{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T22:14:23Z","timestamp":1787004863685,"version":"build-2736575974"},"reference-count":35,"publisher":"Georg Thieme Verlag KG","issue":"06","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Zentralbl Chir"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n                  <jats:p>Die wissenschaftliche Evidenz, um das klinische Management des Peutz-Jeghers-Syndroms (PJS) fundiert zu untermauern, ist gering. Hier fassen wir die europ\u00e4ischen Leitlinien zusammen, die\n          von der EHTG (European Hereditary Tumor Group) 2021 publiziert wurden, erg\u00e4nzt mit seit der Publikation hinzugekommenen, f\u00fcr die klinische Betreuung bedeutsamen neuen Aspekten. Die EHTG hat\n          mit der neuen publizierten Leitlinie 1 die vorangegangene Fassung von 2010 \u00fcberarbeitet und ma\u00dfgeblich erg\u00e4nzt. Hierzu wurde die gesamte vorhandene Literatur systematisch auf Evidenz gepr\u00fcft und anschlie\u00dfend\n          die St\u00e4rke der Empfehlung mit der GRADE-Methode (Grading of Recommendations Assessment, Development and Evaluation) festgelegt. Anschlie\u00dfend erfolgte ein Delphi-Prozess, wobei ein Konsensus\n          dann erreicht wurde, wenn \u2265\u00a080% der stimmberechtigten Mitglieder ein \u00fcbereinstimmendes Ergebnis erzielten.<\/jats:p>\n                  <jats:p>Die einzigen weiteren Leitlinien neueren Datums, die das PJS-Management thematisieren, beschr\u00e4nkten sich ausschlie\u00dflich auf die gastrointestinalen und pankreatischen Manifestationen. Diese\n          Empfehlungen wurden seitens der Leitlinienkommission \u00fcberpr\u00fcft und mangels neuerer, relevanter Literatur unver\u00e4ndert \u00fcbernommen. Zus\u00e4tzliche, bisher nicht ber\u00fccksichtigte klinische Aspekte\n          wurden definiert und hinzugenommen und in eine weitere systematische Literatursuche einbezogen. Somit wurden erg\u00e4nzende und teilweise erstmalig thematisierte Empfehlungen f\u00fcr seltenere\n          assoziierte Manifestationen erg\u00e4nzt. Es muss festgestellt werden, dass 10 Jahre nach der Vorg\u00e4ngerversion dieser Leitlinien nur wenig neue Evidenz in der Literatur generiert wurde. Wie bei\n          allen seltenen Erkrankungen wird appelliert, nationale und internationale Kooperationsstudien zu planen, um die Fallzahlen zu erh\u00f6hen und offene klinische Fragestellungen mit Evidenzen zu\n          belegen, die dann zu einer besseren Patientenversorgung f\u00fchren. Innerhalb der skizzierten Restriktionen stellen diese europ\u00e4ischen Leitlinien die aktuellste Referenz f\u00fcr die Betreuung von\n          Patienten mit PJS dar.<\/jats:p>","DOI":"10.1055\/a-2109-3140","type":"journal-article","created":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T19:32:37Z","timestamp":1693942357000},"page":"492-501","source":"Crossref","is-referenced-by-count":1,"title":["Management des Peutz-Jeghers-Syndroms"],"prefix":"10.1055","volume":"148","author":[{"given":"Gabriela","family":"M\u00f6slein","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Heredit\u00e4re Tumore, Ev. 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