The benign, non-invasive lesions of tuberous sclerosis can appear in any organ like the brain, heart, skin, eyes, kidney, lung, and liver. Therefore, TS has a wide clinical spectrum. The diagnosis of definitive TS is based on specific clinical features and requires the presence of two major criteria, or one major and two minor [3]. Pulmonary lymphangioleiomyomatosis, renal angiomyolipoma and facial angiofibroma are some of the major clinical features.. The most frequent cause of death in patients with TS is renal complication [3, 5]. Multifocal, bilateral angiomyolipomas are found in about 70-90% of adult patients [3], and the prevalence increases with age, being less frequent in children [3, 4]. These lesions are more often prevalent in women, suggesting a hormonal component to the tumor growth [6]. The angiomyolipomas are composed of varying amounts of mature adipose tissue, smooth muscle, and abnormal blood vessels [3, 6]. The demonstration of intratumoral fat with negative attenuation values ...
Lymphangioleiomyomatosis: a case report and review of diagnosis and treatment Yi Liu,* Zhibin Guo,* Chenlong Zhao, Xin Li, Hongyu Liu, Jun Chen Department of Lung Cancer Surgery, Tianjin Key Laboratory of Lung Cancer Metastasis and Tumor Microenvironment, Tianjin Lung Cancer Institute, Tianjin Medical University General Hospital, Tianjin 300052, China *These authors contributed equally to this work Abstract: 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). This disease is rare in males. It is hard to distinguish between lung cancer and pulmonary LAM, especially during early stages. Herein, we present a case of a 66-year-old man with a small nodule in the right upper lobe that was first diagnosed as a lung malignancy using a chest CT scan. After a wedge dissection, a pathologist performed a histologic and
Lymphangioleiomyomatosis (LAM) is a low-grade neoplasm characterized by the pulmonary infiltration of smooth muscle-like cells (LAM cells) and cystic destruction. Patients usually present with airway obstruction in pulmonary function tests (PFTs). Previous studies have shown correlations among histological parameters, lung function abnormalities and prognosis in LAM. We investigated the lung tissue expression of proteins related to the mTOR pathway, angiogenesis and enzymatic activity and its correlation with functional parameters in LAM patients. We analyzed morphological and functional parameters of thirty-three patients. Two groups of disease severity were identified according to FEV1 values. Lung tissue from open biopsies or lung transplants was immunostained for SMA, HMB-45, mTOR, VEGF-D, MMP-9 and D2-40. Density of cysts, density of nodules and protein expression were measured by image analysis and correlated with PFT parameters. There was no difference in the expression of D2-40 between the more
Lymphangioleiomyomatosis (LAM) is a rare, low-grade multisystem neoplastic disease. Most LAM patients are at a high risk of losing lung function at an accelerated rate and developing progressive dyspnea. Recently, several studies have reported their experience with pharmacological treatments for LAM. Therefore, we conducted a systematic review and meta-analysis to assess the efficacy and safety of these therapies. PubMed (Medline), EMBASE, Cochrane Library, Web of Science and EBSCO Host were searched (until March 31, 2019) for eligible prospective studies regarding LAM patients treated with pharmacological treatments. Random effect models were used for quantitative analysis. Fourteen prospective studies regarding five pharmacological treatments (including sirolimus, everolimus, doxycycline, triptorelin, and a combination therapy of sirolimus and hydroxychloroquine) were enrolled in our systematic review, and ten of them were used for the meta-analysis. Seven prospective studies reported that sirolimus
TY - JOUR. T1 - Rapid growth and regression of intracranial meningiomas in lymphangioleiomyomatosis. T2 - case report. AU - Pozzati, Eugenio. AU - Zucchelli, Mino. AU - Schiavina, Mario. AU - Contini, Paola. AU - Foschini, Maria Pia. PY - 2007/12. Y1 - 2007/12. N2 - Background: Lymphangioleiomyomatosis is a progressive interstitial lung disease that affects young women. It has been suggested that estrogens play a role in its evolution, and progesterone therapy is often provided in these cases. Case Description: We present a case of a postmenopausal woman with LAM treated with progesterone; subsequently, rapid growth of multiple intracranial meningiomas was observed. One prominent lesion was excised, and 3 other lesions regressed spontaneously over 2 years. Conclusions: This is a rare case of a non-pregnancy-related regression of meningiomas in a woman affected by LAM. The significance of this association and the hormonal treatment of the disease are discussed.. AB - Background: ...
The purpose of the study is to test if the drug doxycycline is effective in slowing the progression of lung disease in LAM. Lymphangioleiomyomatosis (LAM) is a rare lung disease which affects young women. Women with LAM develop enlarged air spaces in the lungs called cysts, caused by an excess of matrix metalloproteinases (MMPs), protein-digesting enzymes. LAM is associated with kidney tumours, called angiomyolipomas, and causes recurrent lung collapse, breathlessness and death or need for lung transplant. There is no proven treatment. Doxycycline, a commonly used antibiotic can block MMP production and a small number of patients have shown some benefit from doxycycline. The investigators will perform a study to test if doxycycline can slow the fall in lung function in patients with LAM. Forty patients who consent to participate will take doxycycline or a placebo (dummy) tablet for two years in addition to their standard treatment ...
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. Two-thirds of patients suffer pneumothoraces. Hormonal therapy with progesterone or tamoxifen is usually given for progressive disease; medical or surgical pleurodesis is advisable; lung transplantation is the main option for advanced disease....
Relevant aspects of lymphangioleiomyomatosis: almost exclusively affects women of child-bearing age it is a multi-system disorder and can affect many organs recurrent pneumothorax can occur in up to 80% of cases in the lungs it manifests as m...
LAM also occurs in patients who do not have TSC. This form, termed "sporadic LAM" or "S-LAM," is diagnosed in at least 2.5-5 per million women, or roughly 10,000 women worldwide, although it is likely that S-LAM is substantially underdiagnosed. To date, only one biopsy-documented case of S-LAM in a male has been reported. Although less common, most patients seen in the clinic have S-LAM rather than TSC-LAM.. In LAM, lung destruction occurs as a result of neoplastic proliferation of benign-appearing smooth muscle cells in the lung. Genetic evidence indicates that the LAM cells that infiltrate the lung arise from an unknown extrapulmonary source and spread via the bloodstream and lymphatics. Leading candidates for the site of origin include the uterus, renal angiomyolipomas, and lymphatics. LAM cells express the lymphangiogenic growth factors VEGF-C and VEGF-D and induce disordered lymphatic channel formation in the lung and lymphatics. "Frustrated lymphangiogenesis" likely contributes to the ...
Differential diagnosis of multiple lung cysts on HRCT: Lymphangioleiomyomatosis, emphysema (cysts and walls usually less well-defined), chronic interstitial lung diseases (honeycombed cysts usually peripheral), Langerhans cell histiocytosis (nodules ± cavities, presence of thicker-walled cysts, upper lung predominance), bronchiectasis (tree-and-bud pattern often present), sarcoidosis (cysts usually apical), and pneumatoceles (commonly seen with pneumocystis pneumonia) Histologic differential diagnosis: Lymphangioleiomyomatosis; emphysema (if smooth muscle nodules not recognized); smooth muscle proliferations such as "benign" metastasizing leiomyoma, metastatic stromal sarcoma of the uterus, and metaplastic muscle bundles in fibrosing lung diseases; and alveolar hemorrhage syndromes (if hemosiderin deposits frequent) Diagnosis: Lymphangioleiomyomatosis. Diagnostic features of lymphangioleiomyomatosis on HRCT. ...
... Classification & external resources CT scan of a lung with LAM. ICD-O: 9174/1 OMIM 606690 DiseasesDB 30755
TY - JOUR. T1 - Distinguishing between lymphangioleiomyomatosis and carcinomatous peritonitis in a patient with ovarian cancer. AU - Hirasawa, Akira. AU - Sato, Takashi. AU - Ueno, Mari. AU - Akahane, Tomoko. AU - Susumu, Nobuyuki. AU - Betsuyaku, Tomoko. AU - Aoki, Daisuke. PY - 2013/10/1. Y1 - 2013/10/1. UR - http://www.scopus.com/inward/record.url?scp=84891522232&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84891522232&partnerID=8YFLogxK. U2 - 10.1200/JCO.2012.45.3019. DO - 10.1200/JCO.2012.45.3019. M3 - Short survey. C2 - 23752115. AN - SCOPUS:84891522232. VL - 31. SP - e427-e429. JO - Journal of Clinical Oncology. JF - Journal of Clinical Oncology. SN - 0732-183X. IS - 28. ER - ...
TY - JOUR. T1 - Distinguishing between lymphangioleiomyomatosis and carcinomatous peritonitis in a patient with ovarian cancer. AU - Hirasawa, Akira. AU - Sato, Takashi. AU - Ueno, Mari. AU - Akahane, Tomoko. AU - Susumu, Nobuyuki. AU - Betsuyaku, Tomoko. AU - Aoki, Daisuke. PY - 2013/10/1. Y1 - 2013/10/1. UR - http://www.scopus.com/inward/record.url?scp=84891522232&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84891522232&partnerID=8YFLogxK. U2 - 10.1200/JCO.2012.45.3019. DO - 10.1200/JCO.2012.45.3019. M3 - Short survey. C2 - 23752115. AN - SCOPUS:84891522232. VL - 31. SP - e427-e429. JO - Journal of Clinical Oncology. JF - Journal of Clinical Oncology. SN - 0732-183X. IS - 28. ER - ...
The purpose of this medical research study is to evaluate the safety and effectiveness of a new medication called imatinib mesylate in the treatment of Lymphangioleiomyomatosis (LAM). LAM is a rare disease in which abnormal cells (called LAM cells) grow out of control. Over time, LAM cells destroy healthy lung tissue and cause respiratory disease or failure. Many patients with LAM are currently treated with a medication called sirolimus (rapamycin). Sirolimus slows the growth of LAM cells. Imatinib mesylate (hereafter called imatinib) is approved by the Food and Drug Administration (FDA) for the treatment of some cancers that share common pathways with LAM cells. Laboratory studies suggest that imatinib could completely block the growth of LAM cells through initiation of targeted cell death.. An important purpose of this research is to determine the safety of imatinib in people with LAM. This study will also evaluate the short-term effectiveness of imatinib. Participants will be randomized to ...
A brief introduction to radiographs and chest CT scans for the diagnosis of cystic lung diseases.  Lymphangioleiomyomatosis (LAM) is the focus – including diagnosis of the disease and its complications.  Other cystic lung diseases such as Langerhans Histiocytosis, Birt-Hogg-Dube and Lymphocytic Interstitial Pneumonia (LIP) will also be mentioned.
LAM is a neoplastic disorder in which smooth muscle-like cells with biallelic inactivation of TSC2 leads to hyperactivation of mTORC1. This fundamental mechanistic insight led to the recent reported success in treating LAM patients with rapamycin. However, mTORC1-independent effects of TSC2 loss are also known (Yu and Henske, 2010; Neuman and Henske, 2011). The marked female predominance of LAM suggests that estradiol promotes disease progression. In this study, we identified an estradiol-induced prostaglandin metabolic signature in TSC2-deficient ELT3 cells in vitro and in vivo. Prostaglandins, synthesized via COX-1/COX-2, appear to play important roles in cancer progression (FitzGerald and Patrono, 2001; Müller, 2004; Wang et al., 2007; Wang and Dubois, 2010), although their precise role is still hotly debated. Estradiol-dependent prostaglandin production has been studied in other models. Estradiol activated COX-2 and increased prostacyclin synthesis in mouse aortic smooth muscle cells (Egan ...
Home spirometry is being added as a secondary end point in a lymphangioleiomyomatosis (LAM) trial soon to begin enrollment at the University of Cincinnati (UC)
Lymphangioleiomyomatosis or LAM is a rare lung disease that occurs only in women and nearly always starts before the menopause. LAM mainly affects the lungs, where an abnormal type of cell (called a LAM cell) builds up around the airways and the lymph vessels. The LAM cells lead to cysts developing in the lung. One of the main symptoms is breathlessness due to the effect of the cells on the airways and also to the cysts which take up space in the lungs. Click here for more information.. ...
To date, this study is the largest air travel survey of women with LAM, and provides some revealing, albeit retrospective, data on the experiences of air travel in women with LAM. It should be noted, however, that women who were waitlisted for or who had undergone lung transplantation for end-stage disease were excluded from this study, therefore findings may not generalise to women with more advanced disease. Nonetheless, the group of women who travelled by air had, on average, better dyspnoea scores than those who did not, and there was a wide range in values of dyspnoea scores for both women who flew and those who did not. Therefore, the participants include women with LAM who had differing severities of disease, thereby affirming the generalisability of our results.. Although hypoxaemia-related problems such as dyspnoea and chest pain occurred during air travel in women with LAM, these results yield an approximate risk of 2% for pneumothorax. Although the risk of pneumothorax is small, it is ...
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Lu,C.; Lee,H. S.; Pappas,G. P.; Dilling,D. F.; Burger,C. D.; Shifren,A.; Veeraraghavan,S.; Chapman,J. T.; Parambil,J.; Ruoss,S. J.; Young,L. R.; Hammes,S. R.; Kopras,E. J.; Roads,T.; Krischer,J. P.; McCormack,F. X.; Trial of an Aromatase Inhibitor in Lymphangioleiomyomatosis GroupA Phase II Clinical Trial of an Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis Annals of the American Thoracic Society 2017 ;14(6):919- ...
Guest contributor. What if you were suddenly diagnosed with a potentially fatal disease just when your life, work and marriage were on track and your plans to start a family were underway?. Thats what happened to Sue R. Levy.. In 2008, at age 37, she was diagnosed with Pulmonary Lymphangioleiomyomatosis, otherwise known as LAM, a rare, chronic, progressive lung disease in which the lungs fill up with cysts. The result is gradual destruction of the normal lung architecture, compromised breathing and, in many cases, an eventual lung transplant - a procedure with major risks. The LAM Foundation reports 10-year survival, following a lung transplant, at 47 percent.. Fueled by estrogen, LAM primarily affects women in their childbearing years. With only 1,300 documented cases in North America, LAM is poorly understood; currently, there are a few experimental medications in use, but no proven treatments exist.. Prior to the diagnosis, Sue, who lives in Brookline, Mass., had a successful career as a ...
CHICAGO, IL--(Marketwired - Mar 23, 2015) - About 1,400 American women and some 3,000 globally have been diagnosed with LAM or Lymphangioleiomyomatosis, a progressive and rare deadly lung disease which affects women during their child bearing years. One in 10 Americans is affected by a rare disease, and there are...
Doctors who treat patients with a severe and progressive respiratory disease called lymphangioleiomyomatosis (LAM) can face an agonizing treatment decision.
The statins, as pharmacologic inhibitors of HMG-CoA reductase, have a wide array of biological effects including antiproliferative and anticancer properties (17, 35-37). Our results indicate that atorvastatin, given at near-maximal dosage with major effects on both total and free serum cholesterol, had little or no effect on the size of ENU-accelerated or spontaneous tumors in Tsc2+/− mice. This study was prompted by in vivo observations that atorvastatin was highly selective and effective at inhibiting the growth of cells lacking Tsc2 (26). Although no murine model perfectly replicates the tumor spectrum seen in TSC and LAM patients, we chose to test this therapy in a Tsc2+/− model where tumors develop de novo to more closely parallel human TSC and LAM. LAM cells in human lung and tumors that develop in both human TSC and Tsc2+/− mice have all been shown to bear markers of Rheb-mTORC1-S6K activation (31, 38, 39), and growth of LAM lesions and TSC-associated tumors is relatively indolent. ...
The TSC1-TSC2 complex has emerged as a central signal-integrating node within the cell. Mutations in the tumor suppressor genes encoding TSC1 and TSC2 give rise to a multisystemic tumor syndrome called tuberous sclerosis complex (TSC), which is characterized by widespread dysplastic and neoplastic lesions (i.e., hamartomas) (reviewed in reference 7). TSC2 mutations are also found in sporadic cases of lymphangioleiomyomatosis (LAM), which is characterized by aberrant smooth muscle cell proliferation and cystic destruction in the lung (5). In addition, the TSC1-TSC2 complex lies downstream of numerous oncogenes (e.g., RTKs, phosphatidylinositol 3-kinase [PI3K]-Akt, and Ras) and tumor suppressors (e.g., PTEN, LKB1, and NF1) and, therefore, is predicted to be functionally altered by posttranslational modifications in the majority of human cancers (11, 17).. Within the TSC1-TSC2 complex, TSC1 stabilizes TSC2 (3, 6), while TSC2 acts as a GTPase-activating protein (GAP) for the small GTPase Rheb (Ras ...
Pulmonary involvement manifests as cystic lung disease and spontaneous pneumothorax. The cysts are variable in shape and size, but are discrete and thin-walled. Larger cysts are usually multiseptated. The cysts tend to be larger and more numerous in the lower lobes, a distribution that can help differentiate Birt-Hogg-Dubé syndrome from other causes of multiple cystic lung lesions such as Langerhans cell histiocytosis and lymphangioleiomyomatosis (see below ...
Message from Frank McCormack, MD, Division Director of Pulmonary, Critical Care & Sleep Medicine. Dear Colleague:. Greetings from the University of Cincinnati Medical Center Division of Pulmonary, Critical Care, & Sleep Medicine. Starting in 2015, we are pleased to share Pulmonary Insights, an educational resource with information on our ongoing research in rare lung disease, as well as innovative diagnostics and therapeutics that have resulted. Our goal is to collaborate with our peers, using Pulmonary Insights as one of many ways to create a dialogue with you - fellow physicians who seek the best ways to care for patients.. Research in lymphangioleiomyomatosis (LAM) has been particularly rewarding. We studied the safety and efficacy of the drug sirolimus (rapamycin) in an NIH-funded randomized controlled trial called MILES based at our academic health center and conducted by physician scientists here and around the world. Since the New England Journal of Medicine published the findings in ...
SUMMIT REPORT. Fifty seven researchers,clinicians, patients and patient family members representing seventeen countries met in Oxford UK in January.. Bronwyn Gray represented both NZ and Australian LAM patients and their families in her capacity as Vice President of LARA (LAM Australasia Research Alliance) and as Director of the New Zealand LAM Trust.. The aim of the Summit, which was a follow on from the meeting hosted by the LAM Treatment Alliance in Stockholm February 2007, was to involve patients in progress towards finding a treatment for LAM. Discussion re collaboration and coordinated efforts to make the fastest possible progress in the quest for a treatment for LAM dominated the meeting.. There was much discussion on establishing ways in which patients can support and facilitate research. It was agreed that the most limiting factor for research is not lack of interest from researchers or lack of money - but access to LAM tissue and LAM patient data.. Scientists are often deterred by the ...
This is a report of the June 11, 2012 American Thoracic Society Public Advisory Roundtable Meeting that Karen Deitemeyer attended in Washington, DC, on behalf of EFFORTS. If you do not have time to read the entire report, please be sure to read the four Q & A at the end. Dr. Tony Punturieri, Program Director, Division of Lung Diseases at the NHLBI answered questions provided by EFFORTS members .. There were 21 people present, representing the ATS staff and staff or patients from various lung disease patient organizations. The attendees with COPD groups were Karen Deitemeyer, representing EFFORTS, Vlady Rosenbaum from COPD-ALERT and Miriam O Day, representing the Alpha-1 Foundation.. Other attendees represented the Hermansky-Pudlak Syndrome Network, Inc; the Asthma and Allergy Foundation of America; the Lymphangiomatosis and Gorham s Disease Alliance; the Pulmonary Hypertension Association, Inc.; the LAM Foundation (lymphangioleiomyomatosis); the Coalition for Pulmonary Fibrosis; the Children s ...
Insulin secretory function and survival of pancreatic β cells are intimately linked to nutrient availability and its sensing (Efeyan et al., 2015). Here, we report that mice lacking mTOR, a common nutrient-sensing component of mTORC1 and mTORC2, in β cells exhibited glucose intolerance, insulin resistance, a decrease in β cell mass, and an increase in β cell death. These results suggest that impaired glucose homeostasis is connected to a defect in β cell survival caused by mTOR deficiency. On the other hand, TSC2 deficiency in β cells, which leads to constitutive activation of mTORC1 signaling, results in a decrease in blood glucose levels, hyperinsulinemia, and improved glucose tolerance, which is the opposite phenotype of mTOR-deficient mice (Rachdi et al., 2008). Conversely, rapamycin and other mTORC1 inhibitors such as sirolimus, everolimus, and zatarolimus have been used to treat chronic diseases like cancer, hyperinsulinism, tuberous sclerosis complex, and lymphangioleiomyomatosis ...
Includes angiomyolipoma (AML), clear cell sugar tumor of lung (CCST), lymphangioleiomyomatosis (LAM), clear cell myomelanocytic tumor of falciform ligament/ligamentum teres Etiology/Pathogenesis • Only AML, CCST, and LAM are associated with tuberous sclerosis, but not other types Clinical Issues • Most are benign, but rare malignant cases reported • Rare tumors overall; very rare in skin Microscopic • PEC cell…
Looking for online definition of PLAM or what PLAM stands for? PLAM is listed in the Worlds largest and most authoritative dictionary database of abbreviations and acronyms
The LAM Foundation urgently seeks safe and effective treatments, and ultimately a cure, for lymphangioleiomyomatosis (LAM) through advocacy and the funding of promising research.
BACKGROUND: Recent studies indicate that the smooth muscle-like cells contributing to neointimal hyperplasia after vascular injury derive from circulating precursor cells. Here, we define the time course of precursor cell influx, the roles of separat
I am very lucky to be alive after a double-lung transplant in July 2006. I suffered the rarest lung disease called, Lymphangioleiomyomatosis (Lam). I kept fighting for my life and trying to breathe each time my lung collapsed (15 times). I used to be on 24 hour oxygen to help me breathe and also wheelchair bound. At one stage I ended up in a coma (3weeks) and remained on life support for almost 2 months. I had to learn how to walk again with a zimaframe, and start to re-build my life/confidence/strength up. I want to enjoy my life with the extension this transplant has given me. My journey post transplant has been challenging with health issues, but, I try to remain as positive as possible ...
I am very lucky to be alive after a double-lung transplant in July 2006. I suffered the rarest lung disease called, Lymphangioleiomyomatosis (Lam). I kept fighting for my life and trying to breathe each time my lung collapsed (15 times). I used to be on 24 hour oxygen to help me breathe and also wheelchair bound. At one stage I ended up in a coma (3weeks) and remained on life support for almost 2 months. I had to learn how to walk again with a zimaframe, and start to re-build my life/confidence/strength up. I want to enjoy my life with the extension this transplant has given me. My journey post transplant has been challenging with health issues, but, I try to remain as positive as possible ...
... program, lymphangioleiomyomatosis, smoke induced lung injury, cigarette smoke related vascular disease, tissue destruction in alternative lung diseases
This disorder occurs in young adult smokers, and smoking cessation can lead to partial or total remission of the pulmonary lesions. In the early stages, it is characterized by bronchoalveolar inflammatory changes and, in the most advanced phases, by cystic lung destruction. The clinical manifestations vary widely: dyspnea, cough, weakness, fever, weight loss, pleuritic chest pain and, occasionally, spontaneous pneumothorax. The incidence of hemoptysis is minor; thus, should it occur, the cause should be looked for elsewhere (mainly tumor-related). The disease can be detected incidentally in radiological studies performed in asymptomatic patients, occurring in around 15% of the cases, and the same proportion can be applied to those with extrapulmonary manifestations, such as bone cysts, diabetes insipidus or exanthema.2,3 Computed tomography is the technique of choice to characterize pulmonary involvement. The combination of multiple cysts and bilateral nodules distributed throughout the medium ...
Pathology Research International is a peer-reviewed, Open Access journal that publishes original research articles, review articles, and clinical studies in all areas of pathology.
Diffuse cystic lung diseases are characterized by cysts in more than one lung lobe, the cysts originating from various mechanisms, including the expansion of the distal airspaces due to airway obstruction, necrosis of the airway walls, and parenchymal destruction. The progression of these diseases is variable. One essential tool in the evaluation of these diseases is HRCT, because it improves the characterization of pulmonary cysts (including their distribution, size, and length) and the evaluation of the regularity of the cyst wall, as well as the identification of associated pulmonary and extrapulmonary lesions ...
I believe now that there is a reason I have not been able to concieve. God controls all things, and at the moment, I am thankful that I havent had the opportunity.....
We all know that womens bodies are different from men, but to what extent do we truly understand how women can take unique preventative measures to live a long and healthy life?Here are 5 health conditions that affect women, and how you can take care of yourself now to prevent them from arising or escalating in the future.
Der er nu nye fordele med hensyn til at påvise aktiv tuberkulose (TB) hos HIV-smittede. Alere Determine™ TB LAM Ag-testen gør det nu muligt at screene for aktiv TB i HIV-positive patienter på behandlingsstedet, og testen leverer resultater på få minutter.
Specific Aim 1: To investigate whether, in Lymphangioleiomyomatosis (LAM) patients, the combination of sirolimus and hydroxychloroquine is safe and well tolerated. Specific Aim 2: To investigate whether, in LAM patients, 6 months of combination therapy with sirolimus and hydroxychloroquine results in improvement of indicators of disease, and whether the gains are sustained after stopping therapy.. Specific Aim 3: To investigate the potential role of a LAM-specific peripheral blood signature to predict rates of disease progression and determine responsiveness to combination therapy.. This will be a phase I dose escalation study of the combination of sirolimus (2 mg adjusted to keep trough levels between 5-15 ng/ml) and hydroxychloroquine (200 mg or 400 mg) taken orally daily. Up to 18 adult women with LAM will be enrolled. ...
Lymphangioleiomyomatosis (LAM) is a condition that affects the lungs, the kidneys, and the lymphatic system. The lymphatic system consists of a network of vessels that transport lymph fluid and immune cells throughout the body. Lymph fluid helps exchange immune cells, proteins, and other substances between the blood and tissues.. LAM is found almost exclusively in women. It often occurs as a feature of an inherited syndrome called tuberous sclerosis complex. When LAM occurs alone it is called isolated or sporadic LAM.. Signs and symptoms of LAM most often appear during a womans thirties. Affected women have an overgrowth of abnormal smooth muscle-like cells (LAM cells) in the lungs, resulting in the formation of lung cysts and the destruction of normal lung tissue. They may also have an accumulation of fluid in the cavity around the lungs (chylothorax).. The lung abnormalities resulting from LAM may cause difficulty breathing (dyspnea), chest pain, and coughing, which may bring up blood ...
CINCINNATI, OH--(Marketwired - September 26, 2016) - The LAM Foundation hosted its annual LAMposium conference in conjunction with the 2016 International Rare Lung Diseases Research Consortium, the largest rare lung diseases conference of its kind. Both events were held concurrently in Northern Kentucky from September 22-25.More than 450 scientists and...
Angiomyolipomas are the most common benign tumour of the kidney. Although regarded as benign, angiomyolipomas may grow such that kidney function is impaired or the blood vessels may dilate and burst leading to bleeding. Angiomyolipomas are strongly associated with the genetic disease tuberous sclerosis, in which most individuals will have several angiomyolipomas affecting both kidneys. They are also commonly found in women with the rare lung disease lymphangioleiomyomatosis. Angiomyolipomas are less commonly found in the liver and rarely in other organs. Whether associated with these diseases or sporadic, angiomyolipomas are caused by mutations in either the TSC1 or TSC2 genes, which govern cell growth and proliferation. They are composed of blood vessels, smooth muscle cells and fat cells. Large angiomyolipoma can be treated with embolisation. Drug therapy for angiomyolipoma is at the research stage. The Tuberous Sclerosis Alliance has published guidelines on diagnosis, surveillance and ...
OBJECTIVES: We examined the long-term effects of everolimus in patients with renal angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis. METHODS: Following favorable results from the double-blind core phase of EXIST-2 (NCT00790400), patients were allowed to receive open-label everolimus (extension phase). Patients initially randomly assigned to everolimus continued on the same dose; those who were receiving placebo crossed over to everolimus 10 mg/day. Dose modifications were based on tolerability. The primary end point was angiomyolipoma response rate, defined as a ≥50% reduction from baseline in the sum volume of target renal angiomyolipomas in the absence of new target angiomyolipomas, kidney volume increase of >20% from nadir, and angiomyolipoma-related bleeding grade ≥2. The key secondary end point was safety. RESULTS: Of the 112 patients who received ≥1 dose of everolimus, 58% (95% CI, 48.3% to 67.3%) achieved angiomyolipoma response. Almost ...
University of Houston - News:A Health and Human Performance study is investigating how physical activity impacts quality of life of those living with a rare lung disease called LAM.