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Prune-belly-Syndrom (PBS)

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Abstract

Rückständig in mesenchymaler Entwicklung, zusammen mit hochgradiger vesi-koureterorenaler refluxiver Dysplasie, verbunden mit defekter, dysmorpher trigonaler Organisation, weiter verbunden mit Aplasie der Bauchdecke, mit hochgradigem Maldes-census testis, mit unterentwickelter Prostata, zudem noch kompliziert mit peripherer Korrelationspathologie — so ist das Prune-belly-syndrome eine schwerwiegende Mehrfachfehlbildung mit autosomal rezessivem Hintergrund, Plastisch operative Korrekturen an Blase und Harnleiter haben eine begrenzte, dennoch vorteilhafte Indikation, Die Prognose ist oft ungunstig. Viele dieser Kinder müssen sich der Dialyse und Nierentransplantation unterziehen, Mädchen sind kaum beteiligt — und wenn, nur in schwächerer Ausprägung,

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© 2001 Springer-Verlag Berlin Heidelberg

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Sigel, A. (2001). Prune-belly-Syndrom (PBS). In: Sigel, A., Ringert, RH. (eds) Kinderurologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-08080-1_9

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  • DOI: https://doi.org/10.1007/978-3-662-08080-1_9

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-08081-8

  • Online ISBN: 978-3-662-08080-1

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