Emerging clinical picture of lymphangioleiomyomatosis

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Emerging clinical picture of lymphangioleiomyomatosis.

OBJECTIVE To provide a comprehensive update of the clinical picture of lymphangioleiomyomatosis (LAM) using two large patient registries. METHODS A cross sectional questionnaire survey which included questions on 14 LAM symptoms, pneumothorax, tuberous sclerosis complex (TSC), date of diagnosis, and pulmonary function tests (PFTs). RESULTS The response rate was 70.5% (n = 328). The mean age...

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ORPHAN LUNG DISEASE Emerging clinical picture of lymphangioleiomyomatosis

Objective: To provide a comprehensive update of the clinical picture of lymphangioleiomyomatosis (LAM) using two large patient registries. Methods: A cross sectional questionnaire survey which included questions on 14 LAM symptoms, pneumothorax, tuberous sclerosis complex (TSC), date of diagnosis, and pulmonary function tests (PFTs). Results: The response rate was 70.5% (n = 328). The mean age ...

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Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a multisystem disease of women, characterized by proliferation of abnormal smooth muscle-like LAM cells, leading to the formation of lung cysts, fluid-filled cystic structures in the axial lymphatics (eg, lymphangioleiomyomas), and renal angiomyolipomas. LAM is caused by mutations of the TSC1 or TSC2 genes, which encode, respectively, hamartin and tuberin, two ...

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Clinical experience of lymphangioleiomyomatosis in the UK.

BACKGROUND Lymphangioleiomyomatosis is a rare lung disease that affects only women. No controlled trials of management have been performed and, until such data are available, management must be based on clinical experience. This study provides data on the natural history of lymphangioleiomyomatosis in the UK and compares this with experience from other centres. METHODS We tried to identify al...

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Clinical utility of RAS mutations in thyroid cancer: a blurred picture now emerging clearer.

RAS mutations play an important role in thyroid tumorigenesis. Considerable effort has been made in the last decade to apply RAS mutations as molecular markers to the clinical management of thyroid nodules and thyroid cancer. Yet, for the low diagnostic sensitivities and specificities of RAS mutations, when used alone, and for their uncertain role in the clinical outcomes of thyroid cancer, it ...

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ژورنال

عنوان ژورنال: Thorax

سال: 2005

ISSN: 0040-6376

DOI: 10.1136/thx.2004.035154