Results 101 to 110 of about 11,099 (227)

Cathepsin K Aggravates Pulmonary Fibrosis Through Promoting Fibroblast Glutamine Metabolism and Collagen Synthesis

Advanced Science, Volume 12, Issue 34, September 11, 2025.
CTSK plays a critical role in pulmonary fibrosis. Excessive CTSK accumulation interacts with SNX9 to enhance TGF‐β1‐induced SMAD3 activation and GLS1 expression in fibroblasts, driving glutamine metabolism for collagen biosynthesis and exacerbating pulmonary fibrosis.
Mengting Chen, Xiaoxiao Meng, Yong Zhu, Dapeng Wang, Mengmeng Wang, Ziyuan Wang, Xue Tian, Jiaxiang Zhang, Zhiying Yue, Zhengfeng Yang, Ruilan Wang   +10 more
wiley   +1 more source

Treatment Patterns and Use of Resources in Patients With Tuberous Sclerosis Complex: Insights From the TOSCA Registry. [PDF]

, 2019
Tuberous Sclerosis Complex (TSC) is a rare autosomal-dominant disorder caused by mutations in the TSC1 or TSC2 genes. Patients with TSC may suffer from a wide range of clinical manifestations; however, the burden of TSC and its impact on healthcare ...
Belousoye, Elena, Benedik, Mirjana P, Carter, Tom, Cottin, Vincent, Curatolo, Paolo, D'Amato, Lisa, d'Augeres, Guillaume Beaure, Dahlin, Maria, de Vries, Petrus J, Ferreira, Jose C, Feucht, Martha, Fladrowski, Carla, Hertzberg, Christoph, Jansen, Anna C, Jozwiak, Sergiusz, Kingswood, John C, Lawson, John A, Macaya, Alfons, Marques, Ruben, Nabbout, Rima, O'Callaghan, Finbar, Qin, Jiong, Sander, Valentin, Sauter, Matthias, Shah, Seema, Takahashi, Yukitoshi, TOSCA Consortium and TOSCA Investigators, Touraine, Renaud, Youroukos, Sotiris, Zonnenberg, Bernard   +29 more
core   +4 more sources

BRAF V600E–Mutant Diffuse Pulmonary Langerhans Cell Histiocytosis Successfully Treated With Dabrafenib Plus Trametinib: A Case Report

Respirology Case Reports, Volume 13, Issue 9, September 2025.
We report the case of a 30‐year‐old woman diagnosed with BRAF V600E–mutant pulmonary Langerhans cell histiocytosis who was treated with dabrafenib in combination with trametinib. Chest computed tomography performed 3 months after the initiation of therapy demonstrated improvement in pericystic opacities.
Akiko Sakurai, Tetsutaro Nagaoka, Chisato Tadokoro, Shunichi Kataoka, Yuriko Terayama, Yuichi Nagata, Yoshifumi Suzuki, Taichi Miyawaki, Motoyasu Kato, Takuo Hayashi, Kazuhisa Takahashi   +10 more
wiley   +1 more source

Lymphangioleiomyomatosis and mTOR inhibitors in real world—a narrative review [PDF]

, 2023
Sanghoon Park, Eun Joo Lee
openalex   +1 more source

Deformable image registration of dark‐field chest radiographs for functional lung assessment

Medical Physics, Volume 52, Issue 8, August 2025.
Abstract Background Dark‐field radiography of the human chest has been demonstrated to have promising potential for the analysis of the lung microstructure and the diagnosis of respiratory diseases. However, most previous studies of dark‐field chest radiographs evaluated the lung signal only in the inspiratory breathing state.
Fabian Drexel, Vasiliki Sideri‐Lampretsa, Henriette Bast, Alexander W. Marka, Thomas Koehler, Florian T. Gassert, Daniela Pfeiffer, Daniel Rueckert, Franz Pfeiffer   +8 more
wiley   +1 more source

Everolimus dosing recommendations for tuberous sclerosis complex–associated refractory seizures [PDF]

, 2018
ObjectiveThe present analysis examined the exposure-response relationship by means of the predose everolimus concentration (C-min) and the seizure response in patients with tuberous sclerosis complex-associated seizures in the EXIST-3 study ...
Brandt, Christian, Cheung, Wing, Coello, Neva, Franz, David N, French, Jacqueline A, Grosch, Kai, Kohrman, Michael H, Lawson, John A, Milh, Mathieu, Voi, Maurizio, Wiemer-Kruel, Adelheid, Wong, Michael, Yapici, Zuhal   +12 more
core   +2 more sources

Sporadic lymphangioleiomyomatosis with multiple atypical features: A case report and literature review

Indian Journal of Pathology and Microbiology, 2018
Lymphangioleiomyomatosis (LAM) is a rare, genetically determined, progressive interstitial lung disease, which almost exclusively affects women, especially at the childbearing age.
Xiaokang Wang, Fei Su, Fenfang Zhou, Maohui Feng   +3 more
doaj   +1 more source

Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis [PDF]

, 2017
Background: Understanding the true prevalence of lymphangioleiomyomatosis (LAM) is important in estimating disease burden and targeting specific interventions.
Byrnes, S., Chang, W.Y.C., Cohen, M.M., Geiling, S., Gray, B., Harknett, E.C., Hubbard, R.B., Johnson, J., Johnson, S.R., Lazor, R., Tattersfield, A.E., Telford, H.   +11 more
core  

Fatty images of the heart: spectrum of normal and pathological findings at computed tomography and cardiac magnetic resonance imaging. [PDF]

, 2018
Ectopic cardiac fatty images are not rarely detected incidentally at computed tomography and cardiac magnetic resonance, either in exams focused on the heart as in general thoracic imaging evaluations.
Cannavale, Giuseppe, Carbone, Iacopo, Catalano, Carlo, Francone, Marco, Galea, Nicola, Molisso, Antonio, Vullo, Francesco   +6 more
core   +3 more sources

Pneumothorax as a Presentation of Tuberous Sclerosis-Associated Lymphangioleiomyomatosis

European Journal of Case Reports in Internal Medicine, 2019
Tuberous sclerosis complex (TSC) is a rare, autosomal dominant disorder with a recognized phenotypic variability with clinical manifestations developing continuously throughout life. The follow-up of TSC patients is challenging.
Duarte Lages Silva, Pedro Pinto, Miguel Costa, Rosana Maia, Carmelia Rodrigues   +4 more
doaj   +1 more source