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Synergistic effects of mTOR inhibitors with VEGFR3 inhibitors on the interaction between TSC2-mutated cells and lymphatic endothelial cells.
Sci China Life Sci
January 2025
Department of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.
Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease affecting the lung, kidney, and lymphatic system with a molecular mechanism of tuberous sclerosis complex 2 (TSC2) mutations. Vascular endothelial growth factor D (VEGF-D), a ligand for vascular endothelial growth factor receptor 3 (VEGFR3), is a diagnostic biomarker of LAM and is associated with lymphatic circulation abnormalities. This study explored the interaction between LAM cells and lymphatic endothelial cells (LECs) and the effects of rapamycin on this interaction, which may help to identify new targets for LAM treatment.
View Article and Find Full Text PDFLymphangioleiomyomatosis and Pregnancy-Do We Have All the Answers for a Woman Who Desires to Conceive?-Literature Review.
Cancers (Basel)
January 2025
Department of Obstetrics and Gynecology, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania.
Lymphangioleiomyomatosis (LAM) is a rare, progressive, and poor-prognosis systemic disorder that primarily affects women of reproductive age, with a higher prevalence among individuals of Caucasian origin. However, there are limited reliable data on the prevalence of LAM during pregnancy. The fulminant respiratory clinical presentation that often includes progressive dyspnea on exertion, cough, or hemoptysis, frequently complicated by pneumothorax, and the increased risk of spontaneous abortion due to increased estrogen and progesterone production during gestation, are arguments that most often make the diagnosed woman avoid pregnancy.
View Article and Find Full Text PDFCharacteristics and outcomes of patients with LAM receiving sirolimus in France based on real-life data.
Front Med (Lausanne)
January 2025
Department of Respiratory Medicine, Reference Centre for Rare Pulmonary Diseases, APHP, Hôpital Avicenne, INSERM U 1272, Université Sorbonne Paris-Nord, Bobigny, France.
LAM is a rare multi-cystic lung disease for which treatment with sirolimus is indicated in cases of moderate or severe lung disease or declining lung function. The aim of this study was to describe patients treated with sirolimus for LAM and their outcomes. This retrospective observational study was based on data from the French national health insurance data system (SNDS).
View Article and Find Full Text PDFThirty years of lung transplantation: development of postoperative outcome and survival over three decades.
J Thorac Dis
December 2024
Division of Thoracic Surgery, University Hospital of Munich, LMU, Munich, Germany.
Background: Lung transplantation (LuTX) can be the last resort for patients with end-stage lung diseases. In the last decades, improvements were implemented in transplant medicine, from immunosuppression throughout preservation of the donor organ to enhance lung allograft survival. This retrospective study aims to illustrate the development of the LuTX-program at the University Hospital of Munich, LMU, Munich, Germany, since its launch in 1990 by depicting and comparing postoperative outcome.
View Article and Find Full Text PDFImmature Smooth Muscle Infiltration in Sporadic Lymphangioleiomyomatosis: A Case Study.
S D Med
November 2024
Sanford USD Medical Center, Sioux Falls, South Dakota.
Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that causes progressive pulmonary damage. It typically affects young reproductive-age females with tuberous sclerosis complex (TSC). The clinical manifestations of LAM result from the progressive invasion of abnormal smooth muscle cells into lung parenchyma, lymphatics, or pulmonary vasculature.
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