Pneumologie
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Personalized medicine promises that medical decisions, practices and products are tailored to the individual patient. Cystic fibrosis, an inherited disorder of chloride and bicarbonate transport in exocrine glands, is the first successful example of customized drug development for mutation-specific therapy. There are two classes of CFTR modulators: potentiators that increase the activity of CFTR at the cell surface, and correctors that either promote the read-through of nonsense mutations or facilitate the translation, folding, maturation and trafficking of mutant CFTR to the cell surface. ⋯ Phe508del, respectively. This report provides an overview of the basic defect in cystic fibrosis, the population genetics of CFTR mutations in Germany and the bioassays to assess CFTR function in humans together with the major achievements of preclinical research and clinical trials to bring CFTR modulators to the clinic. Some practical information on the use of ivacaftor and lumacaftor in daily practice and an update on pitfalls, challenges and novel strategies of bench-to-bedside development of CFTR modulators are also provided.
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The therapy of choice in hypoxemic respiratory failure (type 1) is the application of supplemental oxygen at flow rates of 1 to 15 l/min via nasal prongs or mask. Non-invasive or invasive positive pressure ventilation will be initiated when the oxygen therapy effects are not sufficient or if hypercapnic respiratory failure (type 2) is the underlying problem. Recently, an alternative therapy option is available, from the pathophysiology it can be classified between oxygen therapy and positive pressure ventilation. ⋯ Thus the NHF therapy attracts attention in intensive care medicine. The application in other fields like chronic respiratory insufficiency is less well clarified. The objectives of this review are to present the pathophysiological effects and mechanisms of NHF, as far as understood, and to give an overview over the current state of relevant studies.
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The non-invasive ventilation (NIV) is widespread in the clinical medicine and has attained meanwhile a high value in the clinical daily routine. The application of NIV reduces the length of ICU stay and hospitalization as well as mortality of patients with hypercapnic acute respiratory failure. Patients with acute respiratory failure in context of a cardiopulmonary edema should be treated in addition to necessary cardiological interventions with continuous positive airway pressure (CPAP) or NIV. ⋯ With appropriate monitoring in intensive care NIV can also be successfully applied in pediatric patients with acute respiratory insufficiency. Furthermore NIV can be useful within palliative care for reduction of dyspnea and improving quality of life. The aim of the guideline update is, taking into account the growing scientific evidence, to outline the advantages as well as the limitations of NIV in the treatment of acute respiratory failure in daily clinical practice and in different indications.
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All mechanically ventilated patients must be weaned from the ventilator at some stage. According to an International Consensus Conference the criteria for "prolonged weaning" are fulfilled if patients fail at least 3 weaning attempts (i. e. spontaneous breathing trial, SBT) or require more than 7 days of weaning after the first SBT. This occurs in about 15 - 20 % of patients. ⋯ It is an initiative of the German Respiratory Society (Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V., DGP) in cooperation with other societies (see acknowledgement) engaged in the field chaired by the Association of Scientific and Medical Societies in Germany (Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften, AWMF). This guideline deals with the definition, epidemiology, weaning categories, underlying pathophysiology, therapeutic strategies, the weaning unit, transition to out-of-hospital ventilation and therapeutic recommendations for end of life care. This short version summarises recommendations on prolonged weaning from the German guideline.
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Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. ⋯ The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF.