Zusammenfassung
Es wird immer noch angenommen, dass Kinder mit cystischer Fibrose (CF) mit histoanatomisch normalen Lungen zur Welt kommen [30]. Zweifelsohne beginnt sich aber der pulmonale Befall bei den meisten Patienten immunologisch und funktionell bereits in den ersten Lebensmonaten zu manifestieren. Im Vordergrund steht bereits in diesem Alter einerseits die Imbalance zwischen Proteasen (Leukozytenelastase) und Antiproteasen (α1-Antitrypsin etc.) im Schleimhautfilm des Bronchialtraktes [4]. Andererseits ist lungenphysiologisch bei vielen Säuglingen eine pulmonale Überblähung festzustellen, ein Befund, welcher klinisch über Monate verborgen bleiben kann, und vom Genotyp abhängig zu sein scheint [13]
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Aebi, C. et al. (2001). Atemwegserkrankung. In: Reinhardt, D., Götz, M., Kraemer, R., Schöni, M.H. (eds) Cystische Fibrose. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-56796-4_7
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