Successful treatment of a patient with chyluria due to lymphangioleiomyomatosis using sirolimus
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چکیده
منابع مشابه
Successful treatment of a patient with chyluria due to lymphangioleiomyomatosis using sirolimus
Lymphangioleiomyomatosis (LAM) is a rare and progressive neoplastic disease of young woman, characterized by the proliferation of abnormal smooth muscle-like cells (LAM cells) in the lungs and axial lymphatics. A 44-year-old woman was referred to our hospital because pleural effusion was detected during a health checkup. She had chylothorax, chylous ascites, and chyluria, and her computed tomog...
متن کاملLymphangioleiomyomatosis treatment with sirolimus.
Lymphangioleiomyomatosis (LAM) is a rare lung disease, that predominantly affects young females and generally progresses to respiratory failure. There is not sufficient evidence to support the routine use of any treatment in LAM. The only treatment for severe LAM is currently lung transplantation. Activation of mammalian target of rapamycin (mTOR) signalling pathway has been observed in LAM. LA...
متن کاملEfficacy of hormonal suppression in a patient with chyluria due to lymphangioleiomyomatosis
Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting almost exclusively young women, characterised by abnormal proliferation of atypical smooth muscle cells. We describe a young woman presenting with chyluria secondary to the presence of a large retroperitoneal lymphangioleiomyoma. Immunohistochemical analysis revealed HMB45-negative LAM cells (HMB45 staining is absent only in rare c...
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We report a successful pregnancy in a patient with longstanding LAM on treatment with sirolimus. During temporary discontinuation fo sirolimus in early pregnancy, lung function declined but recovered after resumption of sirolimus. Pregnancy was complicated by a persistent pneumothorax which was treated surgically postnatally. The child has had a normal development despite exposure to low dose s...
متن کاملEffects of sirolimus on Lung function in patients with Lymphangioleiomyomatosis
Background: Lymphangioleiomyomatosis (LAM) is a progressive lungs disease that affects women at reproductive years. Sirolimus inhibits mammalian target of rapamycin (mTOR) and its administration in past studies was hopeful in treatment of patients with LAM. The aim of this study was to evaluate sirolimus therapy on lung function in LAM patients. Methods: We conducted a trial to evaluate the ef...
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ژورنال
عنوان ژورنال: Respiratory Medicine Case Reports
سال: 2018
ISSN: 2213-0071
DOI: 10.1016/j.rmcr.2018.01.002