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Systemerkrankungen

  • Martin Burdelski
  • Thomas Lang
  • Dietrich von Schweinitz

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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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Martin Burdelski
    • 1
  • Thomas Lang
    • 2
  • Dietrich von Schweinitz
    • 3
  1. 1.Campus KielUniversitätsklinikum Schleswig-HolsteinKielDeutschland
  2. 2.Klinik für Kinder- und JugendmedizinKlinikum StarnbergStarnbergDeutschland
  3. 3.Kinderchirurgische Klinik und Poliklinik im Dr. von Haunerschen KinderspitalKlinikum der Universität MünchenMünchenDeutschland

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