Sustained effects of sirolimus on lung function and cystic lung lesions in lymphangioleiomyomatosis
…, AM Jones, P Julien-Williams… - American journal of …, 2014 - atsjournals.org
Rationale: Sirolimus therapy stabilizes lung function and reduces the size of chylous effusions
and lymphangioleiomyomas in patients with lymphangioleiomyomatosis. Objectives: To …
and lymphangioleiomyomas in patients with lymphangioleiomyomatosis. Objectives: To …
Sirolimus and autophagy inhibition in lymphangioleiomyomatosis: results of a phase I clinical trial
…, M Haughey, D Bienfang, AM Jones, P Julien-Williams… - Chest, 2017 - Elsevier
Background Animal and cellular studies support the importance of autophagy inhibition in
lymphangioleiomyomatosis (LAM). In a cohort of subjects with LAM, we tested the hypothesis …
lymphangioleiomyomatosis (LAM). In a cohort of subjects with LAM, we tested the hypothesis …
Long-term effect of sirolimus on serum vascular endothelial growth factor D levels in patients with lymphangioleiomyomatosis
AM Taveira-DaSilva, AM Jones, P Julien-Williams… - Chest, 2018 - Elsevier
Background Sirolimus reduces serum levels of vascular endothelial growth factor D (VEGF-D);
the size of chylous effusions, lymphangioleiomyomas, and angiomyolipomas; and …
the size of chylous effusions, lymphangioleiomyomas, and angiomyolipomas; and …
[HTML][HTML] Phenotypic distinctions between mosaic forms of tuberous sclerosis complex
…, O Oyerinde, S Raiciulescu, P Julien-Williams… - Genetics in …, 2019 - Elsevier
Purpose To determine if mosaic tuberous sclerosis complex (TSC) can be stratified into
subtypes that correspond with prognosis and extent of disease. Methods Next-generation …
subtypes that correspond with prognosis and extent of disease. Methods Next-generation …
Severity and outcome of cystic lung disease in women with tuberous sclerosis complex
…, AM Jones, P Julien-Williams… - European …, 2015 - Eur Respiratory Soc
What are the clinical features, severity, and rate of progression of lung disease in women
with tuberous sclerosis and lymphangioleiomyomatosis (LAM) and how do they differ from …
with tuberous sclerosis and lymphangioleiomyomatosis (LAM) and how do they differ from …
Assessment of lung structure and regional function using 0.55 T MRI in patients with lymphangioleiomyomatosis
…, T Machado, A Jones, P Julien-Williams… - Investigative …, 2022 - journals.lww.com
Objectives Contemporary lower-field magnetic resonance imaging (MRI) may offer advantages
for lung imaging by virtue of the improved field homogeneity. The aim of this study was to …
for lung imaging by virtue of the improved field homogeneity. The aim of this study was to …
Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts
AM Taveira-DaSilva, SR Johnson, P Julien-Williams… - Thorax, 2020 - thorax.bmj.com
Pregnancy in women with lymphangioleiomyomatosis (LAM) has been associated with
increased complications and worsening lung function although objective data to advise patients …
increased complications and worsening lung function although objective data to advise patients …
CA-125 in disease progression and treatment of lymphangioleiomyomatosis
…, WK Steagall, ME Haughey, PA Julien-Williams… - Chest, 2018 - Elsevier
Background Lymphangioleiomyomatosis (LAM) is a destructive lung disease of women
caused by proliferation of neoplastic-like LAM cells, with mutations in the TSC1/2 tumor …
caused by proliferation of neoplastic-like LAM cells, with mutations in the TSC1/2 tumor …
Chest CT scan at radiation dose of a posteroanterior and lateral chest radiograph series: a proof of principle in lymphangioleiomyomatosis
…, J Yao, T Machado, AM Jones, P Julien-Williams… - Chest, 2019 - Elsevier
Background Given the rising utilization of medical imaging and the risks of radiation, there is
increased interest in reducing radiation exposure. The objective of this study was to evaluate…
increased interest in reducing radiation exposure. The objective of this study was to evaluate…
Retrospective review of combined sirolimus and simvastatin therapy in lymphangioleiomyomatosis
AM Taveira-DaSilva, AM Jones, PA Julien-Williams… - Chest, 2015 - Elsevier
… Jones CRNP a , Patricia A. Julien-Williams CRNP a , Mario Stylianou PhD b , Joel Moss
MD, PhD, FCCP a …
MD, PhD, FCCP a …