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Tuberöse Sklerose

  • Ralf Wienecke
Part of the Molekulare Medizin book series (MOLMED)

Zusammenfassung

Die Tuberöse Sklerose ist eine autosomal-dominant vererbte Erkrankung mit variabler Expression. Patienten mit Tuberöser Sklerose entwickeln multiple benigne Tumoren in vielen Organsystemen. Bei den Tumoren handelt es sich meist um hamartöse Fehlbildungen. Hamartome sind benigne Tumoren, die aus Zellen bestehen, die im betroffenen Gewebe auch physiologischerweise vorkommen, aber in ihrer Anzahl, Lokalisation, Organisation oder Morphologie abnormal sind. Es bestehen zelluläre Defekte der Differenzierung, Proliferation und Migration. Gewöhnlich wird die Tuberöse Sklerose unter den neurokutanen Syndromen eingeordnet. Diese Gruppe umfasst z.B. die Neurofibromatose Typ 1 und Typ 2, das Von-Hippel-Lindau-Syndrom und das Sturge-Weber-Syndrom. Im Unterschied zu diesen Erkrankungen betrifft die Tuberöse Sklerose neben der Haut und dem zentralen Nervensystem fast alle Organsysteme, insbesondere auch die Nieren und das Herz.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2001

Authors and Affiliations

  • Ralf Wienecke
    • 1
  1. 1.Klinik und Poliklinik für Dermatologie und AllergologieLudwig-Maximilians-Universität MünchenMünchen

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