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Krankheiten der extrahepatischen Gallenwege

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Pädiatrie

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Zusammenfassung

Choledochuszysten sind kongenitale, segmentale, zystische Erweiterungen des Gallengangssystems. Die Gruppe der Choledochuszysten wird entsprechend der Klassifikation von Todani in 5 Subtypen eingeteilt:

  • Typ I: zystische Dilatation des gemeinsamen extrahepatischen Gallengangs ohne Erweiterung der intrahepatischen Gallengänge:

    • Typ Ia: Erweiterung beschränkt auf Ductus choledochus auf Höhe des Ductus cysticus;

    • Typ Ib: Erweiterung des Ductus choledochus distal des Zystikusabgangs;

    • Typ Ic: Dilatation des Ductus choledochus und des Ductus hepaticus communis;

  • Typ II: Malformation des Ductus choledochus mit Divertikel;

  • Typ III: Choledochozele mit Obstruktion der Papilla Vateri;

  • Typ IV: multiple intrahepatische und/oder extrahepatische zystische Malformationen;

  • Typ V: einzelne oder multiple zystische Erweiterungen der intrahepatischen Gallengänge.

Die Häufigkeit der Choledochuszysten wird in unseren Breiten auf 1:13.000 geschätzt. Es bestehen erhebliche regionale Unterschiede. So werden die meisten Patienten aus dem asiatischen Raum berichtet (50 % aus Japan). Etwa die Hälfte der Patienten wird in den ersten 2 Lebensjahren diagnostiziert. Am häufigsten werden die Typen I und IV beobachtet. Mädchen sind 4-mal häufiger betroffen als Jungen.

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Notes

  1. 1.

    Umrechnung: mg/dl×17,1=μmol/l.

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Authors and Affiliations

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

    Thomas Lang Prof. Dr. med.

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  1. Thomas Lang Prof. Dr. med.
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Editors and Affiliations

  1. Zentrum für Kinder und Jugendmedizin, Universitätsklinikum Heidelberg, Heidelberg, Germany

    Georg F. Hoffmann

  2. Forschungsinstitut für Kinderernährung, Dortmund, Germany

    Michael J. Lentze

  3. Zentrum für Kinder- und Jugendmedizin, Universitätsmedizin Mainz, Mainz, Germany

    Jürgen Spranger

  4. Zentrum für Kinder- und Jugendmedizin, Universitätsmedizin Mainz, Mainz, Germany

    Fred Zepp

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Lang, T. (2014). Krankheiten der extrahepatischen Gallenwege. In: Hoffmann, G., Lentze, M., Spranger, J., Zepp, F. (eds) Pädiatrie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-41866-2_139

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  • DOI: https://doi.org/10.1007/978-3-642-41866-2_139

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