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European respiratory society guidelines for the diagnosis and management of lymphangioleiomyomatosis
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A recent evidence-based, consensus guidelines for the diagnosis, assessment and treatment of LAM
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Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J 2010;35:14-26. A recent evidence-based, consensus guidelines for the diagnosis, assessment and treatment of LAM.
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Abnormal growth of smooth muscle-like cells in lymphangioleiomyomatosis: Role for tumor suppressor TSC2
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Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis
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Sirolimus therapy in tuberous sclerosis or sporadic lymphangioleiomyomatosis
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Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: A phase 2 trial
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trial demonstrates that sirolimus could reduce the size of angiomyolipoma in LAM. No effect was observed on lung function in this small series
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Davies DM, de Vries PJ, Johnson SR, et al. Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: a phase 2 trial. Clin Cancer Res 2011;17:4071-4081. The trial demonstrates that sirolimus could reduce the size of angiomyolipoma in LAM. No effect was observed on lung function in this small series.
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Efficacy and safety of sirolimus in lymphangioleiomyomatosis
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first placebo controlled randomized trial with sirolimus for pulmonary LAM demonstrates lung function stabilization with treatment, and the need for long-term administration to maintain this effect
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McCormack FX, Inoue Y, Moss J, et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med 2011;364:1595-1606. The first placebo controlled randomized trial with sirolimus for pulmonary LAM demonstrates lung function stabilization with treatment, and the need for long-term administration to maintain this effect.
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Selective inhibition of growth of Tuberous Sclerosis complex 2-null cells by atorvastatin is associated with impaired Rheb and Rho GTPase function and reduced mTOR/S6 kinase activity
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Dramatic effect of sirolimus on renal angiomyolipomas in a patient with tuberous sclerosis complex
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Herry I, Neukirch C, Debray MP, et al. Dramatic effect of sirolimus on renal angiomyolipomas in a patient with tuberous sclerosis complex. Eur J Intern Med 2007;18:76-77. (Pubitemid 46091655)
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Activation of the estrogen receptor contributes to the progression of pulmonary lymphangioleiomyomatosis via matrix metalloproteinase-induced cell invasiveness
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Glassberg MK, Elliot SJ, Fritz J, et al. Activation of the estrogen receptor contributes to the progression of pulmonary lymphangioleiomyomatosis via matrix metalloproteinase-induced cell invasiveness. J Clin Endocrinol Metab 2008;93:1625-1633. (Pubitemid 351656497)
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Effect of doxycycline on proliferation, MMP production, and adhesion in LAM-related cells
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Chang WY, Clements D, Johnson SR. Effect of doxycycline on proliferation, MMP production, and adhesion in LAM-related cells. Am J Physiol Lung Cell Mol Physiol 2010;299:L393-400.
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Doxycycline inhibits matrix metalloproteinase-2 secretion from TSC2-null mouse embryonic fibroblasts and lymphangioleiomyomatosis cells
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Moir L, Ng H, Poniris M, et al. Doxycycline inhibits matrix metalloproteinase-2 secretion from TSC2-null mouse embryonic fibroblasts and lymphangioleiomyomatosis cells. Br J Pharmacol 2011. doi:10.1111/j.1476-5381. 2011. 01344.x. [Epub ahead of print].
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Serum vascular endothelial growth factor-D levels in patients with lymphangioleiomyomatosis reflect lymphatic involvement
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Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases
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This prospective study provides evidence on the role of serum levels of VEGF-D to identify LAM among patients with other cystic lung diseases
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Young LR, Vandyke R, Gulleman PM, et al. Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest 2010;138:674-681. This prospective study provides evidence on the role of serum levels of VEGF-D to identify LAM among patients with other cystic lung diseases.
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An experience with the modified total pleural covering technique in a patient with bilateral intractable pneumothorax secondary to lymphangioleiomyomatosis
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