Revue des maladies respiratoires
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The occurrence of pulmonary fibrosis in numerous individuals from the same family suggests a genetic cause for the disease. During the last 10 years, mutations involving proteins from the telomerase complex and from the surfactant system have been identified in association with pulmonary fibrosis. Mutations of TERT, the coding gene for the telomerase reverse transcriptase, are the most frequently identified mutations and are present in 15% of cases of familial pulmonary fibrosis. ⋯ Other genetic variations associated with pulmonary fibrosis such as a polymorphism in the promoter of MUC5B or a polymorphism in TERT have been recently described, and could be considered to be part of a polygenic transmission. Evidence for mutations associated with the development of pulmonary fibrosis raises numerous clinical questions from establishing a diagnosis, providing counselling to deciding on therapy, and requires specific studies. From a pathophysiological point of view, the function of the genes highlights the central role of alveolar epithelium and aging in fibrogenesis.
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Mechanical ventilation is associated with ventilator-induced diaphragmatic dysfunction (VIDD) in animal models and also in humans. ⋯ At this date, only spontaneous ventilatory cycles and perhaps phrenic nerve stimulation appear to diminish the severity of VIDD in humans but several pathways are currently being examined using animal models. Specific pharmacological options are currently under investigation in animal models.
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Extra-corporeal membrane oxygenation (ECMO) effectively replaces the lung in providing oxygenation and carbon dioxide (CO2) removal. For some years, and in parallel to the H1N1 influenza pandemic, this technique has gained interest in relation to significant technological improvements, leading to new concepts of "awake and mobile ECMO" or rehabilitation with ECMO. ⋯ This general review aims to outline the definition, classification and principles of ECMO and to give some current information about the indications and possibilities of the technique to the pulmonologist and intensivist. Further possible uses for this technique include extra-corporeal removal of CO2 during hypercapnic respiratory failure and assistance during lung transplantation from the preoperative to the early postoperative period.