Carsten Schwarz, Jutta Bend, Helge Hebestreit, Michael Hogardt, Christian Hügel, Stephan Illing, Jochen G Mainz, Ernst Rietschel, Sebastian Schmidt, Bernhard Schulte-Hubbert, Helmut Sitter, Marc Oliver Wielpütz, Jutta Hammermann, Ingo Baumann, Frank Brunsmann, Doris Dieninghoff, Ernst Eber, Helmut Ellemunter, Patience Eschenhagen, Caroline Evers, Saskia Gruber, Assen Koitschev, Julia Ley-Zaporozhan, Uta Düesberg, Hans-Joachim Mentzel, Thomas Nüßlein, Felix C Ringshausen, Ludwig Sedlacek, Christina Smaczny, Olaf Sommerburg, Sivagurunathan Sutharsan, Ralf-Peter Vonberg, Ann-Katrin Weber, Jovita Zerlik
Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the airway epithelial lining fluid which leads to reduction of the mucociliary clearance.Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur...
February 13, 2024: Pneumologie