Pankreasinsuffizienz bei Mukoviszidose und metabolische Komplikationen — ein diagnostisch vielschichtiges Problem

  • S. Koletzko
Conference paper

Zusammenfassung

Die cystische Fibrose (CF) oder Mukoviszidose ist eine autosomal rezessiv vererbte Multiorganerkrankung. Sie ist die häufigste Ursache einer exokrinen Pankreasinsuffizienz im Kindes- und Jugendalter. Ca. 85–90% aller CF-Patienten sind pankreasinsuffizient und bedürfen einer oralen Substitution mit Pankreasenzymen [62]. Neben dem Pankreas sind alle exokrinen Drüsen und Organe betroffen, die durch Epithelzellen ausgekleidet sind. Im Gegensatz zur Lungenbeteiligung beginnt die Pankreaserkrankung meistens bereits in utero. So sind es im jungen Kindesalter meistens die Zeichen der Malassimilation mit ihren Folgen, die eine CF vermuten lassen und zur Diagnose führen.

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  • S. Koletzko

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