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Lymphangioleiomyomatosis

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https://www.readbyqxmd.com/read/28325364/retroperitoneal-lymphangioleiomyoma-with-lymph-node-involvement-a-pathologic-radiologic-correlation-of-a-rare-form-of-myomelanocytic-tumor
#1
Amrou Abdelkader, Cesar A Lam, Kaushik S Shahir, Kathleen Christians, Saul M Suster
Lymphangioleiomyomatosis (LAM) is a rare and slowly progressive disorder that usually arises in the lung, affects exclusively women in their childbearing years, and typically presents with progressive dyspnea on exertion and pneumothorax. Infrequently, extra-pulmonary LAM can occur in the retroperitoneum, uterine wall, mediastinum and intraperitoneal lymph nodes. Histologically, LAM is characterized by a proliferation of perivascular epithelioid cells (PEC) that express markers for both melanocytes and smooth muscle cells...
April 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28298732/imaging-of-tuberous-sclerosis-complex-a-pictorial-review
#2
Felipe Mussi von Ranke, Igor Murad Faria, Gláucia Zanetti, Bruno Hochhegger, Arthur Soares Souza, Edson Marchiori
Tuberous sclerosis complex (TSC) is a genetically determined hamartomatous neurocutaneous disease with high phenotypic variability. TSC is characterized by widespread hamartomas and benign, or rarely malignant, neoplasms distributed in several organs throughout the body, especially in the brain, skin, retina, kidney, heart, and lung. Common manifestations include cortical tubers, subependymal nodules, white matter abnormalities, retinal abnormalities, cardiac rhabdomyoma, lymphangioleiomyomatosis, renal angiomyolipoma, and skin lesions...
January 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/28289456/lymphangioleiomyomatosis-lam
#3
(no author information available yet)
LAM is characterised by lung cysts (air-filled sacs which gradually destroy the lung), changes to the lymphatic system and tumours in the kidneys.
March 2017: Breathe
https://www.readbyqxmd.com/read/28288285/reply-to-official-ats-jrs-clinical-practice-guidelines-lymphangioleiomyomatosis-diagnosis-and-management
#4
Francis X McCormack, Nishant Gupta, Geraldine Finlay, Lisa R Young, Simon Johnson, Vincent Cottin, Kevin Wilson, Joel Moss
No abstract text is available yet for this article.
March 13, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28288282/official-ats-jrs-clinical-practice-guidelines-lymphangioleiomyomatosis-diagnosis-and-management
#5
Sergio Harari, Roberto Cassandro, Olga Torre
No abstract text is available yet for this article.
March 13, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28272193/pelvic-lymphangioleiomyomatosis-treated-successfully-with-everolimus-two-case-reports-with-literature-review
#6
Sharjil Wahid, Ping Chia Chiang, Hao Lun Luo, Shun-Chen Huang, Eing-Mei Tsai, Po Hui Chiang
BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare disease affecting young women caused by abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs and extrapulmonary sites (extrapulmonary LAM). The objective of this case series is to demonstrate marked regression in 2 cases of retroperitoneal LAM after treatment with everolimus, an mTOR inhibitor. METHODS: We enrolled 2 cases with large volume, extrapulmonary pelvic LAM, and evaluated them with contrast-enhanced abdominal computed tomographic (CT) scans at presentation and serially during treatment with everolimus...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28264540/diagnosis-and-treatment-of-cystic-lung-disease
#7
REVIEW
Sanghoon Park, Eun Joo Lee
Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually < 2 mm). The recognition of CLDs has increased with the widespread use of computed tomography. This article addresses the mechanisms of cyst formation and the diagnostic approaches to CLDs. A number of assessment methods that can be used to confirm CLDs are discussed, including high-resolution computed tomography, pathologic approaches, and genetic/ serologic markers, together with treatment modalities, including new therapeutic drugs currently being evaluated...
March 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28202529/aberrant-syk-kinase-signaling-is-essential-for-tumorigenesis-induced-by-tsc2-inactivation
#8
Ye Cui, Wendy K Steagall, Anthony M Lamattina, Gustavo Pacheco-Rodriguez, Mario Stylianou, Pranav Kidambi, Benjamin Stump, Fernanda Golzarri, Ivan O Rosas, Carmen Priolo, Elizabeth P Henske, Joel Moss, Souheil El-Chemaly
Somatic or germline mutations in the tuberous sclerosis complex (TSC) tumor suppressor genes are associated closely with the pathogenesis of lymphangioleiomyomatosis, a rare and progressive neoplastic disease that predominantly affects women in their childbearing years. Serum levels of the lymphangiogenic growth factor VEGF-D are elevated significantly in lymphangioleiomyomatosis. However, there are gaps in knowledge regarding VEGF-D dysregulation and its cellular origin in lymphangioleiomyomatosis. Here, we show that increased expression and activation of the tyrosine kinase Syk in TSC2-deficient cells and pulmonary nodules from lymphangioleiomyomatosis patients contributes to tumor growth...
March 15, 2017: Cancer Research
https://www.readbyqxmd.com/read/28192114/sirolimus-and-autophagy-inhibition-in-lam-results-of-a-phase-i-clinical-trial
#9
Souheil El-Chemaly, Angelo Taveira-Dasilva, Hilary J Goldberg, Elizabeth Peters, Mary Haughey, Don Bienfang, Amanda M Jones, Patricia Julien-Williams, Ye Cui, Julian A Villalba, Shefali Bagwe, Rie Maurer, Ivan O Rosas, Joel Moss, Elizabeth P Henske
BACKGROUND: Animal and cellular studies support the importance of autophagy inhibition in lymphangioleiomyomatosis (LAM). In a cohort of subjects with LAM, we tested the hypothesis that treatment with sirolimus and hydroxycholoroquine (an autophagy inhibitor) at 2 different dose levels is safe and well tolerated. Secondary endpoints included changes in lung function. METHODS: This 48-week, two-center Phase I trial evaluated the safety of escalating oral hydroxychloroquine doses (100-200 mg) given twice a day in combination with sirolimus to eligible patients ≥18 years old with LAM...
February 10, 2017: Chest
https://www.readbyqxmd.com/read/28149746/multifocal-micronodular-pneumocyte-hyperplasia-mmph-in-a-patient-with-tuberous-sclerosis-evidence-for-long-term-stability
#10
Bibek S Pannu, Dinesh R Apala, Aditya Kotecha, Jennifer M Boland, Vivek N Iyer
Multifocal micronodular pneumocyte hyperplasia (MMPH) is rare entity seen mostly in patients with the tuberous sclerosis complex (TSC). We present the case of a 50 year old woman with TSC (confirmed TSC2 mutation) found to have multiple ground glass opacities with an upper lobe predominance on a screening chest CT. No abnormalities were detected in other viscera. A thoracoscopic lung biopsy obtained from right upper lobe confirmed the diagnosis of MMPH. There were no lesions suggestive of lymphangioleiomyomatosis (LAM) either on the chest CT or lung biopsy...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28132530/computed-tomography-lymphangiography-findings-in-27-cases-of-lymphangioleiomyomatosis
#11
Chunyan Zhang, Xiaobai Chen, Tingguo Wen, Qijin Zhang, Meng Huo, Jian Dong, Wen-Bin Shen, Rengui Wang
Background Lymphangioleiomyomatosis (LAM) is a rare disease involving the bronchi, lymphatic vessels, and veins. However, there are few reports about lymphatic vessel abnormalities associated with LAM. Purpose To evaluate computed tomography (CT) lymphangiography findings in cases of LAM, especially lymphatic vessel abnormalities. Material and Methods Twenty-seven patients with LAM underwent direct lymphangiography (DLG), followed by a post-procedural thoracoabdominal CT examination. Results All 27 patients were diagnosed with LAM, including 25 (93%) with chylothorax, eight (30%) with chyloperitoneum, seven (26%) with chylous fluid in the chest and abdomen, and one (4%) with lower-limb lymphedema...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28099079/lymphangioleiomyomatosis-a-monogenic-model-of-malignancy
#12
Vera P Krymskaya, Francis X McCormack
Lymphangioleiomyomatosis (LAM) is a rare, low-grade, metastasizing neoplasm that arises from an unknown source, spreads via the lymphatics, and targets the lungs. All pulmonary structures become infiltrated with benign-appearing spindle and epithelioid cells (LAM cells) that express smooth-muscle and melanocyte-lineage markers, harbor mTOR-activating mutations in tuberous sclerosis complex (TSC) genes, and recruit abundant stromal cells. Elaboration of lymphangiogenic growth factors and matrix remodeling enzymes by LAM cells enables their access to lymphatic channels and likely drives the cystic lung remodeling that often culminates in respiratory failure...
January 14, 2017: Annual Review of Medicine
https://www.readbyqxmd.com/read/28096282/the-effect-of-mtor-inhibitors-on-respiratory-infections-in-lymphangioleiomyomatosis
#13
REVIEW
Andrew M Courtwright, Hilary J Goldberg, Elizabeth Petri Henske, Souheil El-Chemaly
Lymphangioleiomyomatosis (LAM) is a destructive cystic lung disease. Mammalian target of rapamycin (mTOR) inhibitors are the primary treatment for LAM but it is unknown whether these immunosuppressing medications increase the risk for or the severity of respiratory infections in LAM patients.We searched multiple databases for original articles that reported the rate of respiratory infections in LAM patients treated with mTOR inhibitors or placebo. We calculated incidence rates for respiratory infections in these groups and incidence rate ratios for respiratory infections and severe respiratory infections in mTOR inhibitors treated versus placebo treated patients...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28057044/tuberous-sclerosis-registry-to-increase-disease-awareness-tosca-baseline-data-on-2093-patients
#14
John C Kingswood, Guillaume B d'Augères, Elena Belousova, José C Ferreira, Tom Carter, Ramon Castellana, Vincent Cottin, Paolo Curatolo, Maria Dahlin, Petrus J de Vries, Martha Feucht, Carla Fladrowski, Gabriella Gislimberti, Christoph Hertzberg, Sergiusz Jozwiak, John A Lawson, Alfons Macaya, Rima Nabbout, Finbar O'Callaghan, Mirjana P Benedik, Jiong Qin, Ruben Marques, Valentin Sander, Matthias Sauter, Yukitoshi Takahashi, Renaud Touraine, Sotiris Youroukos, Bernard Zonnenberg, Anna C Jansen
BACKGROUND: Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder. Many gaps remain in the understanding of TSC because of the complexity in clinical presentation. The TuberOus SClerosis registry to increase disease Awareness (TOSCA) is an international disease registry designed to address knowledge gaps in the natural history and management of TSC. Here, we present the baseline data of TOSCA cohort. METHODS: Patients of any age diagnosed with TSC, having a documented visit for TSC within the preceding 12 months, or newly diagnosed individuals were included...
January 5, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28005429/mammalian-target-of-rapamycin-a-target-for-lung-diseases-and-aging
#15
Brian K Kennedy, Juniper K Pennypacker
The mammalian target of rapamycin (mTOR) signaling pathway has been studied in the context of an impressive number of biological processes and disease states, including major diseases of the lung such as idiopathic pulmonary fibrosis and chronic obstructive pulmonary disease, as well as the rare condition lymphangioleiomyomatosis. The involvement of mTOR in so many disease states (in and out of the lung) raises the question how one signaling pathway can have overlapping but diverse roles seemingly everywhere...
December 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27986407/lung-cancer-in-a-patient-with-lymphangioleiomyomatosis
#16
Luis Gorospe Sarasúa, Edwin Mercedes-Noboa, María Eugenia Olmedo-García
No abstract text is available yet for this article.
December 13, 2016: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/27938553/-research-advances-on-animal-models-of-tuberous-sclerosis-and-lymphangioleiomyomatosis
#17
(no author information available yet)
No abstract text is available yet for this article.
December 12, 2016: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/27935023/causes-of-mortality-in-individuals-with-tuberous-sclerosis-complex
#18
Sam Amin, Andrew Lux, Nuala Calder, Matthew Laugharne, John Osborne, Finbar O'callaghan
AIM: The causes of death in patients with tuberous sclerosis complex (TSC) have rarely been studied, with only one published account, which was reported from the Mayo Clinic in 1991. We aimed to investigate mortality in a large cohort of patients with TSC from one of two national referral clinics in the UK. METHOD: We identified 284 patients who attended Bath TSC clinic between 1981 and 2015, and ascertained causes of death by reviewing medical records, death certificates, and postmortem reports...
December 9, 2016: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27907099/analysis-of-a-mouse-skin-model-of-tuberous-sclerosis-complex
#19
Yanan Guo, John R Dreier, Juxiang Cao, Heng Du, Scott R Granter, David J Kwiatkowski
Tuberous Sclerosis Complex (TSC) is an autosomal dominant tumor suppressor gene syndrome in which patients develop several types of tumors, including facial angiofibroma, subungual fibroma, Shagreen patch, angiomyolipomas, and lymphangioleiomyomatosis. It is due to inactivating mutations in TSC1 or TSC2. We sought to generate a mouse model of one or more of these tumor types by targeting deletion of the Tsc1 gene to fibroblasts using the Fsp-Cre allele. Mutant, Tsc1ccFsp-Cre+ mice survived a median of nearly a year, and developed tumors in multiple sites but did not develop angiomyolipoma or lymphangioleiomyomatosis...
2016: PloS One
https://www.readbyqxmd.com/read/27882343/proapoptotic-protein-bim-attenuates-estrogen-enhanced-survival-in-lymphangioleiomyomatosis
#20
Chenggang Li, Na Li, Xiaolei Liu, Erik Y Zhang, Yang Sun, Kouhei Masuda, Jing Li, Julia Sun, Tasha Morrison, Xiangke Li, Yuanguang Chen, Jiang Wang, Nagla A Karim, Yi Zhang, John Blenis, Mauricio J Reginato, Elizabeth P Henske, Jane J Yu
Lymphangioleiomyomatosis (LAM) is a progressive lung disease that primarily affects young women. Genetic evidence suggests that LAM cells bearing TSC2 mutations migrate to the lungs, proliferate, and cause cystic remodeling. The female predominance indicates that estrogen plays a critical role in LAM pathogenesis, and we have proposed that estrogen promotes LAM cell metastasis by inhibition of anoikis. We report here that estrogen increased LAM patient-derived cells' resistance to anoikis in vitro, accompanied by decreased accumulation of the proapoptotic protein Bim, an activator of anoikis...
November 17, 2016: JCI Insight
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