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https://www.readbyqxmd.com/read/29221318/lung-densitometry-why-how-and-when
#1
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
#2
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
https://www.readbyqxmd.com/read/29214586/epidemiology-of-rare-lung-diseases-the-challenges-and-opportunities-to-improve-research-and-knowledge
#3
Cormac McCarthy, Beatriz Lara Gallego, Bruce C Trapnell, Francis X McCormack
Rare lung diseases encompass a broad spectrum of conditions and affect an estimated 1.2-2.5 million people in North America and 1.5-3 million people in Europe. While individual rare lung diseases affect less than 1 in 2000 individuals, collectively they have a significant impact upon the population at large. Hence it is vital to understand firstly the epidemiology and subsequently the pathogenesis and clinical course of these disorders. Through a greater understanding of these aspects of disease, progress can be made in reducing symptoms, containing healthcare costs and utilizing resources efficiently...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29208258/durability-of-sirolimus-for-lymphangioleiomyomatosis
#4
Alexandra Martirossian, Shiwan Shah, Lola Carrete, Jose Valle, Vincent Valentine
Lymphangioleiomyomatosis (LAM), a rare, multisystem disorder primarily affecting women of reproductive age, is characterized by cystic-appearing lung lesions, progressive loss of lung function, chylous effusions and renal angiomyolipomas. Sirolimus, an mammalian target of rapamycin inhibitor, has been shown to stabilize lung function, reduce symptoms and resolve chylous effusions in the short term for patients with LAM. Herein, we report a premenopausal female with LAM who experienced complete and durable resolution of her chylothoraces with significant and sustained improvement in lung function on sirolimus...
December 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29184052/notch-transactivates-rheb-to-maintain-the-multipotency-of-tsc-null-cells
#5
Jun-Hung Cho, Bhaumik Patel, Santosh Bonala, Sasikanth Manne, Yan Zhou, Surya K Vadrevu, Jalpa Patel, Marco Peronaci, Shanawaz Ghouse, Elizabeth P Henske, Fabrice Roegiers, Krinio Giannikou, David J Kwiatkowski, Hossein Mansouri, Maciej M Markiewski, Brandon White, Magdalena Karbowniczek
Differentiation abnormalities are a hallmark of tuberous sclerosis complex (TSC) manifestations; however, the genesis of these abnormalities remains unclear. Here we report on mechanisms controlling the multi-lineage, early neuronal progenitor and neural stem-like cell characteristics of lymphangioleiomyomatosis (LAM) and angiomyolipoma cells. These mechanisms include the activation of a previously unreported Rheb-Notch-Rheb regulatory loop, in which the cyclic binding of Notch1 to the Notch-responsive elements (NREs) on the Rheb promoter is a key event...
November 29, 2017: Nature Communications
https://www.readbyqxmd.com/read/29171770/emerging-biomarkers-of-lymphangioleiomyomatosis
#6
Julie Nijmeh, Souheil El-Chemaly, Elizabeth P Henske
Lymphangioleiomyomatosis (LAM) is a destructive lung disease affecting primarily women. LAM is caused by inactivating mutations in the tuberous sclerosis complex (TSC) genes, resulting in hyperactivation of mechanistic/mammalian target of rapamycin complex 1 (mTORC1). Over the past five years, there have been remarkable advances in the diagnosis and therapy of LAM, including the identification of vascular endothelial growth factor D (VEGF-D) as a diagnostic biomarker and the US Food and Drug Administration approval of sirolimus as therapy for LAM...
November 24, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/29169151/cystic-lung-disease-in-genetic-syndromes-with-deficient-tumor-suppressor-gene-function
#7
Cécile Daccord, Laurent P Nicod, Romain Lazor
Cystic lung diseases constitute a distinct group of rare lung disorders, among which two result from monogenic defects affecting tumor suppressor genes: lymphangioleiomyomatosis, either sporadic or associated with tuberous sclerosis complex, and Birt-Hogg-Dubé syndrome. These disorders have similarities in their clinical expression, including occurrence in young adults, multiple pulmonary cysts, recurrent pneumothorax, skin hamartomas, and renal tumors. However, they markedly differ in their gender distribution, pathogenesis, disease course, and prognosis...
November 23, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29140122/lymphangioleiomyomatosis-diagnosis-and-management-high-resolution-chest-computed-tomography-transbronchial-lung-biopsy-and-pleural-disease-management-an-official-american-thoracic-society-japanese-respiratory-society-clinical-practice-guideline
#8
Nishant Gupta, Geraldine A Finlay, Robert M Kotloff, Charlie Strange, Kevin C Wilson, Lisa R Young, Angelo M Taveira-DaSilva, Simon R Johnson, Vincent Cottin, Steven A Sahn, Jay H Ryu, Kuniaki Seyama, Yoshikazu Inoue, Gregory P Downey, MeiLan K Han, Thomas V Colby, Kathryn A Wikenheiser-Brokamp, Cristopher A Meyer, Karen Smith, Joel Moss, Francis X McCormack
BACKGROUND: Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now provide additional recommendations regarding four specific questions related to the diagnosis of LAM and management of pneumothoraces in patients with LAM. METHODS: Systematic reviews were performed and then discussed by a multidisciplinary panel. For each intervention, the panel considered its confidence in the estimated effects, the balance of desirable (i...
November 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29132117/an-extrapulmonary-manifestation-of-lymphangioleiomyomatosis-a-rare-case-report
#9
L Volkan Tümay, Osman Serhat Güner, Abdullah Zorluoğlu
Lymphangioleiomyomatosis (LAM) is a rare and fatal disease which occurs almost exclusively in young women. The disease often affects lungs and most of the patients die from respiratory failure. It is often initially misdiagnosed as asthma or chronic obstructive pulmonary disease. The most common presentations of pulmonary LAM (P-LAM) include dyspnea and coughing. Chylothorax and spontaneous pneumothorax may be seen in advanced cases. Although rare, it may present with extrapulmonary LAM (E-LAM). Renal angiomyolipomas and abdominal lymphadenopathies (LAPs) are common in E-LAM cases...
November 9, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29123566/consideration-of-underlying-causes-of-pneumothoraces-in-patients-presenting-with-their-first-pneumothorax
#10
Emma Jackson, Raj Nichani
We report a case of cardiac arrest secondary to pneumothorax. The patient failed to improve, and further investigation showed an underlying condition predisposing the patient to the development of pneumothoraces - lymphangioleiomyomatosis. This highlights the importance of follow-up and investigation in patients presenting with their first pneumothorax.
November 2017: Journal of the Intensive Care Society
https://www.readbyqxmd.com/read/29062682/transudative-chylothorax-in-a-patient-with-lymphangioleiomyomatosis
#11
Adrian Pedro Noriega Aldave, John W Leslie
Transudative chylothorax is a rare type of pleural effusion. It has been described to be present in the setting of liver cirrhosis, heart failure, amyloidosis, nephrotic syndrome, superior vena cava thrombosis, sclerosing mesenteritis and heart failure. We present the case of an 86-year-old woman with a right-side transudative chylothorax associated with heart failure and lymphangioleiomyomatosis.
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29046979/medical-management-of-pulmonary-hypertension-with-unclear-and-or-multifactorial-mechanisms-group-5-is-there-a-role-for-pulmonary-arterial-hypertension-medications
#12
REVIEW
Jason Weatherald, Laurent Savale, Marc Humbert
PURPOSE OF REVIEW: The purpose of this review was to outline the mechanisms and to review recent literature on pulmonary arterial hypertension (PAH) medications in group 5 pulmonary hypertension (PH). RECENT FINDINGS: The first steps in management are to understand the mechanisms and hemodynamic profile and to exclude chronic thromboembolic disease. Recent studies in the past 5 years have found that PAH medications may improve hemodynamics in patients with pre-capillary pulmonary hypertension due to sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, and myeloproliferative disorders with dasatinib-induced PH...
October 18, 2017: Current Hypertension Reports
https://www.readbyqxmd.com/read/29021448/additional-octreotide-therapy-to-sirolimus-achieved-a-decrease-in-sirolimus-refractory-chylous-effusion-complicated-with-lymphangioleiomyomatosis
#13
Masashi Namba, Takeshi Masuda, Takashi Nakamura, Yasushi Horimasu, Shintaro Miyamoto, Taku Nakashima, Hiroshi Iwamoto, Kazunori Fujitaka, Hironobu Hamada, Noboru Hattori
Recently, sirolimus, an inhibitor of mammalian target of rapamycin, was reported to decrease chylous effusion in patients with lymphangioleimyomatosis (LAM). We herein report a case of a 34-year-old woman with LAM who developed refractory chylothorax and chylous ascites during sirolimus therapy. In this case, to reduce chylous effusion, we administered octreotide, which is often used to control postoperative chylous effusion, in addition to the sirolimus therapy. This combination therapy reduced the chylothorax and chylous ascites...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28993539/lung-function-response-and-side-effects-to-rapamycin-for-lymphangioleiomyomatosis-a-prospective-national-cohort-study
#14
Janet Bee, Sharon Fuller, Suzanne Miller, Simon R Johnson
RATIONALE: Mechanistic target of rapamycin inhibitors reduce loss of lung function in lymphangioleiomyomatosis (LAM), although their benefit varies between individuals. We examined lung function response and side effects to rapamycin in a national cohort. METHODS: Subjects were receiving rapamycin for progressive lung disease. Clinical evaluation, detailed phenotyping, serial lung function, rapamycin and safety monitoring were performed according to a clinical protocol...
October 9, 2017: Thorax
https://www.readbyqxmd.com/read/28972182/urokinase-type-plasminogen-activator-upa-is-critical-for-progression-of-tuberous-sclerosis-complex-2-tsc2-deficient-tumors
#15
Victoria Stepanova, Konstantin V Dergilev, Kelci R Holman, Yelena V Parfyonova, Zoya I Tsokolaeva, Mimi Teter, Elena N Atochina-Vasserman, Alla Volgina, Sergei V Zaitsev, Shane P Lewis, Fedor G Zabozlaev, Kseniya Obraztsova, Vera P Krymskaya, Douglas B Cines
Lymphangioleiomyomatosis (LAM) is a fatal lung disease associated with germline or somatic inactivating mutations in tuberous sclerosis complex genes (TSC1 or TSC2). LAM is characterized by neoplastic growth of smooth muscle (SM)-α-actin-positive cells, that destroy lung parenchyma, and by the formation of benign renal neoplasms called angiolipomas. The mTOR complex 1 (mTORC1) inhibitor rapamycin slows progression of these diseases, but is not curative and associated with notable toxicity at clinically effective doses, highlighting the need for better understanding LAM's molecular etiology...
September 27, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28960664/efficacy-and-safety-of-transbronchial-lung-biopsy-for-the-diagnosis-of-lymphangioleiomyomatosis-a-report-of-24-consecutive-patients
#16
Taro Koba, Toru Arai, Masanori Kitaichi, Takahiko Kasai, Masaki Hirose, Kazunobu Tachibana, Chikatoshi Sugimoto, Masanori Akira, Seiji Hayashi, Yoshikazu Inoue
BACKGROUND AND OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a diffuse cystic lung disease that occurs in women of childbearing age. LAM can be diagnosed on a clinical basis in patients with typical high-resolution computed tomography (HRCT) patterns and at least one other corroborating disease feature, such as chylothorax, angiomyolipoma, tuberous sclerosis complex or elevated serum vascular endothelial growth factor (VEGF)-D. However, patients who do not meet these criteria require tissue confirmation for a definitive diagnosis, and the utility of methods that are less invasive than surgical lung biopsy, such as transbronchial lung biopsy (TBLB), are not well studied...
September 28, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28954765/new-insights-in-lymphangioleiomyomatosis-and-pulmonary-langerhans-cell-histiocytosis
#17
REVIEW
Olga Torre, Davide Elia, Antonella Caminati, Sergio Harari
Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. In this review, the recent advances in the understanding of the molecular pathogenesis, as well as the current and most promising biomarkers and therapeutic approaches, are described...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28910919/-the-value-of-serum-vegf-d-concentration-and-tsc-gene-in-diagnosis-and-treatment-of-lymphangioleiomyomatosis
#18
J Lin, G X Lai, W Wen
No abstract text is available yet for this article.
September 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28899738/angiomyomatous-hamartoma-of-lymph-nodes-revisited-clinico-pathologic-study-of-21-cases-emphasizing-its-distinction-from-lymphangioleiomyomatosis-of-lymph-nodes
#19
Michelle Moh, Ankur Sangoi, Joseph T Rabban
Angiomyomatous hamartoma of lymph nodes (AMH-LN) is an uncommon benign proliferation of smooth muscle, blood vessels, collagenous stroma and adipocytes, most commonly affecting inguinal LN. A similar constellation of cell types constitutes various members of the perivascular epithelioid cell tumor (PEComa) family, including lymphangioleiomyomatosis (LAM) which can involve LN in women. Because some LN-LAM patients have tuberous sclerosis complex (TSC) and/or other PEComa family lesions, it is clinically relevant to distinguish LN-LAM from AMH-LN...
September 9, 2017: Human Pathology
https://www.readbyqxmd.com/read/28888335/dramatic-relapse-of-seizures-after-everolimus-withdrawal
#20
Alessia Mingarelli, Aglaia Vignoli, Francesca La Briola, Angela Peron, Lucio Giordano, Giuseppe Banderali, Maria Paola Canevini
Tuberous sclerosis complex (TSC) is an autosomal dominant multisystemic disorder caused by deregulation of the mTOR pathway, and represents one of the leading genetic causes of epilepsy. mTOR inhibitors (Sirolimus and Everolimus) are currently approved only for the treatment of growing subependymal giant cell astrocytomas, renal angiomyolipomas and lymphangioleiomyomatosis in TSC. However, preclinical and clinical evidence supports their potential role in effectively treating TSC-associated epilepsy, but no consensus on its use in seizures has been reached yet and there are few data on epilepsy outcome after the suspension of mTOR inhibitors treatment...
August 3, 2017: European Journal of Paediatric Neurology: EJPN
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