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Systemerkrankungen

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Pädiatrische Gastroenterologie, Hepatologie und Ernährung

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Zusammenfassung

Systemerkrankungen als Ursache einer Lebererkrankung sind häufig, ohne dass man genaue Zahlen angeben kann. Die verschiedenen Grunderkrankungen sind für sich betrachtet zwar selten, nur dadurch, dass viele Erkrankungen in Betracht gezogen werden müssen, ergibt sich eine relative Häufung. Durch Fortschritte auf dem Gebiet der molekularbiologischen Diagnostik insbesondere bei den Stoffwechselerkrankungen lassen sich heute bereits viele der in Frage kommenden Grunderkrankungen eindeutig nachweisen. Allerdings ist bei keiner der Erkrankungen ein hundertprozentiger molekularbiologischer Nachweis möglich. Damit ergibt sich eine sichere Diagnose nur bei einem positiven Nachweis. Bei fehlendem Nachweis einer bisher bekannten für die Erkrankung spezifischen Mutation bleibt die Zuordnung entweder enzymatischen Tests oder klinischer Diagnose vorbehalten. Insbesondere bei der Manifestation als akutes Leberversagen ist die für die Diagnosesicherung erforderliche Zeit damit oft nicht vorhanden.

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Authors and Affiliations

  1. Campus Kiel, Universitätsklinikum Schleswig-Holstein, Schwanenweg 20, 24105, Kiel, Deutschland

    Martin Burdelski Prof. Dr.

  2. Klinik für Kinder- und Jugendmedizin, Klinikum Starnberg, Oßwaldstr. 1, 82319, Starnberg, Deutschland

    Thomas Lang Prof. Dr.

  3. Kinderchirurgische Klinik und Poliklinik im Dr. von Haunerschen Kinderspital, Klinikum der Universität München, Lindwurmstr. 4, 80337, München, Deutschland

    Dietrich von Schweinitz Prof. Dr.

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  1. Martin Burdelski Prof. Dr.
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  3. Dietrich von Schweinitz Prof. Dr.
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  1. Zentrum für Kinder- und Jugendmedizin, Christliches Kinderhospital Osnabrück, Johannisfreiheit 1, 49074, Osnabrück, Deutschland

    Burkhard Rodeck Priv.-Doz. Dr.

  2. Abteilung Allgemeine Pädiatrie und Neonatalogie, Universitätsklinikum Gießen und Marburg GmbH, Zentrum für Kinderheilkunde und Jugendmedizin, Feulgenstr. 12, 35392, Gießen, Deutschland

    Klaus-Peter Zimmer Prof. Dr.

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Burdelski, M., Lang, T., von Schweinitz, D. (2013). Systemerkrankungen. In: Rodeck, B., Zimmer, KP. (eds) Pädiatrische Gastroenterologie, Hepatologie und Ernährung. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-24710-1_20

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