Results 41 to 50 of about 11,099 (227)

Cathepsin K in lymphangioleiomyomatosis: LAM cell-fibroblast Interactions enhance protease activity by extracellular acidification [PDF]

, 2017
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 assumed LAM nodule derived proteases lead to
Akhenblit, Andrew J. Fisher, Arundhati Dongre, Atochina-Vasserman, Badri, Bee, Boonen, Bromme, Chang, Chang, Chilosi, Clements, Debbie Clements, Ferri, Finlay, Goldberg, Goncharova, Goncharova, Greenlee, Hayashi, Henske, Inoue, Johnson, Kaluz, Kang, Kant, Kneissel, Lecaille, Lee, Levy, Livak, Lou, Martignoni, Matsui, McCormack, McCormack, McDonald, McIntyre, Montgomery, Odajima, Onda, Pacchiano, Parkhitko, Pea, Punturieri, Repnik, Riihonen, Romero, Simon R. Johnson, Touisni, Turk, Yu, Zhao, Zhe   +53 more
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Histamine signaling and metabolism identify potential biomarkers and therapies for lymphangioleiomyomatosis

EMBO Molecular Medicine, 2021
Inhibition of mTOR is the standard of care for lymphangioleiomyomatosis (LAM). However, this therapy has variable tolerability and some patients show progressive decline of lung function despite treatment.
Carmen Herranz, Francesca Mateo, Alexandra Baiges, Gorka Ruiz de Garibay, Alexandra Junza, Simon R Johnson, Suzanne Miller, Nadia García, Jordi Capellades, Antonio Gómez, August Vidal, Luis Palomero, Roderic Espín, Ana I Extremera, Eline Blommaert, Eva Revilla‐López, Berta Saez, Susana Gómez‐Ollés, Julio Ancochea, Claudia Valenzuela, Tamara Alonso, Piedad Ussetti, Rosalía Laporta, Antoni Xaubet, José A Rodríguez‐Portal, Ana Montes‐Worboys, Carlos Machahua, Jaume Bordas, Javier A Menendez, Josep M Cruzado, Roser Guiteras, Christophe Bontoux, Concettina La Motta, Aleix Noguera‐Castells, Mario Mancino, Enrique Lastra, Raúl Rigo‐Bonnin, Jose C Perales, Francesc Viñals, Alvaro Lahiguera, Xiaohu Zhang, Daniel Cuadras, Coline H M van Moorsel, Joanne J van der Vis, Marian J R Quanjel, Harilaos Filippakis, Razq Hakem, Chiara Gorrini, Marc Ferrer, Aslihan Ugun‐Klusek, Ellen Billett, Elżbieta Radzikowska, Álvaro Casanova, María Molina‐Molina, Antonio Roman, Oscar Yanes, Miquel A Pujana   +56 more
doaj   +1 more source

An entity that poses continuous challenge despite treatment attempt: lymphangioleiomyomatosis

The Pan African Medical Journal, 2020
In May of 2016, a 37-years-old Hispanic female, with no significant past medical history was brought to our hospital with right-sided chest pain. Chest X ray revealed large right pneumothorax (A) and she underwent chest tube placement.
Munish Sharma, Salim Surani
doaj   +1 more source

Does Unilateral DIPNECH Provide Clues to Pathogenesis? [PDF]

, 1968
Digitalitzat per ...
Miller, York E.
core   +1 more source

Cellular antiseizure mechanisms of everolimus in pediatric tuberous sclerosis complex, cortical dysplasia, and non-mTOR-mediated etiologies. [PDF]

, 2018
The present study was designed to examine the potential cellular antiseizure mechanisms of everolimus, a mechanistic target of rapamycin (mTOR) pathway blocker, in pediatric epilepsy cases. Cortical tissue samples obtained from pediatric patients (n 
Barry, Joshua, Cepeda, Carlos, Fallah, Aria, Levine, Michael S, Levinson, Simon, Mathern, Gary W, Vinters, Harry V, Wu, Joyce Y, Yazon, Vannah-Wila   +8 more
core   +2 more sources

Tuberous Sclerosis Complex (TSC): Expert Recommendations for Provision of Coordinated Care. [PDF]

, 2019
Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem genetic disorder characterized by benign tumors in multiple organs, including the skin, brain, kidneys, and lungs and occasional malignant tumors.
Adriaensen, Amin, Bissler, Black, Bombardieri, Breckenridge, Capal, Crino, Curatolo, Curatolo, Curatolo, Curatolo, De Vries, Franz, French, Jansen, Jansen, Johnson, Jóźwiak, Jóźwiak, Jóźwiak, Kingswood, Kingswood, Kingswood, Koenig, Krueger, Northrup, O'Callaghan, Roth, Sampson, Shepherd, Whitney, Wu   +32 more
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Pulmonary Lymphangioleiomyomatosis

Archives of Pathology & Laboratory Medicine, 2010
Abstract Lymphangioleiomyomatosis is an uncommon lung disease primarily affecting women of childbearing age. It is characterized by the progressive proliferating and infiltrating smooth musclelike cells (lymphangioleiomyomatosis cells), which lead to the cystic destruction of the lung parenchyma; obstruction of airways, blood vessels ...
Xuchen, Zhang, William D, Travis
openaire   +2 more sources

Tuberous sclerosis complex presenting as pulmonary lymphangioleiomyomatosis - a clinicoradiological diagnosis

The Pan African Medical Journal, 2015
Tuberous sclerosis complex (TSC) manifests predominantly as a neurocutaneous disorder. Lymphangioleiomyomatosis (LAM) is a rare pulmonary manifestation of TSC.
Kamini Gupta, Amit Goyal, Kavita Saggar, Avik Banerjee   +3 more
doaj   +1 more source

Recent advances in the management of lymphangioleiomyomatosis [version 1; referees: 4 approved]

F1000Research, 2018
Lymphangioleiomyomatosis is a rare disorder that predominantly affects women and is characterized by progressive cystic changes in the lung, leading to gradually worsening shortness of breath and lung function impairment.
Kai-Feng Xu, Xinlun Tian, Jay H Ryu
doaj   +1 more source

Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: a phase 2 trial [PDF]

, 2011
This study showed sustained regression of renal angiomyolipomas in patients with tuberous sclerosis or sporadic LAM receiving 2 years of sirolimus treatment.
Cox, J A, Cross, J J, Davies, D M, de Vries, P J, Doyle, T, Howe, C J, Johnson, S R, Kingswood, J C, McCartney, D L, Pointon, K, Sampson, J R, Serra, A L, Tattersfield, A E, Watson, P C   +13 more
core   +1 more source