Cathepsin K in lymphangioleiomyomatosis: LAM cell-fibroblast Interactions enhance protease activity by extracellular acidification [PDF]
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+53 more
core +2 more sources
A 47 yo woman underwent resection of an endometrial adenocarcinoma with pelvic lymph node biopsies. She was found to have incidental nodal lymphangioleiomyomatosis (LAM).
Charles M. Lombard
doaj
Highlights of the Brazilian Thoracic Association guidelines for interstitial lung diseases [PDF]
Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists.
Aidé, Miguel Abidon+24 more
core +3 more sources
Meningiomas in Lymphangioleiomyomatosis [PDF]
Lymphangioleiomyomatosis (LAM), a cystic lung disease associated with progressive respiratory failure, is found predominantly in women of childbearing age and therefore has been treated with progesterone and other hormonal agents. However, meningiomas have progesterone receptors, and progesterone is believed to be a mitogen for meningioma cells in ...
Angelo M. Taveira-DaSilva+3 more
openaire +3 more sources
Protease activity sensors enable real-time treatment response monitoring in lymphangioleiomyomatosis
Background Biomarkers of disease progression and treatment response are urgently needed for patients with lymphangioleiomyomatosis (LAM). Activity-based nanosensors, an emerging biosensor class, detect dysregulated proteases in vivo and release a ...
Jesse D. Kirkpatrick+7 more
semanticscholar +1 more source
Epitheloid Variant of Angiomyolipoma in a Patient with Tuberous Sclerosis. [PDF]
Epitheloid angiomyolipoma of kidney is a type of Perivascular endothelial cell derived tumor with an aggressive behaviour with specific pathological, immunohistochemistrical and genetic characteristics.
Amirtham, U+4 more
core +1 more source
Advanced gynecologic malignancies treated with a combination of the VEGF inhibitor bevacizumab and the mTOR inhibitor temsirolimus. [PDF]
BackgroundBevacizumab and temsirolimus are active agents in gynecologic tumors. Temsirolimus attenuates upregulation of HIF-1α levels, a resistance mechanism for antiangiogenics, and targets the PI3-kinase/AKT/mTOR axis, commonly aberrant in these tumors.
Falchook, Gerald S+9 more
core +3 more sources
CrossTORC and WNTegration in Disease: Focus on Lymphangioleiomyomatosis
The mechanistic target of rapamycin (mTOR) and wingless-related integration site (Wnt) signal transduction networks are evolutionarily conserved mammalian growth and cellular development networks. Most cells express many of the proteins in both pathways,
Jilly F. Evans+3 more
semanticscholar +1 more source
ABSTRACT Cardiac rhabdomyomas are often the presenting sign of tuberous sclerosis complex (TSC). Prior reports have shown that maternal sirolimus treatment can reduce rhabdomyomas. We used maternal sirolimus to reverse hydrops fetalis due to a massive cardiac rhabdomyoma in a twin gestation.
David M. Ritter+6 more
wiley +1 more source
The UK guidelines for management and surveillance of Tuberous Sclerosis Complex. [PDF]
Background: The severity of Tuberous Sclerosis Complex (TSC) can vary among affected individuals. Complications of TSC can be life threatening, with significant impact on patients' quality of life.
Amin, S+11 more
core +3 more sources