomes and overall prognosis. Regular medical check-ups, imaging studies, and urinalysis can help in the early identification of these growths, allowing for timely intervention and treatment.

Lung diseases refer to a broad category of disorders that affect the lungs and other structures within the respiratory system. These diseases can impair lung function, leading to symptoms such as coughing, shortness of breath, chest pain, and wheezing. They can be categorized into several types based on the underlying cause and nature of the disease process. Some common examples include:

1. Obstructive lung diseases: These are characterized by narrowing or blockage of the airways, making it difficult to breathe out. Examples include chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, and cystic fibrosis.
2. Restrictive lung diseases: These involve stiffening or scarring of the lungs, which reduces their ability to expand and take in air. Examples include idiopathic pulmonary fibrosis, sarcoidosis, and asbestosis.
3. Infectious lung diseases: These are caused by bacteria, viruses, fungi, or parasites that infect the lungs. Examples include pneumonia, tuberculosis, and influenza.
4. Vascular lung diseases: These affect the blood vessels in the lungs, impairing oxygen exchange. Examples include pulmonary embolism, pulmonary hypertension, and chronic thromboembolic pulmonary hypertension (CTEPH).
5. Neoplastic lung diseases: These involve abnormal growth of cells within the lungs, leading to cancer. Examples include small cell lung cancer, non-small cell lung cancer, and mesothelioma.
6. Other lung diseases: These include interstitial lung diseases, pleural effusions, and rare disorders such as pulmonary alveolar proteinosis and lymphangioleiomyomatosis (LAM).

It is important to note that this list is not exhaustive, and there are many other conditions that can affect the lungs. Proper diagnosis and treatment of lung diseases require consultation with a healthcare professional, such as a pulmonologist or respiratory therapist.

Sirolimus is a medication that belongs to a class of drugs called immunosuppressants. It is also known as rapamycin. Sirolimus works by inhibiting the mammalian target of rapamycin (mTOR), which is a protein that plays a key role in cell growth and division.

Sirolimus is primarily used to prevent rejection of transplanted organs, such as kidneys, livers, and hearts. It works by suppressing the activity of the immune system, which can help to reduce the risk of the body rejecting the transplanted organ. Sirolimus is often used in combination with other immunosuppressive drugs, such as corticosteroids and calcineurin inhibitors.

Sirolimus is also being studied for its potential therapeutic benefits in a variety of other conditions, including cancer, tuberous sclerosis complex, and lymphangioleiomyomatosis. However, more research is needed to fully understand the safety and efficacy of sirolimus in these contexts.

It's important to note that sirolimus can have significant side effects, including increased risk of infections, mouth sores, high blood pressure, and kidney damage. Therefore, it should only be used under the close supervision of a healthcare provider.

Ribosomal Protein S6 (RP S6) is a protein component of the 40S subunit of eukaryotic ribosomes, which are complexes responsible for protein synthesis in cells. Specifically, RP S6 is part of the heterodimer that makes up the head of the 40S subunit.

RP S6 plays a role in regulating translation, the process by which mRNA (messenger RNA) molecules are decoded to produce proteins. It has been found to be involved in the initiation and elongation steps of translation, particularly in response to various cellular signals such as growth factors, hormones, and nutrients.

Phosphorylation of RP S6 is a key regulatory mechanism that modulates its activity during translation. This phosphorylation can be mediated by several kinases, including the p70S6 kinase (p70S6K), which is activated in response to growth factor signaling and nutrient availability.

Abnormalities in RP S6 regulation have been implicated in various diseases, such as cancer, where increased RP S6 phosphorylation has been observed in many tumor types, suggesting a role in promoting cell proliferation and survival.

Lung transplantation is a surgical procedure where one or both diseased lungs are removed and replaced with healthy lungs from a deceased donor. It is typically considered as a treatment option for patients with end-stage lung diseases, such as chronic obstructive pulmonary disease (COPD), cystic fibrosis, idiopathic pulmonary fibrosis, and alpha-1 antitrypsin deficiency, who have exhausted all other medical treatments and continue to suffer from severe respiratory failure.

The procedure involves several steps, including evaluating the patient's eligibility for transplantation, matching the donor's lung size and blood type with the recipient, and performing the surgery under general anesthesia. After the surgery, patients require close monitoring and lifelong immunosuppressive therapy to prevent rejection of the new lungs.

Lung transplantation can significantly improve the quality of life and survival rates for some patients with end-stage lung disease, but it is not without risks, including infection, bleeding, and rejection. Therefore, careful consideration and thorough evaluation are necessary before pursuing this treatment option.

A lung is a pair of spongy, elastic organs in the chest that work together to enable breathing. They are responsible for taking in oxygen and expelling carbon dioxide through the process of respiration. The left lung has two lobes, while the right lung has three lobes. The lungs are protected by the ribcage and are covered by a double-layered membrane called the pleura. The trachea divides into two bronchi, which further divide into smaller bronchioles, leading to millions of tiny air sacs called alveoli, where the exchange of gases occurs.

Lymphatic vessels are thin-walled, valved structures that collect and transport lymph, a fluid derived from the interstitial fluid surrounding the cells, throughout the lymphatic system. They play a crucial role in immune function and maintaining fluid balance in the body. The primary function of lymphatic vessels is to return excess interstitial fluid, proteins, waste products, and immune cells to the bloodstream via the subclavian veins near the heart.

There are two types of lymphatic vessels:

1. Lymphatic capillaries: These are the smallest lymphatic vessels, found in most body tissues except for the central nervous system (CNS). They have blind ends and are highly permeable to allow the entry of interstitial fluid, proteins, and other large molecules.
2. Larger lymphatic vessels: These include precollecting vessels, collecting vessels, and lymphatic trunks. Precollecting vessels have valves that prevent backflow of lymph and merge to form larger collecting vessels. Collecting vessels contain smooth muscle in their walls, which helps to propel the lymph forward. They also have valves at regular intervals to ensure unidirectional flow towards the heart. Lymphatic trunks are large vessels that collect lymph from various regions of the body and eventually drain into the two main lymphatic ducts: the thoracic duct and the right lymphatic duct.

Overall, lymphatic vessels play a vital role in maintaining fluid balance, immune surveillance, and waste removal in the human body.

X-ray computed tomography (CT or CAT scan) is a medical imaging method that uses computer-processed combinations of many X-ray images taken from different angles to produce cross-sectional (tomographic) images (virtual "slices") of the body. These cross-sectional images can then be used to display detailed internal views of organs, bones, and soft tissues in the body.

The term "computed tomography" is used instead of "CT scan" or "CAT scan" because the machines take a series of X-ray measurements from different angles around the body and then use a computer to process these data to create detailed images of internal structures within the body.

CT scanning is a noninvasive, painless medical test that helps physicians diagnose and treat medical conditions. CT imaging provides detailed information about many types of tissue including lung, bone, soft tissue and blood vessels. CT examinations can be performed on every part of the body for a variety of reasons including diagnosis, surgical planning, and monitoring of therapeutic responses.

In computed tomography (CT), an X-ray source and detector rotate around the patient, measuring the X-ray attenuation at many different angles. A computer uses this data to construct a cross-sectional image by the process of reconstruction. This technique is called "tomography". The term "computed" refers to the use of a computer to reconstruct the images.

CT has become an important tool in medical imaging and diagnosis, allowing radiologists and other physicians to view detailed internal images of the body. It can help identify many different medical conditions including cancer, heart disease, lung nodules, liver tumors, and internal injuries from trauma. CT is also commonly used for guiding biopsies and other minimally invasive procedures.

In summary, X-ray computed tomography (CT or CAT scan) is a medical imaging technique that uses computer-processed combinations of many X-ray images taken from different angles to produce cross-sectional images of the body. It provides detailed internal views of organs, bones, and soft tissues in the body, allowing physicians to diagnose and treat medical conditions.

Thoracic surgery, video-assisted (VATS) is a minimally invasive surgical technique used to diagnose and treat various conditions related to the chest cavity, including the lungs, pleura, mediastinum, esophagus, and diaphragm. In VATS, a thoracoscope, a type of endoscope with a camera and light source, is inserted through small incisions in the chest wall to provide visualization of the internal structures. The surgeon then uses specialized instruments to perform the necessary surgical procedures, such as biopsies, lung resections, or esophageal repairs. Compared to traditional open thoracic surgery, VATS typically results in less postoperative pain, shorter hospital stays, and quicker recoveries for patients.

Cathepsin K is a proteolytic enzyme, which belongs to the family of papain-like cysteine proteases. It is primarily produced by osteoclasts, which are specialized cells responsible for bone resorption. Cathepsin K plays a crucial role in the degradation and remodeling of the extracellular matrix, particularly in bone tissue.

This enzyme is capable of breaking down various proteins, including collagen, elastin, and proteoglycans, which are major components of the bone matrix. By doing so, cathepsin K helps osteoclasts to dissolve and remove mineralized and non-mineralized bone matrix during the process of bone resorption.

Apart from its function in bone metabolism, cathepsin K has also been implicated in several pathological conditions, such as osteoporosis, rheumatoid arthritis, and tumor metastasis to bones. Inhibitors of cathepsin K are being investigated as potential therapeutic agents for the treatment of these disorders.

... (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. ... Lymphangioleiomyomatoses are fluid-filled hypodense structures present in the retroperitoneal regions of the abdomen and pelvis ... mTOR inhibitors are effective at shrinking the size of lymphangioleiomyomatosis, and can lead to total resolution. Estrogen- ...
LHCGR Lymphangioleiomyomatosis; 606690; TSC1 Lymphangioleiomyomatosis, somatic; 606690; TSC2 Lymphedema, hereditary I; 153100; ...
Pulmonary lymphangioleiomyomatosis in a man. Am J Respir Crit Care Med 2000; 162(2 pt 1):749-752. Tazelaar HD, Linz LJ, Colby ... Tazelaar was part of the team that described pulmonary lymphangioleiomyomatosis (a disease that occurs almost exclusively in ...
Mutations in TSC2 can cause Lymphangioleiomyomatosis, a disease caused by the enlargement of tissue in the lungs, creating ... Urban T (June 2007). "[Pulmonary lymphangioleiomyomatosis with or without tuberous sclerosis]". Revue des Maladies ...
VEGF-D has also been shown to be over expressed in lymphangioleiomyomatosis and is currently used as a diagnostic biomarker in ... "Diagnostic Potential of Serum VEGF-D for Lymphangioleiomyomatosis". New England Journal of Medicine. 358 (2): 199-200. doi: ...
Evidence of lymphangioleiomyomatosis (LAM) indicates more frequent testing. An mTOR inhibitor drug can help, though a lung ... Cases of TSC-related lymphangioleiomyomatosis recurring following lung transplant have been reported. Small tumours of the ... Surgical treatments for symptoms of lymphangioleiomyomatosis (LAM) in adult TSC patients include pleurodesis to prevent ... known as lymphangioleiomyomatosis (LAM). Recent genetic analysis has shown that the proliferative bronchiolar smooth muscle in ...
... pulmonary lymphangioleiomyomatosis). A pseudo-Darier sign may be present. The renal cell carcinoma tends to be of the papillary ... "Combination of pulmonary lymphangioleiomyomatosis with skin leiomyomatosis and uterine leiomyoma". Arkhiv Patologii. 63 (6): 39 ...
Flieder, D. B.; Travis, W. D. (1997). "Clear cell "sugar" tumor of the lung: Association with lymphangioleiomyomatosis and ... These multifocal lesions are observed in tuberous sclerosis, and can be associated with lymphangioleiomyomatosis and ... No proliferation of immature smooth muscle cells suggestive of lymphangioleiomyomatosis. atypical adenomatous hyperplasia ... "Multifocal micronodular pneumocyte hyperplasia and lymphangioleiomyomatosis in tuberous sclerosis with a TSC2 gene". Modern ...
"Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus Trial (The MILES Trial)". ClinicalTrials.gov (NIH). 6 ... This complication, which only affects women, is now known as lymphangioleiomyomatosis (LAM). 1920 Dutch ophthalmologist Jan van ... Several larger multicentre clinical trials began: lymphangioleiomyomatosis (LAM) and kidney angiomyolipoma (AML) were treated ... lymphangioleiomyomatosis: radiologic-pathologic correlation". Radiographics. 25 (3): 803-28. doi:10.1148/rg.253055006. PMID ...
July 2015). "Surfactant dysfunction and lung inflammation in the female mouse model of lymphangioleiomyomatosis". American ...
They are also commonly found in women with the rare lung disease lymphangioleiomyomatosis. Angiomyolipomas are less commonly ...
Lymphangioleiomyomatosis (LAM) occurs in the lung and may result in pneumothorax, cystic lung destruction and pleural effusions ... "Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex". Clinical ...
When it is determined Spencer's illness, Lymphangioleiomyomatosis, is fatal, Thirteen volunteers to tell her. Later, House asks ...
"Abnormal growth of smooth muscle-like cells in lymphangioleiomyomatosis: Role for tumor suppressor TSC2". American Journal of ...
... recurrent pneumothorax and/or pulmonary cysts can occur with Langerhans cell histiocytosis and lymphangioleiomyomatosis. These ...
... is indicated for the prevention of organ transplant rejection and for the treatment of lymphangioleiomyomatosis (LAM ... In May 2015, the FDA approved sirolimus to treat lymphangioleiomyomatosis (LAM), a rare, progressive lung disease that ... treat a rare lung disease called lymphangioleiomyomatosis, and treat perivascular epithelioid cell tumor (PEComa). It has ... observed with sirolimus in clinical studies for the treatment of lymphangioleiomyomatosis are: peripheral edema, ...
Lymphangioleiomyomatosis is a benign tumour of the smooth muscles of the lymphatics that occurs in the lungs. Lymphoid ...
It is separate and distinct from lymphangiectasis, lymphangioleiomyomatosis (LAM), pulmonary capillary hemangiomatosis, ...
"Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A ...
... potential implications in atherosclerosis and lymphangioleiomyomatosis". Am. J. Pathol. 164 (5): 1575-85. doi:10.1016/S0002- ...
... lymphangioleiomyomatosis, and sarcoidosis. Organ transplant patients, particularly lung, or heart-lung transplant recipients, ...
... lymphangioleiomyomatosis, and, clear-cell myomelanocytic tumour of ligamentum teres/falciform ligament. abdominopelvic sarcoma ... The most common tumours in the PEComa family are renal angiomyolipoma and pulmonary lymphangioleiomyomatosis, both of which are ... although this link is stronger for angiomyolipoma and lymphangioleiomyomatosis than for other members of the PEComa family. ...
In 2006, Carel was diagnosed with lymphangioleiomyomatosis, a very rare life-limiting lung disease, and much of her academic ...
... lymphangioleiomyomatosis, diabetes, and cancer. In particular, the overactivation of mTOR in many cancer types has led to the ...
Lymphangioleiomyomatosis (LAM) Lymphocytic interstitial pneumonia (LIP) Birt-Hogg-Dubé syndrome Pneumocystis pneumonia ...
... information and encouragement to patients with Lymphangioleiomyomatosis (LAM) and their families, and raises funds to advance ...
... lymphangioleiomyomatosis MeSH C04.557.375.480 - lymphangiosarcoma MeSH C04.557.386.345 - histiocytosis, malignant MeSH C04.557. ...
Histiocytosis Oncological Lymphangioleiomyomatosis Environmental, occupational Sarcoid Alternatively: L=Left atrial myxoma ...
... lymphangioleiomyomatosis MeSH C20.683.515.761 - lymphoma MeSH C20.683.515.761.355 - Hodgkin disease MeSH C20.683.515.761.480 - ...
... of which there are several types Sarcoidosis Eosinophilic pneumonia Lymphangioleiomyomatosis Pulmonary Langerhans' cell ...
Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. ... Lymphangioleiomyomatoses are fluid-filled hypodense structures present in the retroperitoneal regions of the abdomen and pelvis ... mTOR inhibitors are effective at shrinking the size of lymphangioleiomyomatosis, and can lead to total resolution. Estrogen- ...
... Chest. 2009 Dec;136(6):1596-1603. doi: 10.1378/chest.09-0624. Epub ... Background: We previously reported that approximately one-fourth of patients with lymphangioleiomyomatosis (LAM) may respond to ...
Lymphangioleiomyomatosis (LAM) is a rare disorder resulting from proliferation in the lung, kidney, and axial lymphatics of ... encoded search term (Lymphangioleiomyomatosis) and Lymphangioleiomyomatosis What to Read Next on Medscape ... Lymphangioleiomyomatosis. Updated: Dec 22, 2019 * Author: Joel Moss, MD, PhD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more ... Lymphangioleiomyomatosis. Clinical course in 32 patients. N Engl J Med. 1990 Nov 1. 323(18):1254-60. [QxMD MEDLINE Link]. ...
... J Heart Lung Transplant. 2005 Sep;24(9):1247 ... Lung transplantation has been increasingly used as a treatment modality for patients with pulmonary lymphangioleiomyomatosis ( ...
Lymphangioleiomyomatosis (LAM) is a rare disorder resulting from proliferation in the lung, kidney, and axial lymphatics of ... encoded search term (Lymphangioleiomyomatosis) and Lymphangioleiomyomatosis What to Read Next on Medscape ... Lymphangioleiomyomatosis. Updated: Dec 22, 2019 * Author: Joel Moss, MD, PhD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more ... Lymphangioleiomyomatosis. Clinical course in 32 patients. N Engl J Med. 1990 Nov 1. 323(18):1254-60. [QxMD MEDLINE Link]. ...
... which eventually revealed a diagnosis of lymphangioleiomyomatosis (LAM). Successful referral to the National LAM Centre in ...
Lymphangioleiomyomatosis (LAM) Clinic Lymphangioleiomyomatosis (LAM) is a rare condition affecting women, with typical onset ...
Krymskaya, a preclinical rare disease researcher focused pulmonary lymphangioleiomyomatosis (LAM) research ... Q: Tell us a little bit about the lam, or lymphangioleiomyomatosis, and your genetic preclinical model. A: Prior to my research ... Vera Krymskaya, a scientist at the forefront of preclinical lymphangioleiomyomatosis (LAM) and rare lung disease research, has ... and now lymphangioleiomyomatosis (LAM) has been my labs primary focus for the past several years. ...
Lymphangioleiomyomatosis - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the MSD Manuals - Medical ... Etiology of Lymphangioleiomyomatosis The cause of lymphangioleiomyomatosis is unknown. The tempting hypothesis that female sex ... Prognosis for Lymphangioleiomyomatosis Prognosis for lymphangioleiomyomatosis is unclear because the disorder is so rare and ... Symptoms and Signs of Lymphangioleiomyomatosis Initial symptoms of lymphangioleiomyomatosis are dyspnea and, less commonly, ...
Lymphangioleiomyomatosis (LAM) is a rare disorder resulting from proliferation in the lung, kidney, and axial lymphatics of ... encoded search term (Lymphangioleiomyomatosis) and Lymphangioleiomyomatosis What to Read Next on Medscape ... Associations between lymphangioleiomyomatosis (LAM) and tuberous sclerosis complex (TSC) are as follows:. * TSC is a ... Lymphangioleiomyomatosis Differential Diagnoses. Updated: Jul 27, 2015 * Author: Joel Moss, MD, PhD; Chief Editor: Zab ...
The advent of pharmacological therapies for lymphangioleiomyomatosis (LAM) has made early diagnosis important in women with ... The advent of pharmacological therapies for lymphangioleiomyomatosis (LAM) has made early diagnosis important in women with ... Lung Function and Radiological Features in a Genotyped Population Investigated for Lymphangioleiomyomatosis. F. Di Marco. Primo ... Lung Function and Radiological Features in a Genotyped Population Investigated for Lymphangioleiomyomatosis / F. Di Marco, S. ...
Lymphangioleiomyomatosis (LAM) is a rare disorder resulting from proliferation in the lung, kidney, and axial lymphatics of ... encoded search term (Lymphangioleiomyomatosis) and Lymphangioleiomyomatosis What to Read Next on Medscape ... Lymphangioleiomyomatosis. Updated: Jul 27, 2015 * Author: Joel Moss, MD, PhD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more ... Lymphangioleiomyomatosis (LAM) primarily is a disease of women; however, rare case reports describe LAM in men with TSC. ...
UT Southwestern lung specialists provide expert diagnosis and treatment for lymphangioleiomyomatosis, a rare lung disease that ... Specialized Treatment for Lymphangioleiomyomatosis (LAM). Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease ... The UT Southwestern Medical Center Lymphangioleiomyomatosis (LAM) Clinic is one of 33 LAM Foundation clinical sites in the ... For more information about lymphangioleiomyomatosis, visit thelamfoundation.org. Clinical Trials. Our LAM Clinic is currently ...
Get natural cures for Lymphangioleiomyomatosis that can make a difference in your life or the life of someone you love with ... Lymphangioleiomyomatosis natural cures. Lymphangioleiomyomatosis Definition. Lymphangioleiomyomatosis, LMA for brevity, is a ... Lymphangioleiomyomatosis in North Carolina. Lymphangioleiomyomatosis in North Dakota. Lymphangioleiomyomatosis in Ohio. ... Lymphangioleiomyomatosis by state. Lymphangioleiomyomatosis in Alabama. Lymphangioleiomyomatosis in Alaska. ...
From: Natural history of angiomyolipoma in lymphangioleiomyomatosis: implications for screening and surveillance ...
Pulmonary lymphangioleiomyomatosis and bilateral renal angiomyolipomas are some presentations of tuberous sclerosis and the ... small thin-walled cysts scattered throughout the lungs characteristic for pulmonary lymphangioleiomyomatosis. Computed ... From: Computed tomographic findings of tuberous sclerosis with pulmonary lymphangioleiomyomatosis and renal angiomyolipomas: a ...
N2 - We present two cases of pulmonary lymphangioleiomyomatosis and one case of tuberous sclerosis with pulmonary involvement, ... AB - We present two cases of pulmonary lymphangioleiomyomatosis and one case of tuberous sclerosis with pulmonary involvement, ... We present two cases of pulmonary lymphangioleiomyomatosis and one case of tuberous sclerosis with pulmonary involvement, ... abstract = "We present two cases of pulmonary lymphangioleiomyomatosis and one case of tuberous sclerosis with pulmonary ...
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Lymphangioleiomyomatosis (LAM) is a progressive cystic lung disease affecting some ladies. Lymphangioleiomyomatosis (LAM) is a ... Lymphangioleiomyomatosis (LAM) a pulmonary manifestation of TSC (2) is definitely a progressive cystic lung disease influencing ...
What Is LAM (Lymphangioleiomyomatosis)? (National Heart, Lung, and Blood Institute) Also in Spanish ... Lymphangioleiomyomatosis: MedlinePlus Genetics (National Library of Medicine) * Primary ciliary dyskinesia: MedlinePlus ...
Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease of the lung and lymphatic system that primarily affects women of ... Sporadic Lymphangioleiomyomatosis Disease: A Case Report. Nikmanesh, Yousef; Shokripour, Mansoureh; Mokhtari, Maral; Khazayi, ...
NCTC it says the best download the official patient\s sourcebook on lymphangioleiomyomatosis: a to treat. Q: How not be ebooks ... The download the official patient\s sourcebook on lymphangioleiomyomatosis: a of box in Europe has even approved that in the ... We are not given any download the official patient\s sourcebook on lymphangioleiomyomatosis: a revised and on this jacket! We ... Xiang YZ, Shang HC, Gao XM, Zhang BL: A download the official patient\s sourcebook on lymphangioleiomyomatosis: a revised of ...
Lymphangioleiomyomatosis Peritoneovenous shunt Chylous ascites Venous thrombosis Warfarin Received 14 Mar 2012 / Accepted 27 ... A 38-year-old female was admitted because of lymphangioleiomyomatosis (LAM)-associated massive chylous ascites and progressive ... Long-term efficacy of a peritoneovenous shunt for chylous ascites complicated with lymphangioleiomyomatosis ...
... and rare forms of ILD such as lymphangioleiomyomatosis (LAM) or Langerhans cell histiocytosis (LCH) account for the remaining ...
The purpose of the present study was to evaluate the lung volume after single lung transplantation for lymphangioleiomyomatosis ... The purpose of the present study was to evaluate the lung volume after single lung transplantation for lymphangioleiomyomatosis ... The purpose of the present study was to evaluate the lung volume after single lung transplantation for lymphangioleiomyomatosis ... The purpose of the present study was to evaluate the lung volume after single lung transplantation for lymphangioleiomyomatosis ...
  • Lung transplantation has been increasingly used as a treatment modality for patients with pulmonary lymphangioleiomyomatosis (LAM). (nih.gov)
  • The Krymskaya lab focuses on preclinical and translational research in rare and common lung diseases from pulmonary lymphangioleiomyomatosis (LAM) to asthma. (scireq.com)
  • Immunohistochemical study of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) in pulmonary lymphangioleiomyomatosis (LAM). (medscape.com)
  • Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score. (medscape.com)
  • Pulmonary lymphangioleiomyomatosis (LAM): examining oral contraceptive pills and the onset of disease. (medscape.com)
  • Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis. (medscape.com)
  • We present two cases of pulmonary lymphangioleiomyomatosis and one case of tuberous sclerosis with pulmonary involvement, describing the most characteristic features according to plain chest X-ray and high-resolution computed tomography (HRCT). (elsevierpure.com)
  • Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease of the lung and lymphatic system that primarily affects women of childbearing age. (bvsalud.org)
  • Pulmonary lymphangioleiomyomatosis (LAM) is a rare lymphatic disease that affects 5-9 million women of child-bearing age. (tamhsc.edu)
  • This prompted a referral to the specialist team where further investigations were undertaken including a high-resolution CT scan followed by lung biopsy, which eventually revealed a diagnosis of lymphangioleiomyomatosis (LAM). (bmj.com)
  • Diagnosis of lymphangioleiomyomatosis is suspected in young women with dyspnea plus interstitial changes with normal or increased lung volumes on chest x-ray, spontaneous pneumothorax, or chylous effusion. (msdmanuals.com)
  • Purpose :Whereas native lung overinflation has been thought to happen in recipients of single lung transplantation for lymphangioleiomyomatosis because of its increased compliance, there is no study that has reported the details on the change of the native lung volume after single lung transplantation by three-dimensional computed tomography volumetry. (elsevierpure.com)
  • The purpose of the present study was to evaluate the lung volume after single lung transplantation for lymphangioleiomyomatosis by three-dimensional computed tomography volumetry and investigate the correlation between the native lung volume change and postoperative pulmonary function. (elsevierpure.com)
  • Progesterone and estradiol synergistically promote the lung metastasis of tuberin-deficient cells in a preclinical model of lymphangioleiomyomatosis. (uc.edu)
  • We previously reported that approximately one-fourth of patients with lymphangioleiomyomatosis (LAM) may respond to therapy with bronchodilators. (nih.gov)
  • The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. (medscape.com)
  • Although primarily a disease of women of childbearing age, lymphangioleiomyomatosis (LAM) has also been reported in postmenopausal patients. (medscape.com)
  • Methods: We retrospectively reviewed the data of 17 patients who underwent single lung transplantation for lymphangioleiomyomatosis. (elsevierpure.com)
  • Lymphangioleiomyomatosis Mortality in Patients with Tuberous Sclerosis Complex. (tamhsc.edu)
  • The LAM Foundation has approved the Norton Thoracic Institute as a treatment center providing exemplary care for patients with lymphangioleiomyomatosis (LAM). (dignityhealth.org)
  • Rapamune is also used for treating patients with sporadic lymphangioleiomyomatosis (S-LAM) with moderate lung disease or worsening lung function. (europa.eu)
  • Lymphangioleiomyomatosis (LAM) is a progressive cystic lung disease affecting some ladies with tuberous sclerosis complex (TSC). (crispr-reagents.com)
  • Lymphangioleiomyomatosis (LAM) a pulmonary manifestation of TSC (2) is definitely a progressive cystic lung disease influencing primarily ladies of childbearing age. (crispr-reagents.com)
  • BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. (bvsalud.org)
  • Sporadic Lymphangioleiomyomatosis Disease: A Case Report. (bvsalud.org)
  • A 38-year-old female was admitted because of lymphangioleiomyomatosis (LAM)-associated massive chylous ascites and progressive cachexia. (or.jp)
  • Lung cysts and surrounding tissues in a patient with lymphangioleiomyomatosis (LAM) seen more clearly using high-performance low field MRI compared with standard MRI. (medgadget.com)
  • The team found that they were able to detect lung cysts and the surrounding anatomy in those with lymphangioleiomyomatosis. (medgadget.com)
  • The UT Southwestern Medical Center Lymphangioleiomyomatosis (LAM) Clinic is one of 33 LAM Foundation clinical sites in the nation and one of only two in Texas. (utswmed.org)
  • Rapamycin-resistant poly (ADP-ribose) polymerase-1 overexpression is a potential therapeutic target in lymphangioleiomyomatosis. (uc.edu)
  • Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. (wikipedia.org)
  • Lymphangioleiomyomatosis (LAM) is a rare lung disease, resulting from proliferation in the lung, kidney, and axial lymphatics of abnormal smooth muscle-like cells (LAM cells) that exhibit features of neoplasia and neural crest origin. (medscape.com)
  • Learn what Dr. Vera Krymskaya, a scientist at the forefront of preclinical lymphangioleiomyomatosis (LAM) and rare lung disease research, has to say about the current state and future of the field. (scireq.com)
  • I then learned about a rare genetic lung disease that had abnormal smooth muscle-like lesions and used LAM to study how abnormal smooth muscle grow, and now lymphangioleiomyomatosis (LAM) has been my lab's primary focus for the past several years. (scireq.com)
  • Lymphangioleiomyomatosis (LAM) is a rare disease typically affecting women between 20 and 40 years. (msdmanuals.com)
  • Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease that affects primarily women, usually ages 20-40 and during their childbearing years. (utswmed.org)
  • Lymphangioleiomyomatosis (LAM) is a rare type of lung disease that occurs almost exclusively in women. (nih.gov)
  • Lymphangioleiomyomatosis (LAM) is a rare lung disease recognized by abnormal growth of smooth muscle cells proliferating in lungs parenchyma, developing benign tumors, migrating to the other organs, and ultimately leading to respiratory failure and death. (waldenu.edu)
  • Insulin growth factor binding protein 2 mediates the progression of lymphangioleiomyomatosis. (uc.edu)
  • Earlier reports indicated a grim prognosis for lymphangioleiomyomatosis (LAM), with progressive respiratory failure and death within 10 years of diagnosis. (medscape.com)
  • Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. (tamhsc.edu)
  • Lymphangioleiomyomatosis (LAM) is an indolent, progressive growth of smooth muscle cells throughout the lungs, pulmonary blood vessels, lymphatics, and pleurae. (msdmanuals.com)
  • Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. (medscape.com)
  • Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. (medscape.com)
  • Johnson SR, Taveira-DaSilva AM, Moss J. Lymphangioleiomyomatosis. (tamhsc.edu)
  • Lymphangioleiomyomatosis (LAM) is a rare condition affecting women, with typical onset early in adulthood but often with delayed diagnosis. (rochester.edu)
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  • 2014), the download the official patient\'s sourcebook on lymphangioleiomyomatosis: a revised and updated trait appetite was eliminated to complete requiring the featured Unexplained libraries from the brown. (peterkim.com)
  • The cause of lymphangioleiomyomatosis is unknown. (msdmanuals.com)
  • The international frequency of lymphangioleiomyomatosis (LAM) is unknown, although Europe and Japan report case series. (medscape.com)
  • Pelvic lymphangioleiomyomatosis treated successfully with everolimus: Two case reports with literature review. (tamhsc.edu)
  • 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. (unimi.it)
  • Lymphangioleiomyomatosis can be associated with micronodular type II pneumocyte hyperplasia, particularly in individuals with tuberous sclerosis. (medscape.com)
  • Lymphangioleiomyomatosis (LAM) may occur sporadically or in association with tuberous sclerosis complex (TSC). (medscape.com)
  • Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. (medscape.com)