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Peutz-Jeghers-Syndrom Beobachtungen am klinikum mannheim über 25 jahre

Peutz-Jeghers syndrome. Cases seen over 25 years at the municipal clinic in Mannheim

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Abstract

Observations in our clinic and others reviewed in the literature result in a new picture of the Peutz-Jeghers syndrome. It turns out to be a hereditary polypose syndrome which is hard to define. The tendency to malignant degeneration of polyps and development of associated neoplasms is almost impossible to forecast. In addition operations frequently involve complications and often need to be repeated more than once. Radical removal of all polyps must be the aim of treatment; the patients must then be closely followed up by clinical examination, endoscopy and radiology to avoid complications of regrowth and to make sure degeneration and associated neoplasms are detected at an early stage.

Zusammenfassung

An Hand eigener Beobachtungen sowie einer Literaturübersicht wird ein neues Bild des Peutz-Jeghers-Syndroms entworfen. Es stellt sich als ein schwer abgrenzbares, hereditäres Polyposesyndrom mit einer kaum einschätzbaren Neigung zu Entartung und Entwicklung assoziierter Neoplasmen einerseits und wiederholten komplikationsträchtigen Operationen andererseits dar. Deshalb ist therapeutisch eine möglichst vollst↭dige Entfernung der Polypen zu fordern. Die Patienten müssen engmaschig klinisch und endoskopisch bzw. radiologisch kontrolliert werden, um polybedingten Komplikationen vorzubeugen und Entartungen und assoziierte Neoplasmen rechtzeitig zu erkennen.

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Authors and Affiliations

  1. Kinderchirurgische Klinik, Klinikum Mannheim der Universität Heidelberg, Theodor-Kutzer-Ufer, D-68135, Mannheim

    S. Loff, L. Wessel, H. Wirth & K. -L. Waag

  2. Abteilung für Chirurgische Endoskopie, Klinikum Mannheim der Universität Heidelberg, Mannheim

    B. C. Manegold

  3. Pathologisches Institut, Klinikum Mannheim der Universität Heidelberg, Mannheim

    H. Pilch

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  1. S. Loff
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  2. L. Wessel
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  4. K. -L. Waag
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Loff, S., Wessel, L., Wirth, H. et al. Peutz-Jeghers-Syndrom Beobachtungen am klinikum mannheim über 25 jahre. Langenbecks Arch Chir 380, 43–52 (1995). https://doi.org/10.1007/BF00184415

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