يعرض 1 - 10 نتائج من 69 نتيجة بحث عن '"Pulmonary lymphangiomyomatosis"', وقت الاستعلام: 0.67s تنقيح النتائج
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    دورية أكاديمية

    المصدر: Orphanet Journal of Rare Diseases, Vol 17, Iss 1, Pp 1-7 (2022)

    الوصف: Abstract Background Pulmonary lymphangiomyomatosis (PLAM) is a rare interstitial lung disease characterized by diffuse cystic changes caused by the destructive proliferation of smooth muscle-like cells or LAM cells. PLAM is more common in young women than other people, and a consensus is lacking regarding PLAM treatment. The clinical treatment of PLAM is currently dominated by rapamycin. By inhibiting the mTOR signaling pathway, rapamycin can inhibit and delay PLAM’s occurrence and development. However, the application of rapamycin also has shortcomings, including the drug’s low oral bioavailability and a high binding rate to hemoglobin, thus significantly decreasing the amount of drug distributed to the lungs. Methods and results Here, we developed a new mode of rapamycin administration in which the drug was injected into the intrathecal space after being nanosized; the directional flow characteristics of the liquid in the intrathecal space were exploited to increase the drug content in the interstitial fluid to the greatest extent possible. We studied the rapamycin content in the interstitial fluid and blood after intervaginal space injection (ISI). Compared with oral administration, ISI significantly increased the drug concentration in the lung interstitial fluid. Conclusions These results provided new ideas for treating PLAM and optimizing the dosing regimens of drugs with similar characteristics to rapamycin.

    وصف الملف: electronic resource

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    دورية أكاديمية

    المصدر: International Journal of Health and Biological Sciences; Vol. 3 No. 02 (2020): International Journal of Health and Biological Sciences; 06-08 ; 2590-3357 ; 2590-3365

    الوصف: Lymphangiomyomatosisis a rare disease characterized by a proliferation of abnormal smooth muscle cells responsible for infiltration with the destruction of tissue architecture and genesis of cystic lung and lymphatic lesions. In addition to lung damage, Bourneville’s tuberous sclerosis (BTS) also affects the skin, brain, retina, kidneys, and, less frequently, the heart and bone. We report the case of a young patient with bilateral pneumothorax revealing pulmonary lymphangiomyomatosis in the context of Bourneville’stuberoussclerosis BTS.

    وصف الملف: application/pdf

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