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Lymphangioleiomyomatos is an update

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During the last 5 years there have been important advances in understanding the disease and lymphangioleiomyomatosis (LAM) treatment, even if there have been difficulties regarding clinical research in this area.
The results of research studies on LAM were influenced by the small number of cases and slow evolution in time. LAM is a rare disease, with a chronic course, that occurs in women during the fertile period. The etiology is unknown. The disease is caused by the non malignant proliferation in lymphatic vessel walls, blood vessels and small airways by the smooth muscle like cells (LAM cells) and by epithelioid cell proliferation around broncho vascular structures. Patients become symptomatic after a long period of evolution.
The case is discovered on the occurrence of pneumothorax or chylothorax or after investigating progressive dyspnea on effort in women. During the evolution there is a functional decline in FEV1 (flow expiratory volume in one second) and DLCO (diffusing capacity for carbon monoxide). The presence of bilateral multiple thin wall cysts is considered a specific feature of HRCT. LAM may evolve independently (sporadic LAM) or may accompany the genetic disease tuberous sclerosis complex (TSC LAM). In LAM pathogenesis, dysregulation of the kinase mammalian target of rapamycin (mTOR0 signaling plays an important role, as a result of mutation in TSC1 (hamartin) level and TSC2 gene (tuberin). More and more studies have proven the efficacy of specific inhibitors of mTOR such as rapamycin (Sirolimus). Lung transplantation remains indication for LAM.

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