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lymphangioleiomyomatosis/diarrhea

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8 結果

Doxycycline use in patients with lymphangioleiomyomatosis: biomarkers and pulmonary function response.

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OBJECTIVE To assess blockade of matrix metalloproteinase (MMP)-2 and MMP-9, as well as the variation in FEV1, in patients with lymphangioleiomyomatosis (LAM) treated with doxycycline (a known MMP inhibitor) for 12 months. METHODS An open-label, single-arm, interventional clinical trial in which LAM

Doxycycline use in patients with lymphangioleiomyomatosis: safety and efficacy in metalloproteinase blockade.

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OBJECTIVE Lymphangioleiomyomatosis (LAM) is characterized by lung cysts, whose development is associated with matrix metalloproteinase (MMP) hyperactivity, principally that of MMP-2 and MMP-9. Our objective was to compare LAM patients and controls in terms of the levels of these MMPs, as well as to

Sirolimus and Autophagy Inhibition in Lymphangioleiomyomatosis: Results of a Phase I Clinical Trial.

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Animal and cellular studies support the importance of autophagy inhibition in lymphangioleiomyomatosis (LAM). In a cohort of subjects with LAM, we tested the hypothesis that treatment with sirolimus and hydroxychloroquine (an autophagy inhibitor) at two different dose levels is safe and well
The mechanistic target of rapamycin inhibitors (mTORi) sirolimus and everolimus stabilize lung function in patients with pulmonary lymphangioleiomyomatosis (LAM) but do not induce remission. Pre-clinical studies suggest that simvastatin in combination with sirolimus induces LAM cell

Retrospective review of combined sirolimus and simvastatin therapy in lymphangioleiomyomatosis.

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BACKGROUND Combined simvastatin and sirolimus therapy reduces tuberous sclerosis complex 2-null lesions and alveolar destruction in a mouse model of lymphangioleiomyomatosis (LAM), suggesting that therapy with both drugs may benefit patients with LAM. METHODS To determine whether simvastatin changed

Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.

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BACKGROUND Angiomyolipomas in patients with the tuberous sclerosis complex or sporadic lymphangioleiomyomatosis are associated with mutations in tuberous sclerosis genes resulting in constitutive activation of the mammalian target of rapamycin (mTOR). The drug sirolimus suppresses mTOR

Pharmaceutical Approval Update.

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Eluxadoline (Viberzi) for irritable bowel syndrome with diarrhea; tiotropium bromide/olodaterol inhalation spray (Stiolto Respimat) for chronic obstructive pulmonary disease; and sirolimus (Rapamune) for lymphangioleiomyomatosis.

Cannabidiol Elevates Mechanistic Target of Rapamycin Inhibitor Levels in Patients With Tuberous Sclerosis Complex.

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The mechanistic target of rapamycin inhibitors everolimus and sirolimus have activity against multiple manifestations of tuberous sclerosis complex and are approved to treat astrocytomas, angiomyolipomas, lymphangioleiomyomatosis, and epilepsy. Cannabidiol is a novel antiepileptic
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