منابع مشابه
Adolescent form of sporadic lymphangioleiomyomatosis (S-LAM).
1. Kücüksezer UC, Palomares O, Rückert B, Jartti T, Puhakka T, Nandy A, et al. Triggering of specific toll-like receptors and proinflammatory cytokines breaks allergen-specific T-cell tolerance in human tonsils and peripheral blood. J Allergy Clin Immunol. 2013;131:875--85. 2. Palomares O, Rückert B, Jartti T, Kücüksezer UC, Puhakka T, Gomez E, et al. Induction and maintenance of allergen-speci...
متن کاملAdolescent form of sporadic lymphangioleiomyomatosis ( S - LAM )
1. Kücüksezer UC, Palomares O, Rückert B, Jartti T, Puhakka T, Nandy A, et al. Triggering of specific toll-like receptors and proinflammatory cytokines breaks allergen-specific T-cell tolerance in human tonsils and peripheral blood. J Allergy Clin Immunol. 2013;131:875--85. 2. Palomares O, Rückert B, Jartti T, Kücüksezer UC, Puhakka T, Gomez E, et al. Induction and maintenance of allergen-speci...
متن کاملSirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: a phase 2 trial.
PURPOSE Renal angiomyolipomas are a frequent manifestation of tuberous sclerosis and sporadic lymphangioleiomyomatosis (LAM). These disorders are associated with mutations of TSC1 or TSC2 that lead to overactivation of mTOR complex 1 (mTORC1), suggesting an opportunity for targeted therapy by using mTORC1 inhibitors. This study investigated the efficacy and safety of the mTORC1 inhibitor siroli...
متن کاملMutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis.
Lymphangioleiomyomatosis (LAM) is a progressive and often fatal interstitial lung disease characterized by a diffuse proliferation of abnormal smooth muscle cells in the lungs. LAM is of unusual interest biologically because it affects almost exclusively young women. LAM can occur as an isolated disorder (sporadic LAM) or in association with tuberous sclerosis complex. Renal angiomyolipomas, wh...
متن کاملEverolimus treatment of abdominal lymphangioleiomyoma in five women with sporadic lymphangioleiomyomatosis.
OBJECTIVE Lymphangioleiomyomatosis (LAM) is a rare systemic disease of young women arising from mutations in the tuberous sclerosis complex (TSC) genes, TSC1 or TSC2. This disrupts the mammalian target of rapamycin (mTOR) pathway, affecting cellular proliferation and growth. mTOR inhibitors are a promising novel therapy in LAM. The mTOR inhibitor sirolimus is reported to produce resolution of l...
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ژورنال
عنوان ژورنال: QJM
سال: 2014
ISSN: 1460-2725,1460-2393
DOI: 10.1093/qjmed/hcu019