منابع مشابه
Pulmonary Lymphangioleiomyomatosis *
Lymphangioleiomyomatosis (LAM) is a rare lung disease of unknown etiology that primarily affects women of childbearing age. Clinically, it manifests as progressive dyspnea, hemoptysis, pneumothorax and chylous pleural effusion resulting from abnormal smooth muscle proliferation in the lung parenchyma, lymph nodes, small airways and blood vessels. Recent cytogenetic studies have disclosed mutati...
متن کاملManagement of lymphangioleiomyomatosis
Lymphangioleiomyomatosis (LAM), a multisystem disease affecting almost exclusively women, is characterized by cystic lung destruction and presents with dyspnea, recurrent pneumothoraxes, chylous effusions, lymphangioleiomyomas, and angiomyolipomas. It is caused by the proliferation of a cancer-like LAM cell that possesses a mutation in either the tuberous sclerosis complex (TSC)1 or TSC2 genes....
متن کاملExtrathoracic angiomyolipomas in lymphangioleiomyomatosis.
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder that affects women and can lead to serious respiratory impairment. Since Bourneville's tuberous sclerosis (TS) was first reported, the striking similarities between the two entities have led many to believe that LAM is a forme fruste of TS. This is suggested by reports that angiomyolipomas, rare tumours in themselves, are reported in 4...
متن کاملMeningiomas in lymphangioleiomyomatosis.
CONTEXT Lymphangioleiomyomatosis (LAM), a cystic lung disease associated with progressive respiratory failure, is found predominantly in women of childbearing age and therefore has been treated with progesterone and other hormonal agents. However, meningiomas have progesterone receptors, and progesterone is believed to be a mitogen for meningioma cells in culture. Since 30% to 40% of patients w...
متن کاملMilestones in lymphangioleiomyomatosis research.
L ymphangioleiomyomatosis (LAM), a slowly progressive disease affecting mostly young and middle-aged females [1], is one of the few orphan lung diseases that has attracted major attention in recent years from both clinicians and researchers [2], with rapidly evolving progress. With an estimated prevalence of one in 400,000 adult females aged 20–69 yrs for the sporadic form [3] and ,30% of patie...
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ژورنال
عنوان ژورنال: Nihon Naika Gakkai Zasshi
سال: 2012
ISSN: 0021-5384,1883-2083
DOI: 10.2169/naika.101.138b