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Lymphangioleiomyomatosis

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https://www.readbyqxmd.com/read/29438097/rapamycin-for-lymphangioleiomyomatosis-optimal-timing-and-optimal-dosage
#1
EDITORIAL
Kai-Feng Xu, Xinlun Tian, Yanli Yang, Hongbing Zhang
No abstract text is available yet for this article.
February 2, 2018: Thorax
https://www.readbyqxmd.com/read/29408242/chylothorax-in-a-32-year-old-patient-with-lymphangioleiomyomatosis-after-bilateral-lung-transplant
#2
Panja M Boehm, Bahil Darsoon Ghanim, José Matilla, Walter Klepetko, Shahrokh Taghavi
We report on the case of a 32-year-old female patient suffering from persistent chylothorax after double-lung transplant for lymphangioleiomyomatosis. Dietary restrictions failed to decrease chylous effusions, making surgical revision necessary. The choice of an abdominal approach and postoperative treatment with somatostatin proved successful. The patient shows no recurrence of chylothorax in her two-year follow up.
February 2, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29406787/the-lam-lung-cell-and-its-human-cell-models
#3
Wendy K Steagall, Gustavo Pacheco-Rodriguez, Thomas N Darling, Olga Torre, Sergio Harari, Joel Moss
Lymphangioleiomyomatosis (LAM) is a multisystem disease of women, affecting lungs, kidneys, and lymphatics. It is caused by the proliferation of abnormal smooth muscle-like LAM cells, with mutations and loss of heterozygosity in the tuberous sclerosis complex (TSC) 1 or, more frequently, 2 genes. Isolated pulmonary LAM cells have been difficult to maintain in culture and most studies of LAM lung cells involve mixtures of TSC2 wild-type and TSC2-null cells. A clonal population of LAM lung cells has not been established, making analysis of the cells challenging...
February 6, 2018: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/29406234/mounier-kuhn-syndrome-mimicking-lymphangioleiomyomatosis
#4
Gustavo G Pacheco, Amanda M Jones, Jianhua Yao, David E Kleiner, Angelo M Taveira-DaSilva, Joel Moss
We present the case of a man with Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, who was referred to the National Institutes of Health Clinical Research Center because of a potential diagnosis of lymphangioleiomyomatosis (LAM), a rare condition in men. The patient was evaluated using ongoing protocols and provided written informed consent. The case demonstrates the presence of chronic inflammation surrounding the dilated airways and histologic changes of the lung parenchyma with emphysematouslike disruption in areas adjacent to the dilated airways...
February 2018: Chest
https://www.readbyqxmd.com/read/29406212/the-10-000-piece-puzzle-of-lymphangioleiomyomatosis
#5
EDITORIAL
Daniel F Dilling
No abstract text is available yet for this article.
February 2018: Chest
https://www.readbyqxmd.com/read/29402641/-emphysema-did-you-say-emphysema
#6
J Benzaquen, J Pradelli, B Padovani, C H Marquette, S Leroy
INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is a common condition that may initially look simple but may conceal other diseases capable of accelerating its natural history or even simulating it. We describe four cases presenting as COPD with emphysema that were reclassified on the basis of certain clinical characteristics and the radiological pattern. CASE REPORTS: A 52 year old never smoking woman presenting with emphysema was eventually diagnosed as having lymphangioleiomyomatosis on the basis of an abdominal CT scan showing kidney angiomyolipomas...
February 2, 2018: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/29398412/superselective-transcatheter-arterial-embolization-for-large-unruptured-renal-angiomyolipoma-in-lymphangioleiomyomatosis
#7
Hitomi Kato, Ryohei Kuwatsuru, Tatsuro Inoue, Shingo Okada, Mari Aida, Yuki Yamashiro
PURPOSE: To retrospectively evaluate therapeutic performance and complications of superselective transcatheter arterial embolization (TAE) for unruptured renal angiomyolipoma (AML) in patients with lymphangioleiomyomatosis (LAM) and to investigate the correlation between percentage reduction in tumor volume and intratumoral fat content. MATERIALS AND METHODS: Superselective TAE was performed in 14 consecutive patients with 15 large unruptured renal AMLs associated with LAM (mean age, 38 y; range, 21-57 y)...
February 2, 2018: Journal of Vascular and Interventional Radiology: JVIR
https://www.readbyqxmd.com/read/29373116/uncommon-hereditary-gynaecological-tumour-syndromes-pathological-features-in-tumours-that-may-predict-risk-for-a-germline-mutation
#8
REVIEW
Karuna Garg, Anthony N Karnezis, Joseph T Rabban
The most common hereditary gynaecological tumour syndromes are hereditary breast and ovarian cancer syndrome and Lynch syndrome. However, pathologists also may encounter gynaecological tumours in women with rare hereditary syndromes. Many of these tumours exhibit distinctive gross and microscopic features that are associated with a risk for an inherited gene mutation. The sensitivity and specificity of these tumour pathology features for predicting an inherited mutation vary depending on the syndrome. By recognising these tumour features, pathologists may potentially contribute to the diagnosis of an unsuspected syndrome by recommending referral of the patient for formal risk assessment by genetic counselling...
January 17, 2018: Pathology
https://www.readbyqxmd.com/read/29356560/reply-to-treatment-of-pulmonary-lymphangioleiomyomatosis-during-pregnancy
#9
Nishant Gupta, Simon R Johnson, Joel Moss, Francis X McCormack
No abstract text is available yet for this article.
January 22, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29356556/treatment-of-pulmonary-lymphangioleiomyomatosis-during-pregnancy
#10
Satoru Yanagisawa
No abstract text is available yet for this article.
January 22, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29349035/successful-treatment-of-a-patient-with-chyluria-due-to-lymphangioleiomyomatosis-using-sirolimus
#11
Takahiko Sakaue, Masaki Tominaga, Takashi Niizeki, Yoshiaki Zaizen, Ken Matsukuma, Masamichi Koganemaru, Tomoaki Hoshino, Takuji Torimura
Lymphangioleiomyomatosis (LAM) is a rare and progressive neoplastic disease of young woman, characterized by the proliferation of abnormal smooth muscle-like cells (LAM cells) in the lungs and axial lymphatics. A 44-year-old woman was referred to our hospital because pleural effusion was detected during a health checkup. She had chylothorax, chylous ascites, and chyluria, and her computed tomography scan showed a solid tumor in the pelvis. Surgical biopsy was performed; she was diagnosed as having LAM. We could not control the fluid collection and chyluria using standard medical treatments...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29344553/reprogramming-patient-derived-tumor-cells-generates-model-cell-lines-for-tuberous-sclerosis-associated-lymphangioleiomyomatosis
#12
EDITORIAL
Lisa M Julian, William L Stanford
No abstract text is available yet for this article.
November 2017: Oncoscience
https://www.readbyqxmd.com/read/29340502/recurrence-of-retroperitoneal-localized-perivascular-epithelioid-cell-tumor-two-years-after-initial-diagnosis-case-report
#13
Yasemin Benderli Cihan, Engin Kut, Ali Koç
CONTEXT: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radiotherapy and/or chemotherapy are administered according to the patient's clinical characteristics. CASE REPORT: A 42-year-old female patient was operated to treat a retroperitoneal mass. The diagnosis was established as PEComa with benign behavior. Two years after the diagnosis, chest and abdominal computed tomography scans showed intra-abdominal recurrence and lymphangioleiomyomatosis in the lung...
January 15, 2018: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/29340326/mtorc1-inhibition-is-an-effective-treatment-for-sporadic-renal-angiomyolipoma
#14
Oren Pleniceanu, Dorit Omer, Einat Azaria, Orit Harari-Steinberg, Benjamin Dekel
Introduction: Renal angiomyolipoma (AML) is the most common benign renal tumor. Despite a generally benign histology, AML can result in significant morbidity, from intra-abdominal hemorrhage and reduction in kidney function. While classically associated with the autosomal dominant disorder tuberous sclerosis complex (TSC) or with pulmonary lymphangioleiomyomatosis, most AMLs are sporadic. Mammalian target of rapamycin complex 1 (mTORC1) inhibitors (e.g., sirolimus) have been found to be effective in treating TSC- or lymphangioleiomyomatosis-associated AML, but to date it is unknown whether this strategy is effective for sporadic AML...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29339522/effect-of-beta-agonists-on-lam-progression-and-treatment
#15
Kang Le, Wendy K Steagall, Mario Stylianou, Gustavo Pacheco-Rodriguez, Thomas N Darling, Martha Vaughan, Joel Moss
Lymphangioleiomyomatosis (LAM), a rare disease of women, is associated with cystic lung destruction resulting from the proliferation of abnormal smooth muscle-like LAM cells with mutations in the tuberous sclerosis complex (TSC) genes TSC1 and/or TSC2 The mutant genes and encoded proteins are responsible for activation of the mechanistic target of rapamycin (mTOR), which is inhibited by sirolimus (rapamycin), a drug used to treat LAM. Patients who have LAM may also be treated with bronchodilators for asthma-like symptoms due to LAM...
January 30, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29300337/emerging-roles-for-vegf-d-in-human-disease
#16
REVIEW
Steven A Stacker, Marc G Achen
Blood vessels and lymphatic vessels are located in many tissues and organs throughout the body, and play important roles in a wide variety of prevalent diseases in humans. Vascular endothelial growth factor-D (VEGF-D) is a secreted protein that can promote the remodeling of blood vessels and lymphatics in development and disease. Recent fundamental and translational studies have provided insight into the molecular mechanisms by which VEGF-D exerts its effects in human disease. Hence this protein is now of interest as a therapeutic and/or diagnostic target, or as a potential therapeutic agent, in a diversity of indications in cardiovascular medicine, cancer and the devastating pulmonary condition lymphangioleiomyomatosis...
January 4, 2018: Biomolecules
https://www.readbyqxmd.com/read/29258863/fibrous-cephalic-plaques-in-tuberous-sclerosis-complex
#17
Oyetewa Oyerinde, Danielle Buccine, Alison Treichel, Claire Hong, Chyi-Chia Richard Lee, Joel Moss, Thomas N Darling
BACKGROUND: Fibrous cephalic plaques (FCPs) stereotypically develop on the forehead of tuberous sclerosis complex (TSC) patients. They constitute a major feature for TSC diagnosis, and may present before other TSC-related cutaneous hamartomas. OBJECTIVE: To describe the clinical characteristics of FCPs in TSC. METHODS: 113 patients with TSC were enrolled in an observational cohort study. Retrospective analysis of medical records and skin photography was performed...
December 16, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29228355/pulmonary-lymphangioleiomyomatosis-fertility-treatment
#18
Hiroaki Kanemura, Torahiko Jinta, Tomohide Tamura
No abstract text is available yet for this article.
December 7, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/29221318/lung-densitometry-why-how-and-when
#19
REVIEW
Mario Mascalchi, Gianna Camiciottoli, Stefano Diciotti
Lung densitometry assesses with computed tomography (CT) the X-ray attenuation of the pulmonary tissue which reflects both the degree of inflation and the structural lung abnormalities implying decreased attenuation, as in emphysema and cystic diseases, or increased attenuation, as in fibrosis. Five reasons justify replacement with lung densitometry of semi-quantitative visual scales used to measure extent and severity of diffuse lung diseases: (I) improved reproducibility; (II) complete vs. discrete assessment of the lung tissue; (III) shorter computation times; (IV) better correlation with pathology quantification of pulmonary emphysema; (V) better or equal correlation with pulmonary function tests (PFT)...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29220357/difference-of-the-progression-of-pulmonary-cysts-assessed-by-computed-tomography-among-copd-lymphangioleiomyomatosis-and-birt-hogg-dub%C3%A3-syndrome
#20
Kazunori Tobino, Toyohiro Hirai, Takeshi Johkoh, Kiminori Fujimoto, Atsushi Kawaguchi, Noriyuki Tomiyama, Kazuhisa Takahashi, Kuniaki Seyama
Many groups developed the methods to quantitatively analyze low attenuation area (LAA) on chest CT in patients with cystic lung diseases. Especially in COPD, it was reported that the cumulative size distribution of LAA clusters follows a power law characterized by the exponent D, which reflect the fractal dimension of terminal airspace geometry. We hypoyhesized that the quantitative charateristics of LAA clusters including fractal property might indicate the different features of the progression of cysts in cystic lung diseases...
2017: PloS One
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