Der Internist
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Mucoviscidosis (cystic fibrosis [CF]) is the most common autosomal recessive inherited multisystem disease with fatal outcome. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which lead to a dysfunctional chloride channel and a defective CFTR protein. As a consequence, retention of insufficiently hydrated mucus affects multiple essential organs, mainly the lungs and airways, pancreas, liver, biliary tract and intestines. ⋯ This improvement in the prognosis was achieved by the cooperative spirit and networking of the very active and international CF research community and by establishing a multidisciplinary clinical CF team that implements the existing evidence in various aspects of standardized care together with the CF patient. This narrative review article presents the evidence in selected aspects of CF treatment, with special consideration of the most recent development of highly effective CFTR modulator treatment. This treatment will soon become available for more than 90% of the global CF patients and transform the pathophysiology as well as the course of disease towards a treatable chronic condition in internal medicine.
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The term sepsis was redefined in 2016 as a life-threatening organ dysfunction caused by an inadequate host response to an infection. The German S3 guidelines for the treatment of sepsis were published in 2018. ⋯ Despite an improved understanding of the pathophysiology, there is still no effective causal sepsis treatment, i.e. directed against the pathological host reaction. The treatment of patients with sepsis is therefore still based on the basic principles of correction of volume deficits, anti-infective agents, source control and organ support, including the symptomatic treatment of vasoplegia with catecholamines.