Presse Med
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Lymphangioleiomyomatosis is a rare pulmonary disease encountered almost exclusively in women of reproductive age. Pulmonary involvement is characterized by multiple thin-walled cysts in the lungs, recurrent pneumothorax, obstructive lung disorders, and progression to chronic respiratory failure over a mean period of 10 years. Certainty of diagnosis requires a lung biopsy, but international criteria have been proposed for a diagnosis without such a biopsy. ⋯ Hormone treatment is not recommended. Hopes are high for mTor inhibitors (sirolimus and everolimus) and treatment trials are currently underway. Lung transplantation must be considered when chronic respiratory failure occurs in patients younger than 60 years.
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Aneurysms of the ascending aorta carry a high risk of aortic dissection or rupture in the absence of surgical treatment. Aneurysms of the ascending aorta are classified into 2 distinct types according to their etiology and surgical treatment: aortic root aneurysms, in which the dilatation of the aorta involves the initial portion of the aorta and includes the sinuses of Valsalva and supravalvular aortic aneurysms, which involve the ascending thoracic aorta above the sinuses of Valsalva. Echocardiography and CT scan are the main exams in the evaluation of the diameter of the ascending aorta and its follow up. ⋯ Beyond 50mm, an aneurysm is at risk for complications. Supravalvular aortic aneurysms can be treated by a simple supracoronary tube graft unlike aortic root aneurysms in which replacement or repair of the aortic valve is often necessary. A family history and investigation of other members is essential in patients with aortic aneurysms.
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Idiopathic pulmonary fibrosis is the most common of the idiopathic interstitial pneumonias. It occurs more often in men than in women and in those older than 65 years than in younger people. It is strongly associated with smoking. ⋯ Disease exacerbations contribute to deterioration and to death. There is no effective treatment for this disease. Median survival is about 2-3 years after diagnosis.