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Lymphangioleiomyomatosis

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https://www.readbyqxmd.com/read/28649310/lung-ultrasound-has-limited-diagnostic-value-in-rare-cystic-lung-diseases-a-cross-sectional-study
#1
Jesper Rømhild Davidsen, Elisabeth Bendstrup, Daniel P Henriksen, Ole Graumann, Christian B Laursen
Background: Lung ultrasound (LUS) used to identify interstitial syndrome (IS) and pleural thickening related to diffuse parenchymal lung disease (DPLD) has shown significant correlations with ground glass opacity (GGO) on high-resolution computed tomography (HRCT). However, the applicability of LUS in patients with DPLD subtypes as rare cystic lung diseases has not previously been investigated. This study aimed to observe if distinctive LUS findings could be found in patients with lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), and Birt-Hogg-Dubé syndrome (BHDS)...
2017: European Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28643793/genomic-rearrangements-in-sporadic-lymphangioleiomyomatosis-an-evolving-genetic-story
#2
Stephen J Murphy, Simone B Terra, Faye R Harris, Aqsa Nasir, Jesse S Voss, James B Smadbeck, Sarah H Johnson, Vishnu Serla, Jay H Ryu, Eunhee S Yi, Benjamin R Kipp, George Vasmatzis, Eva M Carmona
Sporadic lymphangioleiomyomatosis is a progressive pulmonary cystic disease resulting from the infiltration of smooth muscle-like lymphangioleiomyomatosis cells into the lung. The migratory/metastasizing properties of the lymphangioleiomyomatosis cell together with the presence of somatic mutations, primarily in the tuberous sclerosis complex gene (TSC2), lead many to consider this a low-grade malignancy. As malignant tumors characteristically accumulate somatic structural variations, which have not been well studied in sporadic lymphangioleiomyomatosis, we utilized mate pair sequencing to define structural variations within laser capture microdissected enriched lymphangioleiomyomatosis cell populations from five sporadic lymphangioleiomyomatosis patients...
June 23, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28623674/cathepsin-k-in-lymphangioleiomyomatosis-lam-cell-fibroblast-interactions-enhance-protease-activity-by-extracellular-acidification
#3
Arundhati Dongre, Debbie Clements, Andrew J Fisher, Simon R Johnson
Lymphangioleiomyomatosis (LAM) is a rare disease in which clonal LAM cells infiltrate the lungs and lymphatics. In association with recruited fibroblasts, LAM cells form nodules adjacent to lung cysts. It is hypothesized that LAM nodule-derived proteases lead to cyst formation. On protease gene-expression profiling in whole-lung tissue, cathepsin K was 40-fold overexpressed in LAM compared with control lungs (P ≤ 0.0001). Immunohistochemistry confirmed cathepsin K protein in LAM nodules but not in control lungs...
June 14, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28616248/lymphangioleiomyomatosis-an-explosive-presentation-of-a-rare-disease
#4
Faye Pais, Mohamed Fayed, Timothy Evans
Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease, commonly affecting women in the reproductive age group. Exacerbation of pre-existing disease is common during pregnancy likely due to the up-regulation of estrogen and progesterone receptors present within the proliferating smooth muscle cells. This case highlights a dramatic presentation of LAM for the first time in pregnancy, its rapid progression during gestation, and a partial resolution with delivery. The unusual radiographic imaging in this patient, lacked the characteristic cystic lesions commonly associated with LAM, but instead demonstrated a dense interstitial pattern with micronodular expansion of the interlobular septa suggesting severe lymphatic obstruction...
June 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28613922/apparent-sporadic-lymphangioleiomyomatosis-lam-in-a-man-due-to-extreme-mosaicism-for-a-tsc2-mutation
#5
MeiLan K Han, Magdalena E Tyburczy, Thomas N Darling, Ella A Kazerooni, Jeffrey L Myers, Francis X McCormack, Joel Moss, David J Kwiatkowski
No abstract text is available yet for this article.
June 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28590337/spontaneous-pneumothorax-in-diffuse-cystic-lung-diseases
#6
Joseph Cooley, Yun Chor Gary Lee, Nishant Gupta
PURPOSE OF REVIEW: Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis...
July 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28584685/renal-epithelioid-angiomyolipoma-associated-with-pulmonary-lymphangioleiomyomatosis-imaging-findings
#7
Athina C Tsili, Alexandra Ntorkou, Maria I Argyropoulou
Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are the most common tumors of the perivascular epithelioid cell (PEComa) family. Both may be associated with tuberous sclerosis (TS) complex. Epithelioid AML (EAML) is a rare variety of AMLs, with a potential aggressive behavior. There are few reports in the English literature addressing on the imaging findings of renal EAMLs, which are considered nonspecific. We present the sonographic, computed tomographic, and magnetic resonance imaging findings of a renal EAML in a pregnant woman with concomitant pulmonary lesions indicative of LAM, without stigmata of TS...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/28577037/metabolic-impact-of-rapamycin-sirolimus-and-b-estradiol-using-mouse-embryonic-fibroblasts-as-a-model-for-lymphangioleiomyomatosis
#8
Katherine M Marsh, David Schipper, Alice S Ferng, Kitsie Johnson, Julia Fisher, Shannon Knapp, Destiny Dicken, Zain Khalpey
INTRODUCTION: Lymphangioleiomyomatosis (LAM) is a rare, progressive cystic lung disease that predominantly affects women of childbearing age. Exogenous rapamycin (sirolimus) has been shown to improve clinical outcomes and was recently approved to treat LAM, whereas estrogen (E2) is implicated in disease progression. No consistent metabolic model currently exists for LAM, therefore wild-type mouse embryonic fibroblasts (MEF +/+) and TSC2 knockout cells (MEF -/-) were used in this study as a model for LAM...
June 2, 2017: Lung
https://www.readbyqxmd.com/read/28576630/ca-125-in-disease-progression-and-treatment-of-lymphangioleiomyomatosis
#9
Connie G Glasgow, Gustavo Pacheco-Rodriguez, Wendy K Steagall, Mary E Haughey, Patricia A Julien-Williams, Mario P Stylianou, Bernadette R Gochuico, Joel Moss
BACKGROUND: Lymphangioleiomyomatosis (LAM) is a destructive lung disease of women caused by proliferation of neoplastic-like LAM cells, with mutations in the TSC1/2 tumor suppressor genes. Based on case reports, CA-125 (an ovarian cancer biomarker) can be elevated in LAM patients. We hypothesized that elevated serum CA-125 levels seen in some LAM patients were due to LAM, not other malignancies, and might respond to sirolimus treatment. METHODS: Serum CA-125 levels were measured for 241 patients at each visit...
May 30, 2017: Chest
https://www.readbyqxmd.com/read/28570161/a-phase-ii-clinical-trial-of-an-aromatase-inhibitor-for-postmenopausal-women-with-lymphangioleiomyomatosis
#10
Calvin Lu, Hye-Seung Lee, George P Pappas, Daniel F Dilling, Charles D Burger, Adrian Shifren, Srihari Veeraraghavan, Jeffrey T Chapman, Joseph Parambil, Stephen J Ruoss, Lisa R Young, Stephen R Hammes, Elizabeth J Kopras, Tammy Roads, Jeffrey P Krischer, Francis X McCormack
RATIONALE: Lymphangioleiomyomatosis (LAM) is a progressive cystic lung disease that predominantly affects women and can worsen with pregnancy, estrogen treatment, and the menstrual cycle, suggesting an important role for estrogen in disease pathogenesis. OBJECTIVES: To assess the efficacy and safety of the aromatase inhibitor letrozole in the treatment of LAM. METHODS: Seventeen postmenopausal women with LAM were enrolled in this phase II trial and randomized to receive letrozole 2...
June 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28556973/primary-cutaneous-perivascular-epithelioid-cell-tumor-pecoma-five-new-cases-and-review-of-the-literature
#11
Lauren N Stuart, Russell G Tipton, Michael DeWall, Douglas C Parker, Christina D Stelton, Annie O Morrison, Landon W Coleman, Scott W Fosko, Claudia I Vidal, M Yadira Hurley, Amy H Deeken, Jerad M Gardner
PEComas represent a family of uncommon mesenchymal tumors composed of "perivascular epithelioid cells" with a distinct immunophenotype that typically shows both myogenic and melanocytic differentiation. The PEComa family includes angiomyolipoma (AML), clear cell "sugar" tumor of the lung and extra pulmonary sites, lymphangioleiomyomatosis and clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Very rarely, PEComas may arise in the skin. Primary cutaneous PEComas typically display a dermal proliferation of epithelioid cells with pale, clear, or granular pink cytoplasm arranged in nests and trabeculae with an intervening arborizing network of delicate capillaries...
May 30, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28538782/diffuse-cystic-lung-diseases-differential-diagnosis
#12
Bruno Guedes Baldi, Carlos Roberto Ribeiro Carvalho, Olívia Meira Dias, Edson Marchiori, Bruno Hochhegger
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...
March 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/28533049/long-term-effect-of-sirolimus-on-serum-vegf-d-levels-in-patients-with-lymphangioleiomyomatosis
#13
Angelo M Taveira-DaSilva, Amanda M Jones, Patricia Julien-Williams, Mario Stylianou, Joel Moss
BACKGROUND: Sirolimus reduces serum levels of VEGF-D, size of chylous effusions, lymphangioleiomyomas, and angiomyolipomas, and stabilizes lung function in patients with lymphangioleiomyomatosis. METHODS: To determine whether reductions in VEGF-D levels are sustained over time, and parallel changes in lung function and lymphatic disease, we evaluated 25 patients with lymphangioleiomyomatosis and measured VEGF-D levels, lung function and extent of lymphatic disease before and during sirolimus therapy RESULTS: Treatment with sirolimus stabilized FEV1 and DLCO over a period of 4...
May 19, 2017: Chest
https://www.readbyqxmd.com/read/28457375/evaluation-of-renal-function-in-patients-undergoing-lung-transplantation
#14
C Carillo, Y Pecoraro, M Anile, S Mantovani, A Oliva, A D'Abramo, D Amore, A Pagini, T De Giacomo, F Pugliese, E A Rendina, F Venuta, D Diso
BACKGROUND: Acute kidney injury and chronic kidney failure are serious complications after lung transplantation. Glomerular filtration rate (GFR) is the primary indicator of renal function. Several equations have been proposed to evaluate the estimated GFR (eGFR). We compared three different equations to determine which has the better correlation with the development of acute and chronic renal failure in lung recipients. METHODS: Twenty-two patients with a mean age of 54...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28445558/clinical-characteristics-of-connective-tissue-nevi-in-tuberous-sclerosis-complex-with-special-emphasis-on-shagreen-patches
#15
Michelle A Bongiorno, Neera Nathan, Oyetewa Oyerinde, Ji-An Wang, Chyi-Chia Richard Lee, G Thomas Brown, Joel Moss, Thomas N Darling
Importance: Patients with tuberous sclerosis complex (TSC) frequently develop collagenous connective tissue nevi. The prototypical lesion is a large shagreen patch located on the lower back, but some patients only manifest small collagenomas or have lesions elsewhere on the body. The ability to recognize these variable presentations can be important for the diagnosis of TSC. Objective: To describe the clinical characteristics of connective tissue nevi on the trunk and extremities of patients with tuberous sclerosis complex...
April 26, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28427470/pulmonary-hypertension-in-lymphangioleiomyomatosis-prevalence-severity-and-the-role-of-carbon-monoxide-diffusion-capacity-as-a-screening-method
#16
Carolina S G Freitas, Bruno G Baldi, Carlos Jardim, Mariana S Araujo, Juliana Barbosa Sobral, Gláucia I Heiden, Ronaldo A Kairalla, Rogério Souza, Carlos R R Carvalho
BACKGROUND: Lymphangioleiomyomatosis (LAM) is included within group 5 of the current PH classification (unclear multifactorial mechanisms). However, data regarding the occurrence of PH in LAM are scarce. The aims of the study were to describe the prevalence and characteristics of PH in a large cohort of LAM patients with different levels of severity, and to evaluate the role of echocardiography and carbon monoxide diffusion capacity (DLCO) as screening methods for PH in LAM. METHODS: One hundred five LAM patients underwent transthoracic echocardiography, pulmonary function tests (PFTs) and 6-min walk test (6MWT)...
April 20, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28420844/protein-losing-enteropathy-caused-by-intestinal-or-colonic-lymphangiectasia-complicated-by-sporadic-lymphangioleiomyomatosis-a-report-of-two-cases
#17
Koichi Nishino, Kaku Yoshimi, Tomoyoshi Shibuya, Takuo Hayashi, Keiko Mitani, Etsuko Kobayashi, Masako Ichikawa, Tetsuhiko Asao, Yohei Suzuki, Tadashi Sato, Satomi Shiota, Yuzo Kodama, Kazuhisa Takahashi, Kuniaki Seyama
This report describes two patients with sporadic lymphangioleiomyomatosis complicated by protein-losing enteropathy (PLE). Imaging studies indicated retroperitoneal lymphangioleiomyomas and abnormalities of the adjacent digestive tract. Endoscopic mucosal biopsy revealed colonic lymphangiectasia in one patient; whereas the site in the other patient was intestinal. Treatment with sirolimus led to the complete resolution of PLE within several months; additionally, marked shrinkage was observed in the lymphangioleiomyomas of both cases...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28414661/delayed-presentation-of-tuberour-sclerosis-complex-in-adult-women
#18
J Manalac, S Sadd, G Akoghlanian, T Benoit-Clark
INTRODUCTION: Tuberous sclerosis complex (TSC); is an autosomal dominant disorder characterized by the formation of hamartomatous lesions in multiple organs, with a birth incidence of around one in 10,000. Although it usually manifests itself in early life, we present a case of an adult woman who we diagnosed with TSC. CASE: A 27 year old woman presented to Emergency Department with worsening right flank pain and progressive dyspnea. Physical examination findings revealed Shagreen patches and multiple angiomyolipomas of the skin...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28410230/insulin-growth-factor-binding-protein-2-mediates-the-progression-of-lymphangioleiomyomatosis
#19
Xiangke Li, Xiaolei Liu, Linda Zhang, Chenggang Li, Erik Zhang, Wang Ma, Qingxia Fan, Jane J Yu
Lymphangioleiomyomatosis (LAM) is a progressive pulmonary disease that almost exclusively affects women. LAM cells migrate to the lungs, where they cause cystic destruction of lung parenchyma. Mutations in TSC1 or TSC2 lead to the activation of the mammalian target of rapamycin complex-1, a kinase that regulates growth factor-dependent protein translation, cell growth, and metabolism. Insulin-like growth factor binding protein 2 (IGFBP2) binds insulin, IGF1 and IGF2 in circulation, thereby modulating cell survival, migration, and invasion in neoplasms...
May 30, 2017: Oncotarget
https://www.readbyqxmd.com/read/28325364/retroperitoneal-lymphangioleiomyoma-with-lymph-node-involvement-a-pathologic-radiologic-correlation-of-a-rare-form-of-myomelanocytic-tumor
#20
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
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